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RESPIRATORY PATHOLOGY Disease Assessment and Review Michael Haines, MPH, RRT-NPS, AE-C Respiratory Pathology Table of Contents Topic Introduction Symptoms and Diagnosis of Lung Disorders Diagnostic Tests Medical Terminology Common Respiratory Therapy Terms and Laboratory data Types of Hypoxemia Asthma ARDS ALS Alpha 1 Antitripsyin Disorder Anemia Aspiration Pneumonia Aspergillus Atelectasis Asbestosis AIDS Abruptio Placenta Bronchiectasis Bronchopulmonary Dysplasia Bronchitis Bronchiolitis Cystic Fibrosis Croup Cerebral Vascular Accident (CVA) Congestive Heart Failure (CHF) Coronary Artery Disease (CAD) Cor Pulmonale Congenital Diaphragmatic Hernia Cardiac Tamponade Central Sleep Apnea Choanal Atresia Coarctation of the Aorta Carbon Monoxide Posioning Diabetes/ Diabetic Keto acidosis Eclampsia Emphyema End Stage Renal Disease (ESRD) Epiglottitis Emphysema Endocarditis Flail Chest Foreign Body Airway Obstruction (FBAO) Gullian Barre Syndrome Gastroschisis Gastrointestinal Bleed (GIB) Good Pastures Syndrome Pathology Review Page number 4-6 7-9 9-14 15-17 17-21 21-22 22-26 27-29 29-30 30-31 31-32 32-33 33-34 34 35 35-36 37-38 38-40 41-42 42-44 45-46 46-50 50-52 52-54 54-56 56-57 57-58 58-60 60-61 61-62 62 62-63 64-65 65-66 66 67 67-68 68-70 70-71 71-72 72 73 73-75 75 75-76 76-77 Page 2 Hepatic Encephalopathy HELLP Syndrome Hemothorax Infant Respiratory Distress Syndrome (RDS) Idiopathic Pulmonary Fibrosis Kyphoscoliosis Legionnaires Disease Lung Cancer Meconium Aspiration Muscular Dystrophy Myasthnia Gravis MRSA Mounier-Kuhn’s Syndrome Myocardial Infarction Obstructive Sleep Apnea (OSA) Pleurisy, Pleural effusion, Pneumothorax Pneumonitis Pulmonary Edema Pulmonary Emboli Pneumococcal Pneumonia/other pneumonia Pulmonary Hypertension/PPHN Pierre Robin Syndrome Paroxysmal Supraventricular Tachycardia Patent Ductus Arteriosus (PDA) Pectus Carinatum Poliomyelitis RSV ROP Sarcoidosis Sepsis Silicosis Subcutaneous Emphysema Systemic Lupus Erthematosus Tetanus Tetralogy of Fallot Tracheamalcia Transient Tachypnea of the Newborn Tuberculosis Ventricular Fibrillation Ventricular Septal Defect Ventricular Tachycardia Pathology Review 77-78 78 78-79 79-80 80-84 84-85 85 85-87 87-88 88-89 89-91 91-92 92-93 93-94 94-97 97-101 101-103 103-107 107-109 110-116 117-122 122-123 123-124 124-125 125-126 126-127 127-128 128-129 129-132 132-133 133 133 133-135 135 135-136 137 137-138 138-140 140-141 141-142 142-143 Page 3 Introduction Being able to properly assess, treat and identify a patient with respiratory compromise is vital and the key to a successful and meaningful career in respiratory therapy. In order to do this, one must first have a thorough understanding of pulmonary physiology and anatomy. After this is mastered, you must then understand basic etiologies of the major pulmonary pathologies. Once this is accomplished one must then be able to identify and indirectly diagnose pulmonary pathology as it relates to specific symptoms and diagnostic tests. Diseases and syndromes affecting the lung may occur either in the lung itself (such as in pneumonia, lung cancer, pulmonary fibrosis, asthma and COPD); in the interstial space (pulmonary emboli, widened AC membrane and pulmonary hypertension); in the pleural space (pleural effusion, pleurisy, pneumothorax, or empyema); neurological (brain death, spinal cord injury or anoxia); in muscle strength (myasthenia gravis, guillian baire, muscle dystrophy, or ALS); anatomical (kyphoscoliosis, pigeon chest, flail chest or malformation of lung); or as a secondary illness (ARDS, Sepsis, heart failure or renal failure). There are literally thousands of ailments leading to respiratory compromise. You must master the common ones and be able to treat and sustain ventilation for most. The respiratory system is a complex and vital part of the human body. The respiratory system must be fully operational (either naturally or artificially) to sustain life. To be considered functional, the respiratory system must possess six basic required functions. The first required function includes the location of the gaseous exchange, as it must occur deep within the lungs so that the air has had a chance to be warmed, moistened, and cleansed on its journey to be exchanged for waste gases. Air born particles must be removed during the oxygen’s journey deep into the lungs. Secondly, the membrane lining of the lungs (mucosa) must be moist, as carbon dioxide and oxygen can undergo the process of gaseous exchange only in water soluble material. This process is called diffusion and can not happen in a dry lung. The respiratory system must have an extensive network of capillaries to allow the exchange of oxygen within the body’s tissues. An incompetent network of capillaries will result in some of the body’s tissue being denied life sustaining oxygen (as with diseases in the interstial space outside the lung parenchyma). The respiratory system relies on a competent and efficient ventilation mechanism in order for there to be ample replenishment of the necessary oxygen. Ventilation is usually autonomic controlled by the medulla oblongata and PONS respiratory center. Chemoreceptor organs in the aortic, brain and carotid arteries Pathology Review Page 4 may increase or decrease minute volume depending on high or low levels of paCo2 or paO2. The body’s tissue can not survive off of a minimal amount of oxygenation (minimum being 18-21%). The respiratory system must be able to function automatically and not require constant conscious monitoring. Monitoring and feedback must all happen automatically throughout the body in order for the respiratory system to respond appropriately. The system also requires a conscious over ride in order to intentionally create a variance in airflow if desired or needed (when panicked or excited for example, an individual may increase ventilation to meet demand without help from chemorecptors). By meeting these basic necessities, the respiratory system is designed with very intricate but purposeful properties, which allow it to take on a variety of responsibilities. The metabolic process and the sustaining of life rely entirely on the functions of these basic principles. The respiratory system is responsible for providing the blood stream with life sustaining oxygen while removing toxic waste gases, enables the production of sound by passing over the vocal cords which in turn cause vibration, and is partially responsible for the compression of the abdominal muscles which assist in urination, defecation, and child birth as well as assists in laughter and other basic bodily functions surrounding the abdomen. The abdominal muscles rely on the respiratory system for functions which require significant exertion, as these muscles become more effective with a rush of extra oxygen, hence the natural desire to take a deep breath when pushing the abdominal muscles or even lifting a heavy object. In numerous ways, the back muscles benefit similarly from a rush of fresh oxygen. Without the respiratory functions as they are designed, protective reflexes that require a lapse in breath such as coughing, sneezing, or hiccupping could not effectively occur. These reflexes enable the body to reject foreign substances and keep them from entering the body via the respiratory system. When these functions are inhibited as in neuromuscular or neurological compromise, individuals are more prone to infections. The structure, design, and passages involving the respiratory system are quite extensive and include the nasal cavity, the larynx, the trachea, the pharynx, the bronchi, the bronchioles, and the pulmonary alveoli within the lungs. The upper respiratory system is segregated by the nasal passages, the pharynx, and the associated and necessary related structures while the lower respiratory system is segregated by the larynx, the trachea, the bronchiole system, the pulmonary alveoli, and the lungs. To keep the segregation and division of the respiratory system simple, it is typically divided by the generalization of conduction and respiration. A percentage of the respiratory system is divided up by its ability to relate to breathing in while the other half is associated with the body’s need for transportation of elements. These divisions are known as the respiratory division and the conduction division, respectively. The conduction division is created by any and all relative structures or cavities that “conduct” the gases in either form to and from the pulmonary alveoli while the respiratory division is defined by the pulmonary alveoli as well as any and all cavities or relative structures that assist with the exchange of gases in either form between the air and the blood. The respiratory system, as complex and intricate as it is, is also quite viable and strong. The system is designed to compliment and function cooperatively with other systems to create and sustain life, energy, and endurance in nearly any reasonable situation. Pathology Review Page 5 Many ailments and diseases may directly or indirectly affect the lung itself, pleural space, neurological control of ventilation or diaphragm. Since the muscles surrounding the lung contain no skeletal muscle, they do not contract and therefore rely on either neurological stimulation (phrenic nerve) or assistance from abdominal, intercostals or neck muscles to contract. Conditions affecting muscle control or neurological control will impede normal ventilation even though it does not directly occur in the lung. Some muscular diseases include Myasthenia Gravis, Guilian Barre Syndrome, Muscle Dystrophy and ALS. Neurological conditions would include brain anoxia, spinal cord injuries or severed phrenic nerve. Without proper neurological stimulation, ventilation is erratic and ineffective, cough and gag reflex is absent or dulled requiring the aid of mechanical ventilation and bronchial hygiene techniques for proper gas exchange at a constant rate. The diaphragm, located below the lungs, is the major muscle of respiration. It is a large, dome-shaped muscle that contracts rhythmically and continually, and most of the time, involuntarily. Upon inhalation, the diaphragm contracts and flattens and the chest cavity enlarges. This contraction creates a vacuum, which pulls air into the lungs. Upon exhalation, the diaphragm relaxes and returns to its domelike shape, and air is forced out of the lungs. With diseases affecting the pleural space (which is normally negative) the normal vacuum affect is hindered and tidal volume is reduced; impairing gas exchange. Pathology Review Page 6 Symptoms and Diagnosis of Lung Disorders Being able to first recognize respiratory symptoms as an acute or chronic disease or condition is vital for haste treatment. Some conditions such as COPD have chronic symptoms which become tolerable to patients and may require little intervention while other conditions such as CHF result in acute pulmonary edema requiring immediate attention. In order to classify acute from chronic one must look at the overall picture. Vital signs for example with an acute patient will reflect a panicked metabolic state (uncompensated mechanisms). Chronic on the other hand will reflect a compensated system (enlarged heart, balanced pH, enlarged respiratory muscles and so forth). I Most lung disorders first present themselves with a subjective feeling of shortness of breath. This is later assessed objectively as being dyspnea secondary to an increased work of breathing due to a defined underlying cause. Secondly auscultation of the lungs reveals adventitious sounds indicating either reduced airflow (decreased or diminished breath sounds), fluid in the alveoli (crackles), secretions in the bronchi (rhonci), rubbing of pleural space (pleural friction rub) or narrowing of the air passages (wheezing or stridor). Thirdly cough is produced as a result of compromised airflow with or without accumulation of secretions. Sputum of varying colors may be produced (darker pigments reveal infection while lighter sputum reveals inflammatory cells), blood may also be produced. Increased cough may be a sign of a bacterial infection in the lining of the airway (acute bronchitis) or as a result of decreased or ineffective cilia (chronic bronchitis). Other symptoms of respiratory disease include: Tachypnea= respiratory rate greater than 20 but depends on age. Caused as a result of hypoxemia, pain from displaced pleural pressure, infiltrative processes in the lung, decreased FRC or compensating acidosis. Increased rates may also be caused by fever, being anxious, having pain, emotional distress, or exercise. You must decide if the tachypnea is from a pathological condition or less intrusive means. Cyanosis = A bluish color of the skin and the mucous membranes due to insufficient oxygen in the blood. For example, the lips may show cyanosis. Cyanosis can be evident at birth, as in a "blue baby" who has a heart malformation that permits blood that is not fully oxygenated to enter the arterial circulation. Cyanosis can also appear at any time later in life. The blue discoloration of cyanosis is seen most readily in the beds of the fingernails and toenails, and on the lips and tongue. It often appears transiently as a result of slowed blood flow through the skin due to the cold. As such, it is not a serious symptom. However, in other cases cyanosis is a serious symptom of underlying disease or a chronic symptom as in COPD. Cough = A normal reflex that clears the lung of debris or retained secretion. The character and frequency of the cough may indicate pathology. Asthma often presents with a cough due to inflammation and increased production from goblet cells. Other common conditions with cough include: TB (hemoptysis), bronchitis. Bronchiactasis, emphysema and lung cancer (hemopysis). The amount, color, and consistency of sputum produced from cough are important. For example: Yellow, Green and brownish indicate a bacterial infection whereas white/clear sticky (Mucoid) sputum represents inflammatory cells such as in Asthma. Tachycardia = HR greater than 100 but depends on age. May result from benign origins such as exercise, excitement or agitation. It may be caused from cardiac disease such as artrial fibrillation/flutter, cardiac myopathy or supraventricular tachycardia. It may also present in the presence of hypoxia/hypoxemia from pulmonary or other disease. Dyspnea= A breathlessness condition caused by numerous factors. Normal dyspnea may occur after exercise as oxygen demand increases; or at high altitudes with less barometric pressure or from anxiety. It may occur from anemia, kidney failure (kussmauls) obstructive or restrictive lung disease, cardiac disease or other pulmonary impairment. Types include orthopnea which occurs as fluid in or around the lung shifts as the person lays flat causing a short of breath feeling. Dyspnea with mild exercise may indicate pulmonary disease. Chest Pain= May arise from the heart, indigestion, pleura or in the lung itself. For example: heart attack occurs as the myocardium is deprived of oxygen and nutrients causing a crushing Pathology Review Page 7 sensation felt into the arms and neck. Chest pain on inspiration or cough may be caused by problems inside the lung such as with pneumonia or tumor or within the pleural space as in pleurisy or pleural effusion. Indigestion occurs in the epigastria area and should not be confused with myocardial pain. Adventitious Breath Sounds: 1. Wheezing: caused from airflow obstruction from narrowing bronchi. The bronchi are narrowed by constriction of bronchial smooth muscle, build up of inflammatory cells, or from tumor and/or mucus plugs or FBAO. The most common and reversible cause of wheezing is Asthma. Other persistently chronic diseases such as emphysema may produce chronic non reversible wheezes. Fluid accumulation from cardiac failure may also produce wheezing as the fluid enters the bronchi. Unilateral wheezes usually represent FBAO or tumor while bilateral wheezes represent asthma or COPD. Wheezing may present audibly without the aid of a stethoscope, on inspiration, on expiration, both or absent. If the patient has asthma for example, the wheezing may be so cumbersome that little to no airflow is noted until a bronchodilator is administered and wheezing is heard. 2. 3. 4. Stridor: Caused by the narrowing of the pharynx and trachea. Heard without the aid of a stethoscope or heard by placing stethoscope on neck. Caused by inflammation from bacterial/viral infections as such with croup and epiglottises; from irritation from an ETT or inhalation of hot or poison gases; or from tumor or growths. Treated by reducing inflammation with cool mist, a steroid or racemic epinephrine as well as correcting the underlying disease. If stridor is severe an immediate airway is placed to protect from collapse. Neck x-rays are beneficial in diagnosis but may delay treatment. Crackles: Defined as being coarse or fine. Represents an abnormal accumulation of fluid in the bronchiole and alveoli. Heard primarily on inspiration as a crackling sound (like rice crispies in milk). Primary causes include cardiac pulmonary and non cardiac pulmonary edema; which may be blood or inflammatory cells. Both dilute and destroy surfactant rendering the alveoli defenseless against collapse. May occur anywhere in the lung but primarily occurs bibaisler. Crackles used to be called Rales. Displacing the fluid with positive pressure becomes necessary when ventilation is compromised. Pink or White frothy secretions may result as crackles enter the bronchi. Rhonchi: Caused by an accumulation of secretions in the larger airways. Heard as a dull low pitched raspy sound upon auscultation. The main cause being bacterial infections as Pathology Review Page 8 with pneumonia. Other causes include asthma, COPD, ineffective or absent cough reflex, over hydration of lung and hemorrhage. Secretions may be removed with suctioning with or without hydration or bronchoscopy. Finger Clubbing: An enlargement of the tips of the fingers or toes and a loss of the angle where the nail beds emerge. Caused by chronic hypoxemia as seen with COPD and other heart conditions. Diagnosis Once a clear understanding of symptoms is present; you can now move on to diagnosing symptoms for a more definitive solution. The first step to diagnosis is medical history. Find out the symptoms first. For example: patient Bob comes in with the following symptoms: Febrile Productive cough with thick yellow sputum Tachynpnea Rhonchi in the left middle lobe Tachycardia Painful inspiration and cough Dyspnea Weakness and fatigue Hypoxemia (low Spo2) Dull percussion LML You now have a general idea of what is causing the patient’s distress (Pneumonia) but you have no idea what the exact cause is yet. Next you ask previous history and exposures. Ask the following questions to patient Bob: Smoking history? Past pneumoias and past medical problems (weakened immune How long has he been feeling like systems, older ages, younger age, this? homelessness, alcoholism and drug Work exposures? abuse patients are more prone to Other family members sick? pneumonia) Now that you have a pretty good idea what the problem is, you need to clearly diagnose it with laboratory and diagnostic imaging such as: Chest x-ray (detect where the pneumonia is) Sputum C/S (to detect type of pneumonia) Blood test with CBC (to detect level of infection) Useful diagnostic tests CXR: The chest film will show the practitioner where the pathology is, if it is indeed in the lung or pleural space. This will help determine patient positioning and extent of bronchial hygiene required. It is also used to determine if the problem is resolving or getting worse. To look at an xray you first have to know what is normal. Remember both lung fields should be the same color density (if one is darker or lighter than the other, something is wrong). The left lung is higher Pathology Review Page 9 than the right due to the heart and the right lung is higher than the left due to the liver. If they are not in this order either the heart is on the wrong side or something else is wrong. You should not be able to see bronchioles or extensive pulmonary vasculature. If you see this it means that these small vessels have something in them and this is abnormal. You should see dome shaped diaphragms. If you don’t it means they have air trapping or a pleural effusion. You should see ribs, clavicles, scapulae (depending on if film is PA or AP), spinal cord and sternum. You should note abnormalities in any of these. You should note the size of the heart. It shouldn’t be bigger than 2/3 the diameter of the chest. You should see the trachea and note position of carina for ETT placement. Note any abnormal densities or infiltrates. Common terminology: 1. Infiltrates: anything 5. Bronchogram: fluid in unidentified as being normal bronhcioles 2. Consolidation: usually means 6. Ground glass appearance: RDS pneumonia or ARDS 3. hyperinflation: air trapping 7. Nodules: tumors, TB 4. Vascular congestion: 8. Atelectasis: collapsed area pulmonary edema or CHF Neck X-ray: Useful for determining pathologies involving the narrowing of the tissues in the neck such as with epiglottitis and croup. CAT SCAN: used to visualize tissue as well as cross sectional views. Used for detecting cancers and pulmonary emboli MRI: used to visualize blood vessel abnormalities (aortic aneurysms). Ultrasound: Used to visualize fluid in pleural space and as a guide when aspirating excess fluid in the pleural space. Used to locate internal bleeding and organ damage. V/Q scan: nuclear scanning used with radioactive materials to depict the flow of air and blood through the lungs. Done in two stages: the lung ventilation scan (inhales radioactive gas and scanner shows how gas is distributed throughout airways. Second part is the lung perfusion scan which uses a radioactive substance injected into a vein and watches lung perfusion. Used to detect pulmonary emboli. Pathology Review Page 10 Positron Emission Tomography (PET): detects malignant cancer cells. Thorencentesis: Thoracentesis is a procedure in which a needle is inserted through the back of the chest wall into the pleural space (a space that exists between the two lungs and the interior chest wall) to remove fluid or air. Pleural fluid analysis is the microscopic and chemical lab analysis of the fluid obtained during thoracentesis. The normal function of the lungs is to expand with each breath taken in (like a balloon), fitting closely to the rib cage. With each expansion of the lungs, there is a corresponding expansion of the chest. There is only a little space (the pleural space) between the lungs and the interior wall of the chest. The pleural space is lubricated by fluid that is secreted and reabsorbed at relatively constant rates by the pleura (tissue that encases the lungs). Because of the constant rate of secretion and reabsorption, there is generally only a small amount of fluid in the area at any time. The pleural fluid functions to prevent friction against the chest wall as the lungs inflate and deflate with breathing. In a disease process, the pleural space may fill with fluid, such as blood, pus (from infection), serum, or lymph. Alternately, the pleural fluid may be prevented from being reabsorbed, causing an accumulation of fluid in the pleural space. When such an accumulation occurs, it becomes difficult for the lungs to fully inflate because of the fluid pressing on the lungs. Pleural fluid accumulation can cause shortness of breath, which may become more pronounced with increased activities. It may become necessary to perform a thoracentesis to remove the fluid. The accumulation of fluid in the pleural space indicates an abnormality. A thoracentesis may be performed to identify the reason (diagnostic) and/or to relieve the discomfort and symptoms (therapeutic) of excessive fluid in the pleural space. A diagnostic thoracentesis involves the removal of fluid for analysis (pleural fluid analysis). In general, pleural fluid is classified as exudate (seen in inflammatory, cancerous, or infectious conditions) or transudate (fluid that has leaked from blood or lymph vessels for various reasons). Pleural fluid analysis may help to confirm or rule out infections or diseases such as Pathology Review Page 11 cancer, congestive heart failure, liver failure, or pulmonary hypertension (high pressure in the lungs’ blood vessels). Therapeutic thoracentesis may help to relieve discomfort from shortness of breath due to the pressure caused by fluid accumulation. Bronchoscopy: Bronchoscopy is a procedure in which a cylindrical fiberoptic scope is inserted into the airways. This scope contains a viewing device that allows the visual examination of the lower airways. There are two types of bronchoscopes, a rigid tube and a fiberoptic tube. Because of its flexibility, the fiberoptic tube is usually preferred. However, if the purpose of the procedure is to remove a foreign body caught in the wind-pipe or lungs of a child, the more rigid tube must be used because of its larger size. The patient will either lie face-up on his/her back or sit upright in a chair. Medication to decrease secretions, lessen anxiety, and relax the patient are often given prior to the procedure. While breathing through the nose, anesthesia is sprayed into the mouth or nose to numb it. It will take one to two minutes for the anesthesia to take effect. Once this happens, the bronchoscope will be put into the patient's mouth or nose and moved down into the throat. While the bronchoscope is moving down the throat, additional anesthesia is put into the bronchoscope to numb the lower parts of the airways. Using the eyepiece, the physician then observes the trachea and bronchi, and the mucosal lining of these passageways, looking for any abnormalities that may be present. If the purpose of the bronchoscopy is to take tissue samples or biopsy, forceps or a bronchial brush are used to obtain cells. If the purpose is to identify an infectious agent, a bronchoalveolar lavage (BAL) can be used to gather fluid for culture purposes. Also, if any foreign matter is found in the airways, it can be removed. Another procedure using bronchoscopy is called fluorescence bronchoscopy. This can be used to detect pre-cancerous cells present in the airways. By using a fluorescent light in the bronchoscope, precancerous tissue will appear dark red, while healthy tissue will appear green. This technique can help detect lung cancer at an early stage, so that treatment can be started early. Pathology Review Page 12 Polysomnography is a comprehensive recording of the biophysiological changes that occur during sleep. Polysomnography is usually performed at night during sleep. This diagnostic test monitors many body functions including brain (EEG), eye movements (EOG), muscle activity or skeletal muscle activation (EMG), heart rhythm (ECG), and breathing function or respiratory effort during sleep. For a polysomnogram, the EEG will generally consist of four "exploring" electrodes and two "reference" electrodes (unless a seizure disorder is suspected, in which case more electrodes will be applied to document the appearance of seizure activity). The exploring electrodes are usually attached to the scalp near the central and occipitial portions of the brain via a paste that will conduct electrical signals originating from the neurons of the cortex. These electrodes will provide readout of the brain activity that can be "scored" into different stages of sleep (1, 2, 3, 4, REM, and Wakefulness). Pulmonary Function Test: Used to assess how well the lungs exchange gases, capacity to hold air and ability to move air in and out. Can be performed at bedside or in a lab. Forced Expiration Forced expiration is a simple but extremely useful pulmonary function test. A spirometry tracing is obtained by having a person inhale to total lung capacity and then exhaling as hard and as completely as possible. These tracings are a very effective way of separating normal ventilatory states from obstructive and restrictive states. In a normal forced expiration curve, the volume that the subject can expire in one second (referred to as FEV1) is usually about 80% of the total forced vital capacity (FVC), or something like four liters out of five. In an obstructive condition, however, such as asthma, bronchitis or emphysema, the Pathology Review Page 13 forced vital capacity is not only reduced, but the rate of expiratory flow is also reduced. Thus, an individual with an obstructive defect might have a forced vital capacity of only 3.0 liters, and in the first second of forced expiration, exhale only 1.5 liters, giving a FEV1/FVC of 50%. With a restrictive disease, such as fibrosis, forced vital capacity is also compromised. However, due to the low compliance of the lung in such conditions, and the high recoil, the FEV1/FVC ratio may be normal or even greater than normal. For example, a patient with a restrictive condition might have a FVC of 3.0 liters, as was seen in the obstructive cases, but the FEV1 might be as high as 2.7 liters, giving a FEV1/FVC ratio of 90%. Forced expiration curves are particularly useful because they are so reproducible. At every lung volume there exists a maximal rate of flow which cannot be exceeded. When an individual tries to exceed his maximal flow rate, he forcefully contracts his abdominal muscles to increase his already positive pleural pressure. This increases the driving pressure for air flow from the alveoli to the mouth but also causes the bronchi (whose pressure lies somewhere between that in the alveoli and that at the mouth, but is less than pleural pressure) to collapse. Thus the airways become occluded and flow is slowed until the pressure difference across the airways drops a bit, the airways can reopen, and flow can continue. Peak Flow: used as an assessment tool when determining how a patient is responding to bronchodilators. Most useful with a reversible bronchoconstriction such as Asthma. Normal values should be in the 80-90% range of the patient’s predicted normals (based on height and age). Vital Capacity and MIP: used to measure a patient’s ability to inhale and exhale deeply and quickly. Useful with neuromuscular disorders to properly assesses muscle strength. Normal MIP is -40 to -60. Acceptable is -20. Normal Vital capacity is >15 cc/kg. Thorocotomy: A physician gains access to the chest cavity (called the thorax) by cutting through the chest wall. Reasons for the entry are varied. Thoracotomy allows for study of the condition of the lungs; removal of a lung or part of a lung; removal of a rib; and examination, treatment, or removal of any organs in the chest cavity. Thoracotomy also provides access to the heart, esophagus, diaphragm, and the portion of the aorta that passes through the chest cavity.Lung cancer is the most common cancer requiring a thoracotomy. Tumors and metastatic growths can be removed through the incision (a procedure called resection). A biopsy, or tissue sample, can also be taken through the incision, and examined under a microscope for evidence of abnormal cells. Pathology Review Page 14 Medical Terminology 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24. 25. 26. 27. 28. 29. 30. 31. 32. 33. 34. 35. 36. 37. 38. 39. 40. 41. 42. 43. 44. 45. 46. ab: away from,not acoust(i): hearing acro: extremeties aden: gland adnexa: ties,connection adreno: adrenal (gland) aer: air algia: pain alveol: cavity ambi: both ameb: change amphi: around,on the sides an-,a-: without,not angi: vessel ante: before anti: against antr: cavity apo: away from appendic: appendix arter: artery arthr: joint asthenia: weakness astr: star shaped aur: ear auto: self basi: base benign: mild,not cancerous bi: two,double,both bili: bile blast: bud blephar: eyelid brachy: short brady: slow bronch: bronchus bucc(o): cheek burso: sac calc: heel,stone cantho: angle at the end of the eyelid capit: head carcin: cancer cardi: heart cata: down cau: burn cauda: tail cec: blind passage cele: hernia Pathology Review 47. 48. 49. 50. 51. 52. 53. 54. 55. 56. 57. 58. 59. 60. 61. 62. 63. 64. 65. 66. 67. 68. 69. 70. 71. 72. 73. 74. 75. 76. 77. 78. 79. 80. 81. 82. 83. 84. 85. 86. 87. 88. 89. 90. 91. celio: abdomen centesis: puncture cephal: head cerebr: brain cervic: neck cheil: lip cheir: hand chole: bile chondr: cartilage cilia: eyelash cine: move clas: break col: colon colla: glue colp(o): hollow,vagina contra: against,counter cor: heart corne: horny cost: rib crani: skull crine: to secrete cryo: cold cut: skin cyan: blue cyst: sac containing fluid cyt: cell dacry: tear dactyl: finger,toe dendr: tree,branching dent: teeth dermat: skin desis: binding di: twice dia: through digit: finger,toe dis: apart dors: back duct: tube duodeno: duodenum dura: hard dyn: pain dynam: power dys: bad ectasis: expansion ectomy: surgical removal 92. edema: swelling 93. emesis: vomiting 94. en: in 95. encephal: brain 96. end: inside,within 97. enter: intestines 98. ependym: wrapping 99. epi: upon 100. erythro: red 101. esophag: esophagus 102. esthesia: sensation 103. eu: good 104. eury: broad 105. ex: out,away from 106. fac: make,do 107. fascia: sheet,band 108. fiss: split 109. fistul: pipe 110. furca: fork shaped 111. gangli: swelling 112. gastr: stomach 113. gemin: twin 114. gen: original,production 115. ger: old 116. gingiv: gums 117. glom: ball 118. glosso: tongue 119. glyco: sweet 120. grad: walk,take steps 121. gram: record 122. gran: grain,particle 123. gravid: pregnant 124. gyn: female 125. hallux: great toe (big toe) 126. helio: sun 127. hem(at): blood 128. hemi: half 129. hepat: liver 130. heter: other 131. histo: tissue 132. hom: same 133. hormone: excite 134. hydro: water 135. hyper: above,more than normal 136. hypno: sleep 137. hypo: below 138. hyster: uterus Page 15 139. iasis: condition 140. ile: ileum 141. ili: ilium 142. infer: under 143. infra: beneath 144. inter: between 145. intra: within 146. iris: rainbow 147. iso: equal 148. itis: inflammation 149. labi: lip 150. lacrim: tear 151. lact: milk 152. lal: speech 153. lapar: abdominal wall 154. laryng: larynx 155. later: side 156. leio: smooth 157. lept: slender 158. leuk: white 159. lien: spleen 160. lig: ligament 161. lingua: tongue 162. lip: fat 163. lith: stone 165. lobo: section 166. lumbo: loins 167. lymph: watery fluid 168. lysis: loosening,destructio n 169. macro: large 170. macul: spot 171. mal: bad 172. malacia: soft 173. malign: bad 174. mamm: breast 175. mani: madness 176. mast: breast 177. maxill: upper jawbone 178. mechano: machine 179. med: middle 180. megal: enlarged 181. melan: black 182. mening: membrane 183. ment: mind 184. meta: beyond 185. metabol(e): change 186. metr: uterus 187. mi: less 188. micr: small Pathology Review 189. morph: form 190. my: muscle 191. myco: fungus 192. myel: marrow,spinal cord 193. myring: eardrum 194. necr: dead 195. neo: new 196. nephr: kidney 197. neuro: nerve (nervous system) 198. ocul: eye 199. odont: tooth 200. oid: like 201. olfact: smell 202. ologist: specialist 203. ology: study of 204. oma: tumor 205. oment: covering 206. onco: tumor 207. onych: nail,claw 208. oophor: ovary 208. opthalm-opt: eye 209. or: mouth 210. orchi: testis 211. orth: straight 212. osis: any condition 213. osmo: odor 214. ost: bone 215. ostomy: create an opening 216. oto: ear 217. otomy: cut into 218. ovar: egg 219. ovario: ovary 220. pachy: thick 221. pact: chest 222. palpebr: eyelid 223. pan: all 224. paps: gigest 225. para: beside,beyond 226. pariet: wall 227. part: labor,bring forth 228. path: disease 229. pelvi: pelvis 230. penia: decrease 231. per: throughout 232. peri: about,around 233. pexy: suspension 234. phage: to eat 235. phak: lens 236. pharmac: drug 237. pharyng: pharynx 238. phleb: vein 239. phob: fear 240. phon: voice,sound 241. phot: light 242. phrag: fence 243. phren: mind 244. physio: nature 245. pilo: hair 246. plak: plate 247. plasi: development 248. plast: surgical repair 249. platy: flat 251. plegia: paralysis 252. pleur: pleura,rib,side 253. plexus: braid 254. pneum: lung 255. pod: foot 256. poly: many 257. post: after 258. poster: back part 259. pre: in front of,before 260. pro: in front of,before 261. proct: anus 262. proli: offspring 263. proxim: nearest 264. pseud: false 265. psycho: mind 266. ptosis: falling 267. ptyal: saliva 268. puer: child 269. pulmo: lung 270. pyle: gate 271. rachi: spinal column 272. radi: ray 273. radic: root 274. ramus: branch 275. ren: kidney 276. retr(o): backwards 277. rhexis: break,burst 278. rhin: nose 279. rrhag: burst,burst forth 280. rrhaphy: suture 281. rrhe: flow 282. rug: wrinkle,fold,cease 283. sacro: sacrum 284. salpingo: tube 285. sarc: flesh Page 16 286. schiz: split 287. scirr(h): hard 288. scler(a): hard 289. scol: curved 290. scop: observe 291. sedat: quiet,calm 292. semen: seed 293. semi: half 294. sept: wall,fence 295. sinus: hollow space 296. somato: body 297. somni: sleep 298. spas: pull,draw 299. spasm: involuntary contraction 300. sphenic: wedge 301. spiro: coil 302. splanchn: internal organs 303. splen: spleen 304. spondyl: spinal column 305. squam: scale 306. sta: stand 307. steno: narrow,contracted 308. stoma: mouth 309. strept: twist 310. strict: draw tight 311. sub: under 312. supra: above 313. syn,sym: together 314. tarso: framework of the eyelid 315. tarso: ankle 316. tegument: covering,skin 317. tens: stretch 318. thalam: inner chamber 319. thel: nipple 320. therap: therapy 321. therm: heat 322. thorac: chest 323. thromb: lump,clot 324. thyro: thyroid 325. ton: stretch 326. tope: place 327. trachel: neck 328. trans: through,across 329. traumat: wound 330. tri: three 331. trich: hair 332. trip: rub,friction 333. trophy: development 334. tumor: swelling 335. turbin: shaped like a top 336. tympan: eardrum 337. umbilic: navel 338. ureter: ureter 339. urethr: urethra 340. utero: uterus 341. vaso: vessel 342. vena: vein 343. ventr: front 344. vert: turn 345. vesic: bladder 346. vestibule: entrance 347. viscro: organ 348. volv: to roll 349. vuls: twitch or pull 350. xer: dry Common RT terms 1. Anoxia: without oxygen, anoxic encephalopathy, brain death from lack of oxygen to the brain 2. Apnea: absence of breathing 3. Alv: alveoli 4. Asphyxiation: Choking, impending oxygen from entering 5. Biots breathing: erratic pattern with varying rate and depth followed by periods of apnea, typically as a result of brain injury. 6. Bradypnea: decreased rate of breathing 7. Chyene stokes breathing: increasing rate/depth followed by decreasing rate and depth followed by apnea 8. Crackles: fluid in the alveoli, heard on inspiration 9. Dyspnea: difficulty breathing 10. Eupnea: normal breathing 11. External respiration: bodily process of inhalation and exhalation 12. Hyperventilation: increase rate and depth of breathing 13. Hypoventilation: decreased rate/depth of breathing 14. Internal respiration: metabolic processes whereby certain organisms obtain energy from organic molecules 15. Kussmauls breathing: from ketoacidosis, increased rate/depth 16. Orthopnea: difficult breathing while laying flat, fluid retention/pleural effusion 17. Platypnea: difficult breathing while upright 18. Rhonci: Secretions in the large airways, coarse crackles 19. SOB: Shortness of breath, subjective term 20. Stridor: audible hoarse sounding wheeze of upper airway heard on inspiration 21. Tachypnea: increased rate of breathing Pathology Review Page 17 22. VA: alveolar ventilation, ventilation that takes into consideration tidal volume, respiratory rate and dead space volume 23. VD: dead space ventilation, gas that does not participate in gas exchange 24. Ve: minute volume, measured in liters, the product of respiratory rate times tidal volume per minute. 25. VT: tidal volume, amount a patient inhales and exhales with normal breathing 26. Wheezing: lower airway constriction, can be expiratory/inspiratory or both 27. WOB: Work of breathing, objective Labs and diagnostic tests 28. ABG: arterial blood gas, determines Co2, O2, HCO3 and pH of blood. pH 7.35-7.45, PaCO2 35-45 mmHg, PaO2 80-100, HCO2 22-26 Me/Q, BE +/-2 29. AFB: acid fast bacillus for TB diagnosis 30. Albumin: used for patients with low blood volume, protein, low with renal failure. Normal value 3.2 - 5 g/dl 31. ALT :(Serum Glutamic-Pyruvic Transaminase - SGPT) - Decreased SGPT in combination with increased cholesterol levels is seen in cases of a congested liver. We also see increased levels in mononucleosis, alcoholism, liver damage, kidney infection, chemical pollutants or myocardial infarction Normal Adult Range: 8 - 20 U/L 32. Ammonia: ammonia in the body forms when protein is broken down by bacteria in the intestines. The liver normally converts ammonia into urea, which is then eliminated in urine. Ammonia levels in the blood rise when the liver is not able to convert ammonia to urea. This may be caused by cirrhosis or severe hepatitis. Normal value 20 - 70 mcg/dl 33. ANION GAP: (Sodium + Potassium - CO2 + Chloride) - An increased measurement is associated with metabolic acidosis due to the overproduction of acids (a state of alkalinity is in effect). Decreased levels may indicate metabolic alkalosis due to the overproduction of alkaloids (a state of acidosis is in effect). Normal Adult Range: 7 - 16 mEq/L 34. BASOPHILS: Basophilic activity is not fully understood but it is known to carry histamine, heparin and serotonin. High levels are found in allergic reactions.) Normal Adult Range: 0 - 0.75 percent 35. BILIRUBIN: Elevated in liver disease, mononucleosis, hemolytic anemia, low levels of exposure to the sun, and toxic effects to some drugs, decreased levels are seen in people with an inefficient liver, excessive fat digestion, and possibly a diet low in nitrogen bearing foods Normal Adult Range 0.1 - 1.0 mg/dL 36. BNP: Brain natriuretic peptide, measures the amount of the BNP hormone in your blood. BNP is made by your heart and shows how well your heart is working. Normally, only a low amount of BNP is found in your blood. But if your heart has to work harder than usual over a long period of time, such as from CHF, the heart releases more BNP, increasing the blood level of BNP. The BNP level may drop when treatment for heart failure is working. <100 37. BUN: (Blood Urea Nitrogen) - Increases can be caused by excessive protein intake, kidney damage, certain drugs, low fluid intake, intestinal bleeding, exercise or heart failure. Decreased levels may be due to a poor diet, malabsorption, liver damage or low nitrogen intake.Normal Adult Range: 7 - 18 mg/dl 38. C and S: Culture and sensitivity, used to diagnose specific bacterial and fungal pathogens 39. Ca+: Calcium, normal 8.8 - 10.3 mg/dL, involved in bone metabolism, protein absorption, fat transfer muscular contraction, transmission of nerve impulses, blood clotting and cardiac function. Regulated by parathyroid. 40. CBC: complete blood count, used to determine, WBC, HB, Platelets 41. CBG: capillary blood gas 42. Cl-: Chloride, Elevated levels are related to acidosis as well as too much water crossing the cell membrane. Decreased levels with decreased serum albumin may indicate water deficiency crossing the cell membrane (edema), 95-105 meq/l 43. CPK: Creatine phosphokinase is an enzyme found mainly in the heart, brain, and skeletal muscle Pathology Review Page 18 44. Creatine Kinase: aka Creatine Phosphokinase (CK) - Levels rise 4 to 8 hours after an acute MI (Myocardial Infarction), peaking at 16 to 30 hours and returning to baseline within 4 days.Creatine Kinase (male) 25 - 90 U/L Creatine Kinase (female) 10 - 70 U/L 45. CREATININE: Low levels are sometimes seen in kidney damage, protein starvation, liver disease or pregnancy. Elevated levels are sometimes seen in kidney disease due to the kidneys job of excreting creatinine, muscle degeneration, and some drugs involved in impairment of kidney function. Creatinine, serum 0.6 - 1.2 mg/dl 46. CT: computerized tomography 47. CXR: chest x-ray 48. EOSINOPHILS: Elevated levels may indicate an allergic reactions or parasites.) Normal Adult Range: 1 - 3 percent 49. Fluroscopy: procedure that uses continuous florescent imaging 50. Glucose: This is a measure of the sugar level in your blood. High values are associated with eating before the test, and diabetes. The normal range for a fasting glucose is 70 - 110 mg/dL 51. Hb: hemoglobin, required to carry oxygen, decreased with blood loss, anemia, renal failure, increased with COPD. Normal 12-16 g/dl 52. HEMATOCRIT (HCT): The hematocrit refers to the 'percentage' of one's red blood cells. Normal Adult Female Range: 36 - 46 percent Normal Adult Male Range 41 - 53 percent 53. K+: Potassium is the major intracellular cation. Very low value causes Cardiac arrhythmia. Normal 3.5 - 5.0 mEq/L 54. KUB: kidney, ureter, bladder x-ray 55. Lactic Acid: Most of it is made by muscle tissue and red blood cells. When the oxygen level in the body is normal, carbohydrate breaks down into water and carbon dioxide. When the oxygen level is low, carbohydrate breaks down for energy and makes lactic acid. Lactic acid levels get higher when strenuous exercise or other conditions-such as heart failure, a severe infection (sepsis), or shock-lower the flow of blood and oxygen throughout the body. Lactic acid levels can also get higher when the liver is severely damaged or diseased, because the liver normally breaks down lactic acid. 0.7-2.1 56. LDH: (Lactic Acid Dehydrogenase) - Increases are usually found in cellular death and/or leakage from the cell or in some cases it can be useful in confirming myocardial or pulmonary infarction (only in relation to other tests). Decreased levels of the enzyme may be seen in cases of malnutrition, hypoglycemia, adrenal exhaustion or low tissue or organ activity. Normal Adult Range: 45 - 90 U/L 57. LYMPHOCYTES: Elevated levels may indicate an active viral infections such as measles, rubella, chickenpox, or infectious mononucleosis.) Normal Adult Range: 25 - 33 percent 58. MONOCYTES: Elevated levels are seen in tissue breakdown or chronic infections, carcinomas, leukemia 'monocytic' or lymphomas.) Normal Adult Range: 3 - 7 percent 59. Myoglobin: early and sensitive diagnosis of myocardial infarction in the emergency department This small heme protein becomes abnormal within 1 to 2 hours of necrosis, peaks in 4-8 hours, and drops to normal in about 12 hours. Myoglobin, serum (male): 10 - 95 ng/mL 60. Na+: Sodium is the most abundant cation in the blood and its chief base. It functions in the body to maintain osmotic pressure, acid-base balance and to transmit nerve impulses. Very Low value: seizure and neurologic symptoms, normal 136-145 mEq/L 61. NEUTROPHILS: This is the main defender of the body against infection and antigens. High levels may indicate an active infection.) Normal Adult Range: 54 - 62 percent 62. PLT: platelets, ability to clot is dependent on PLT, decreased with liver failure and blood loss normal 100,000-450,000 63. PTT: prothrombin time, time it takes to clot 64. RBC: Red Blood Cell Count aka Erythrocyte count) RBC count (female) 3.5 - 5.5 mill/mm3 RBC count (male) 4.3 - 5.9 mill/mm3 65. SGOT : Serum glutamic oxaloacetic transaminase is an enzyme that is normally present in liver and heart cells. SGOT is released into blood when the liver or heart is damaged. The blood SGOT levels are elevated with liver damage (hepatitis) or with an insult to the heart (heart attack). Pathology Review Page 19 There are some medications that can also raise SGOT levels. SGOT is also called aspartate aminotransferase (AST). SGOT will begin to rise in 8-12 hours and peak in 18-30 hours, 10-42 U/L 66. VBG: venous blood gas 67. WBC: White Blood Cell Count aka Leukocyte count) Includes Basophils, Neutrophils, Eosinophils, B Cells, T Cells, Band Cells, Monocytes Normal Adult Range: 4,000 - 12,000/mm3, increased leukocytosis (infection), decreased leucopenia (immune problem) Lung/Heart/Liver/Kidney Disease Terminology 68. chronic bronchitis: airways have become inflamed and thickened, and there is an increase in the number and size of mucus producing cells 69. cyanosis: bluish discoloration of the skin, associated with poor circulation, lack of oxygen 70. cystic fibrosis: a genetic disorder in which the lungs and pancreas are clogged with large quantities of abnormally thick mucus 71. epistaxis: bleeding from the nose 72. laryngitis: inflammation of the larynx 73. lung cancer: most common kind of cancer in both men and women, smoking is the main cause 74. sinusitis:inflammation of the sinuses 75. acute nasopharyngitis: common cold 76. Asthma: chronic allergic disorder characterized by episodes of severe breathing difficulty, coughing, and wheezing 77. CABG: Coronary artery bypass surgery 78. CHF: congestive heart failure, from valve leakage or weak myocardium 79. chronic obstructive pulmonary disease : damage to the bronchi obstructs the lungs and makes it hard to breath 80. Coronary artery disease: (CAD) hardening of the arteries from plague build up, precursor to heart attack 81. croup: an acute respiratory syndrome in children and infants characterized by obstruction of the larynx, hoarseness, and a barking cough 82. CVA: cerebral vascular accident, from a stroke 83. Diaphoresis: excessive sweating, usually as a result of respiratory failure 84. diphtheria: acute bacterial infection of the throat and upper respiratory tract 85. Emphysema: a disease characterized by abnormally enlarged and damaged alveoli of the lungs that leads to progressive hypoventilation and, ultimately, respiratory failure 86. ESLD: end stage liver disease, from cirrohsis, jaundice, increase liver enzymes, decreased clotting ability 87. ESRD: end stage renal disease, chronic kidney damage, pre or post renal destruction. Note Creatine and BUN levels, on hemodialysis 88. Hemothorax: blood in the pleural space 89. ICP: intracranial pressure, bleed/hematoma in brain 90. Impending respiratory failure: on the verge of failure, ABG may be normal however patient has an increased WOB 91. Influenza: the flu, highly infectious respiratory disease caused by a virus 92. IPF: interstitial pulmonary fibrosis 93. MI: Myocardial Infarction, heart attack, blocked coronary arteries 94. Needle decompression: removal of air from the pleural space 95. pertussis: whooping cough; contagious infection of the upper respiratory tract 96. Pharyngitis: sore throat; inflammation of the pharynx 97. Pleural effusion: fluid in the pleural space 98. Pleurisy: inflammation of the pleura cavity 99. Pneumonia: (PNA) acute inflammation and infection of alveoli and bronchioles, which fill with pus or products of the inflammatory reaction 100. Pneumothorax: Air in the pleural space Pathology Review Page 20 101. Pneumothorax: collection of air in the pleural cavity, causing the complete or partial collapse of a lung 102. Respiratory Failure: the inability to ventilate 103. rhinitis: inflammation of the nose 104. rhinorrhea: runny nose; watery flow of mucus from the nose 105. SAH: subarachnoid bleed 106. STEMI: ST elevated MI 107. Thorencentesis: removal of fluid from the pleural space 108. Tuberculosis: (Tb)An infectious disease caused by mycobacterium tuberculosis which affects the lungs but can also spread to other parts of the body. Vital signs and assessment 109. ALOC: altered level of consciousness 110. Dx: diagnosis 111. F: frequency, respiratory rate 112. HR: heart rate 113. Hx: history 114. Hypertension: (HTN) increased blood pressure, increase sodium intake, CHF, renal failure, diabetes 115. Hypotension: decreased blood pressure, failing heart, blood or fluid loss 116. Retraction: inspiratory movement of chest on inspiration, lack of surfactant or broken ribs 117. RR: respiratory rate 118. RUL: right upper lobes, RLL, LLL, LUL 119. Tachycardia: >100 adults, medications, exercise, fear/pain/anxiety all possible causes 120. Bradycardia: HR less than 60 for adult, medications, heart block Treatment frequency 121. Ad lib: as desired 122. BID: twice a day (0700, 1900) 123. HS: at bedtime 124. PRN: as needed 125. Q4: Every 4 hours, Q3… 126. QD: once a day 127. QID: four times a day (0700, 1100, 1500, 1900) 128. QS: once per shift 129. STAT: as soon as possible/immediately 130. TID: three times a day, (0700, 1300, 1900) Types of Hypoxia Histotoxic Hypoxia – This form results from tissue poisoning such as from alcohol, narcotics, and certain poisons. The utilization of oxygen by the body tissues is interfered with and the tissues are unable to metabolize the delivered oxygen. metabolic poisoning e.g. cyanide Anemic Hypoxia – The inability of oxygen to bind to the hemoglobin, as a result of a large blood loss, chronic anemia (decreased hemoglobin content), or the forming of compounds with hemoglobin (carbon monoxide, nitrites, sulfa drugs) that reduces the amount of hemoglobin available to form oxyhemoglobin. reduced red blood cell count reduced red blood cell hemoglobin Pathology Review Page 21 abnormal hemoglobin (e.g. sickle cell anaemia) reduced binding capacity of hemoglobin by e.g. carbon monoxide SMOKERS BEWARE: Carbon monoxide has an affinity for the blood 20 times greater than oxygen. Given a choice between carbon monoxide and oxygen, the hemoglobin will choose the carbon monoxide. A regular smoker has a physiologic altitude of 3,000 to 8,000 feet while at sea level. Hypoxic Hypoxia – This is a lack of oxygen as a result of a high altitude (decreased oxygen pressure) or by conditions that prevent or interfere with the diffusion of oxygen across the alveolar membrane (asthma, pneumonia, tumors, arterial venous shunts). breathing air at high altitudes inefficient breathing obstructed airway collapsed or damaged lung Stagnant Hypoxia – This is attributable to a malfunction of the circulatory system resulting in a decrease in blood flow. Causes include high g-loading, exposure to extreme hot or cold temperatures, or by shock. shock heart failure blocked blood vessel PATHOLOGY ASTHMA Asthma is a chronic disease that affects the bronchi. The disease occurs as both bronchial smooth muscle constriction and mucosa inflammation with the release of histamines from mast cells. People with asthma are largely asymptomatic until a stimulus sparks an attack, causing the airways to inflame and constrict. This causes symptoms like wheezing, coughing, chest tightness, and dyspnea especially at night and in the early morning. Asthma cannot be cured, but is completely manageable with proper education. During an asthma attack, muscles around the airways tighten up, making the airways narrower so less air flows through. Inflammation increases, and the airways become more swollen and even narrower. Goblet cells in the airway produce more mucus than usual. This extra mucus also narrows the airways. These changes make it harder to breathe. Asthma usually subsides with proper treatment, however if the symptoms persist despite treatment, a condition known as status asthmaticus occurs, usually resulting in intubation. Repeated episodes of attack Pathology Review Page 22 produce scar tissue secondary to inflammation. The scar tissue reshapes the lung, leading to chronic gas trapping and Co2 retention. What Causes Asthma? It is not clear exactly what makes the airways of people with asthma inflamed in the first place. Your inflamed airways may be due to a combination of things. We know that if other people in your family have asthma, you are more likely to develop it. New research suggests that being exposed to things like tobacco smoke, infections, and some allergens early in your life may increase your chances of developing asthma. The list below gives some examples of things that can bring on asthma symptoms. Allergens Animal dander (from the skin, hair, or feathers of animals) Dust mites (contained in house dust) Cockroaches Pollen from trees and grass Mold (indoor and outdoor) Irritants (spark attacks) Cigarette smoke A condition called gastroesophageal disease that causes heartburn and can Air pollution worsen asthma symptoms, especially at Cold air or changes in weather night Strong odors from painting or cooking Irritants or allergens that you may be Scented products exposed to at your work, such as Strong emotional expression (including special chemicals or dusts crying or laughing hard) and stress Infections Medicines such as aspirin and betablockers Sulfites in food (dried fruit) or beverages (wine) What Are the Signs and Symptoms of Asthma? Common asthma symptoms include: Coughing. Coughing from asthma is often worse at night or early in the morning, making it hard to sleep. The reason being your minute volume while asleep is lessened allowing accumulation of mucus; also norepinephrine is released in less quantaties do to lack of metabolic demand. (Norepinephrine is your bodies natural bronchodilator) Wheezing. May present as inspiratory, expiratory or both. May also be audible or silent depending on degree of constriction. Pathology Review Page 23 Chest tightness. This can feel like something is squeezing or sitting on your chest. Shortness of breath. Some people say they can't catch their breath, or they feel breathless or out of breath. Tachypnea, tachycardia, hypoxemia, increased BP, diaphoresis. dyspnea Not all people have these symptoms, and symptoms may vary from one asthma attack to another. Symptoms can differ in how severe they are: Sometimes symptoms can be mildly annoying, other times they can be serious enough to make you stop what you are doing, and sometimes symptoms can be so serious that they are life threatening. Symptoms also differ in how often they occur. Some people with asthma have symptoms only once every few months, others have symptoms every week, and still other people have symptoms every day. With proper treatment, however, most people with asthma can expect to have few or no symptoms (proper management means being compliant with treatment regimes that may require daily use of steroids). How Is Asthma Diagnosed? Ask the following: Periods of coughing, wheezing, shortness of breath, or chest tightness that come on suddenly, occur often, or seem to happen during certain times of the year or season Colds that seem to "go to the chest" or take more than 10 days to get over Medicines you may have used to help your breathing Your family history of asthma and allergies Things that seem to cause your symptoms or make them worse A spirometer is used to check the degree of reversibility of asthma by having a patient inhale an irritant to trigger an attack and then having them do the test, then the patient is given a bronchodilator and the test is repeated. If flow improved by 12% or 200 cc after treatment, it is considered reversible. Spirometry is also used to check your asthma over time to see how you are doing. Spirometry usually cannot be used in children younger than 5 years. Instead treatments are given for objective relief of symptoms. Also often the only the symptom present in small children is cough. Other tests: Allergy testing to find out if and what allergens affect you; either blood or skin. A peak flow meter every day for 1-2 weeks to check your breathing in the AM and afternoon. Tests to see if you have gastroesophageal reflux disease (one of the causes of Asthma is GERD). A test to see if you have sinus disease. Other tests, such as a chest x ray or an electrocardiogram, may be needed to find out if a foreign object or other lung diseases or heart disease could be causing your symptoms. A correct diagnosis is important because asthma is treated differently from other diseases with similar symptoms. Depending on the results of your physical exam, medical history, and lung function tests, your doctor can determine how severe your asthma is. This is important because the severity of your asthma will determine how your asthma should be treated. One way for doctors to classify asthma severity is by considering how often you have symptoms when you are not taking any medicine or when your asthma is not well controlled. Based on symptoms, the four levels of asthma severity are: Mild intermittent (comes and goes)—you have episodes of asthma symptoms twice a week or less, and you are bothered by symptoms at night twice a month or less; between episodes, however, you have no symptoms and your lung function is normal. Mild persistent asthma—you have asthma symptoms more than twice a week, but no more than once in a single day. You are bothered by symptoms at night more than twice a month. You may have asthma attacks that affect your activity. Moderate persistent asthma—you have asthma symptoms every day, and you are bothered by nighttime symptoms more than once a week. Asthma attacks may affect your activity. Severe persistent asthma—you have symptoms throughout the day on most days, and you are bothered by nighttime symptoms often. In severe asthma, your physical activity is likely to be limited. Anyone with asthma can have a severe attack—even people who have intermittent or mild persistent asthma. Pathology Review Page 24 How Is Asthma Treated? Avoiding things that bring on your asthma symptoms or make your symptoms worse. Doing so can reduce the amount of medicine you need to control your asthma. Fast acting Beta 2 antagonist such as Albuterol or Xopenex should be administered as a front line drug. Both come in MDI form as well as nebulized form Long acting bronchodilators such as Serevent may be given once an acute attack is controlled Corticoid steroids such as Flovent, Asthmacort, Beclovent, or Pulmicort may be given as a preventative drug to combat inflammation. Flovent and Serevent together is Advair Mast cell inhibitor drugs and leukotriene inhibitors such as cingular and xolair Cromolyn Sodium- given before exercise for exercise induced asthma Allergy medicine and shots may also help control asthma in some people. Systemic steroids such as Solumederol may be given after an acute attack to reduce inflammation Oral systemic steroids such as Predisone is given as a 3 or 5 day burst following an attack Antibiotics may be given if the attack was caused by a bacterial infection Medicines for Asthma There are two main types of medicines for asthma: Quick-relief medicines—taken at the first signs of asthma symptoms for immediate relief of these symptoms. You will feel the effects of these medicines within minutes. Long-term control medicines—taken every day, usually over long periods of time, to prevent symptoms and asthma episodes or attacks. You will feel the full effects of these medicines after taking them for a few weeks. People with persistent asthma need long-term control medicines. Quick-relief medicines Everyone with asthma needs a quick-relief or "rescue" medicine to stop asthma symptoms before they get worse. Short-acting inhaled beta-agonists are the preferred quick-relief medicine. These medicines are bronchodilators. They act quickly to relax tightened muscles around your airways so that the airways can open up and allow more air to flow through. You should take your quick-relief medicine when you first begin to feel asthma symptoms, such as coughing, wheezing, chest tightness, or shortness of breath. You should carry your quick-relief inhaler with you at all times in case of an asthma attack. Take quick-relief medicines at other times as well—for example, before exercise. Long-term control medicines The most effective, long-term control medicine for asthma is an inhaled corticosteroid (kor-ti-ko-STE-roid) because this medicine reduces the airway swelling that makes asthma attacks more likely. Inhaled corticosteroids (or steroids for short) are the preferred medicine for controlling mild, moderate, and severe persistent asthma. They are generally safe when taken as directed by your doctor. In some cases, steroid tablets or liquid are used for short periods of time to bring asthma under control. The tablet or liquid form may also be used to control severe asthma. Other long-term control medicines include: Inhaled long-acting beta-agonists. These medicines are bronchodilators, or muscle relaxers, not anti-inflammatory drugs. They are used to help control moderate and severe asthma and to prevent nighttime symptoms. Long-acting beta-agonists are usually taken together with inhaled corticosteroid medicines. Pathology Review Page 25 Leukotriene modifiers (montelukast, zafirlukast, and zileuton), which are used either alone to treat mild persistent asthma or together with inhaled corticosteroids to treat moderate or severe asthma. Cromolyn and nedocromil, which are used to treat mild persistent asthma. Theophylline, which is used either alone to treat mild persistent asthma or together with inhaled corticosteroids to treat moderate persistent asthma. People who take theophylline should have their blood levels checked to be sure the dose is appropriate. Use a Peak Flow Meter to assess reversibility of constriction with medication or to assess daily values. A peak flow meter is an assessment tool not a diagnostic tool. SHOULD BE USED WITH ALL SUSPECTED ASTHMATICS! CLINICAL SCENARIO (Asthma): A patient arrives to the ER with notable dyspnea, tachypnea, tachycardia, hypertension and diaphoresis. The RT is called to bedside for the dyspnea and to assess the patient. You listen to the patient’s breath sounds and find that little to no air is moving in/out. You ask if the patient is able to take a deep breath and they shake their head no. They are unable to speak more than a few words at a time. Spo2 on RA shows a saturation of 87%, RR 34, HR 156 and BP 189/89. The patient’s friend tells you they have asthma and was at a BBQ when symptoms began. What is your first response? Since the patient is obviously SOB with impending respiratory failure you must open the airway. Give them full dose of either Xopenex or Albuterol and assess BS post treatment. If you hear increased aeration and wheezing after the treatment you should attempt a peak flow measurement and give another dose of drugs. The drugs should be given on oxygen to increase the SpO2. Keep giving bronchodilators per the hospitals protocol but peak flow should be greater than 80%. Systemic steroids will be given intravenously. If the patient’s BS have not improved and vitals continue to deplete you should think about intubation. This condition would be assessed as Status Asthmaticus. Place the patient in Pressure control, they will need sedation to reduce airtrapping. Give lots of O2, bronchodilators and steroids. Once recovered from initial attack start on a regimen of Q4 txs slowly reducing frequency as symptoms subside. Start on a corticoid steroid and wean off oxygen. They may need bronchial hygiene for secretions and they will defiantly need education in regards to how to control their asthma by avoiding triggers such as smoke from a BBQ. Ventilator modalities: Place patient in PCV if possible do to acute air trapping. Allow plenty of time to exhale; increased flow or reduced I-time. May use inverse ratios for oxygenation problems. Done so on PCV, patient sedated and vitals monitored. Pathology Review Page 26 ARDS What Is ARDS? ARDS, or acute respiratory distress syndrome is a secondary disease process that forms from an infiltrative process. Inflammatory cells migrate through the interstial space and into the lung via reverse osmosis. These inflammatory cells cause non cardiagenic pulmonary edema causing the alveoli to loose stability and collapse. In ARDS, infections, injuries, or other conditions cause the lung's capillaries to leak inflammatory cells into the alveoli. This leads to a severe loss of compliance in the lung inhibiting proper gas exchange leading to organ hypoxia and death. The progression is rapid, leading to respiratory failure. It is both a ventilation and oxygenation problem requiring mechanical ventilation with pressure controlled breaths or low volume breaths and high levels or PEEP and oxygen. What Causes ARDS? Many conditions or factors can directly or indirectly injure the lungs and lead to ARDS. Some common ones are: Sepsis. This is a condition in which An injury to the chest or head, like a bacteria infect the bloodstream. severe blow. Pneumonia. This is an infection in the Breathing in harmful fumes or smoke. lungs. Inhaling vomited stomach contents from the mouth. Severe bleeding due to an injury to the body. It's not clear why some very sick or seriously injured people develop ARDS and others don't. Researchers are trying to find out why ARDS develops and how to prevent it. Direct Lung Injury Conditions that can directly injure the lungs include: Pneumonia. This is an infection in the Using a ventilator. This is a machine lungs. that helps people breathe, but, rarely, it can injure the lungs. Breathing in harmful fumes or smoke. Nearly drowning. Inhaling vomited stomach contents from the mouth. Severe acute respiratory syndrome (SARS). This is a type of pneumonia. Indirect Lung Injury Conditions that can indirectly injure the lungs include: Sepsis. This is a condition in which bacteria infect the bloodstream. Severe bleeding due to an injury to the body or having many blood transfusions. Pathology Review Page 27 An injury to the chest or head, like a severe blow. Pancreatitis. This is a condition in which the pancreas becomes irritated or infected. The pancreas is a gland that releases enzymes and hormones. Fat embolism. This is a condition in which fat blocks an artery. A physical injury, like a broken bone, can lead to a fat embolism. Drug overdose. What Are the Signs and Symptoms of ARDS? The individual is usually severely ill do to the precipitating cause. They will most likely be on the ventilator already. You will notice declining static compliance whilst the peak pressures increase. The patient’s oxygen demand will increase and their respirations will steadily increase. Chest x-ray will begin to show bilateral densities caused by pulmonary edema and collapsed alveoli. Other symptoms include hypotension, confusion from hypoxia and malaise. How Is ARDS Diagnosed? Diagnosed based on medical history and progression of symptoms as well as CXR, ABG, CBC and sputum samples. The patient will have decreased breath sounds with crackles bilaterally. They may present with retractions as their flow demand increases and compliance decreases. Cyanosis is a late sign, as well as hypotension and agonal respirations. Other Tests Other tests used to diagnose ARDS include: Computed tomography (to-MOG-rah-fee), or CT, scan. This test uses a computer to take detailed pictures of your lungs. It may show lung problems, such as fluid in the lungs, signs of pneumonia, or a lung tumor. Heart tests that look for signs of heart failure. Heart failure is a condition in which the heart can't pump blood the way it should. This condition can cause fluid to build up in your lungs. How Is ARDS Treated? ARDS is treated with oxygen, fluids, and medicines. The main goals of treating ARDS are to get oxygen to your lungs and organs (like the brain and kidneys) and treat the underlying condition that's causing ARDS. The patient will have increasing oxygen demands and refractory hypoxemia due to V/Q mismatches from shunts. They will require O2 demands up to 100% with high PEEP levels. Find appropriate peep level, remember with blood loss or sepsis the BP will most likely be low and supported with vasopressors. Mechanical ventilation should be administered so as to prevent excessive PIP’s which may cause pneumothorax or barotraumas. This is done so with pressure control. Other vent strategies include inverse I:E ratio to increase MAP and therefore oxygenation; prone positioning (more alveoli sit in the anterior of chest); High frequency ventilation to prevent lung damage, and nitric oxide to decrease PVR. Fluids Fluids may be given to improve the flow of blood through your body and to provide nutrition. Too much fluid can fill the lungs, making it harder to get the oxygen you need. Not enough fluid can limit blood and oxygen flow to the body's organs. Fluids usually are given through an IV line inserted in one of your blood vessels. Complications from ARDS If you have ARDS, you can develop other medical problems while in the hospital. The most common are infections, pneumothorax, lung scarring, and blood clots. Infections. Being in the hospital and lying down for a long time can make you prone to infections, such as pneumonia. Being on a ventilator also can put you at higher risk for infections. Infections can be treated with antibiotics. Pneumothorax. This is a condition in which air or gas collects in the space around the lungs, which can cause one or both lungs to collapse. The pressure of the air from a ventilator can cause this condition. Your doctor will put a tube into your chest to remove the air and let your lung(s) expand again. Lung scarring. ARDS causes the lungs to become stiff (scarred) and makes it hard for them to expand and fill with air. Being on a ventilator also can cause lung scarring. Often, lung scarring heals before you leave the hospital; but may lead to permanent damage and pulmonary fibrosis. Pathology Review Page 28 Blood clots. Lying down for long periods can cause blood clots to form in your body. A blood clot that forms in a vein deep in your body is called a deep vein thrombosis. This type of blood clot can break off, travel through the bloodstream to the lungs, and block blood flow. This is called pulmonary embolism. Clinical Scenario (ARDS) You are doing a routine ventilator check on a patient with diagnosed sepsis. The patient whom is elderly is tachypneic. The patient is currently on SIMV 12, PSV 10, 40% O2, Vt 550, and flow 60. The patient’s static compliance has dropped from 60 to 35 over the last 12 hours while PIP has risen from 25 to 50 cmH2O. The patient’s SpO2 is 88% on the 40%, Spont RR is 36, sensitivity is 1.0, and patient looks to be pulling (retractions). What do you do, or assess? Obtain an ABG anytime a patient shows significant change of status. Assess oxygenation and ventilation from previous samples. Check latest x-ray. If there isn’t one recommended to the physician that one should be obtained. Note any new infiltrates. At this point you should increase oxygen up to 60% and add PEEP 5 if Spo2 does not improve. You may want to change to Pressure control at this time, setting a pressure limit under 40 (remember the pressure limit doesn’t count the peep!). If you cannot change to PCV recommend to the physician using smaller VT’s and higher rates; also the flow needs to be increased. Change to a control mode, and suggest sedation if BP is stable So…Protect the lung from barotraumas, increase O2 and Peep and increase the rate. Amyotrophic lateral sclerosis (ALS) Sometimes called Lou Gehrig's Disease, or Maladie de Charcot is a progressive, usually fatal, neurodegenerative disease caused by the degeneration of motor neurons, the nerve cells in the central nervous system that control voluntary muscle movement. As one of the motor neuron diseases, the disorder causes muscle weakness and atrophy throughout the body as both the upper and lower motor neurons degenerate and die, ceasing to send messages to muscles. Unable to function, the muscles gradually weaken, develop fasciculations (twitches) because of denervation, and eventually atrophy due to that denervation. The patient may ultimately lose their ability to initiate and control all voluntary movement except of the eyes. Cognitive function is generally spared except in certain situations such as when ALS is associated with frontotemporal dementia. However there are reports of more subtle cognitive changes of the frontotemporal type in many patients when detailed neuropsychological testing is employed. Sensory nerves and the autonomic nervous system, which controls functions like sweating, generally remain functional. Symptoms The onset of ALS may be so subtle that the symptoms are frequently overlooked. The earliest symptoms may include twitching, cramping, or stiffness of muscles; muscle weakness affecting an arm or a leg; and/or slurred and nasal speech. These general complaints then develop into more obvious weakness or atrophy that may cause a physician to suspect ALS. Eventually patients will not be able to stand or walk, get in or out of bed on their own, or use their hands and arms. Difficulty swallowing and chewing impair the patient's ability to eat normally and increase the risk of choking. Maintaining weight will then become a problem. Because the disease usually does not affect cognitive abilities, patients are aware of their progressive loss of function and may become anxious and depressed. As the diaphragm and intercostal muscles (rib cage) weaken, forced vital capacity and inspiratory pressure diminish. In bulbar onset ALS, this may occur before significant limb weakness is apparent. Bilevel positive pressure ventilation (frequently referred to by the tradename BiPAP) is frequently used to support breathing, first at night, and later during the daytime as well. It is recommended that long before BiPAP becomes insufficient, patients (with the eventual help of his/her family) must decide Pathology Review Page 29 whether to have a tracheostomy and long term mechanical ventilation. Most patients do not elect this route, and instead choose palliative hospice care at this point. Most people with ALS die of respiratory failure or pneumonia, not the disease itself. Alpha-1 Antitrypsin Deficiency What Is Alpha-1 Antitrypsin Deficiency? Alpha-1 antitrypsin (an-tee-TRIP-sin) deficiency, or AAT deficiency, is a condition that raises your risk for certain types of lung disease, especially if you smoke. AAT deficiency is an inherited condition. "Inherited" means it's passed in the genes from parents to children.Some people who have severe AAT deficiency develop emphysema often when they're only in their forties or fifties. A smaller number of people who have AAT deficiency have cirrhosis and other serious liver diseases. Cirrhosis is a disease in which the liver is scarred. This prevents the liver from working properly. In people who have AAT deficiency, cirrhosis and other liver diseases usually occur in infancy and early childhood. A very small number of people who have AAT deficiency have a rare type of skin disease called necrotizing panniculitis (pa-NIK-yu-LI-tis). This skin disease can cause painful lumps under or on the surface of the skin. Overview Alpha-1 antitrypsin, also called AAT, is a protein made in the liver. Normally, the protein goes into the bloodstream and helps protect the body's organs from the harmful effects of other proteins. One of the main organs it protects is the lungs. AAT deficiency occurs when the AAT proteins made in the liver aren't the right shape. They get stuck inside liver cells and can't get into the bloodstream. Because not enough AAT protein travels to the lungs to protect them, the risk of lung disease increases. Also, because too many AAT proteins are stuck in the liver, liver disease can develop. AAT deficiency is considered severe when blood levels of the AAT protein fall below the lowest amount needed to protect the lungs. AAT deficiency is an inherited condition caused by altered genes. It's not known how many people have it. Many people who have it may not know they have it. Estimates of how many people have the condition range from about 1 in every 1,600 people to about 1 in every 5,000 people. What Causes Alpha-1 Antitrypsin Deficiency? Altered alpha-1 antitrypsin (AAT) genes cause AAT deficiency. AAT genes tell cells in the body how to make AAT proteins. AAT deficiency occurs when AAT proteins made in the liver aren't the right shape. These proteins get stuck in the liver cells where they are made. They can't get to the organs in the body that they protect, such as the lungs. Without the proteins protecting the organs, diseases can develop. AAT genes are passed from parents to children. The most common altered AAT gene that can cause AAT deficiency is called PiZ. If you inherit two PiZ genes (one from each of your parents), you will have AAT deficiency. If you inherit a PiZ gene from one parent and a normal AAT gene from the other parent, you will not have AAT deficiency. But, you may pass the PiZ gene to your children.Even if you inherit two altered AAT genes, you may not have any related complications. You may never even realize that you have this inherited condition What Are the Signs and Symptoms of Alpha-1 Antitrypsin Deficiency? You may have alpha-1 antitrypsin (AAT) deficiency if you have signs and symptoms of serious lung disease without any obvious cause. Another sign of AAT deficiency is if you develop emphysema at age 45 years or younger. Signs and symptoms of emphysema include: Shortness of breath Wheezing Decreased ability to do physical activity At first, many people who have AAT deficiency are diagnosed with asthma. This is because wheezing is also a symptom of asthma. Also, people who have AAT deficiency respond well to asthma medicines. How Is Alpha-1 Antitrypsin Deficiency Diagnosed? Alpha-1 antitrypsin (AAT) deficiency is usually diagnosed after you develop a lung or liver disease that's linked to AAT deficiency. Because of this, a number of different health care professionals may be involved Pathology Review Page 30 in the diagnosis of AAT deficiency. These include primary care doctors, pulmonologists (lung specialists), and hepatologists (liver specialists). To check whether the disease you have may be related to AAT deficiency: Ask about possible risk factors. A common sign of AAT deficiency is when you have a lung or liver disease without any obvious causes or risk factors. Another is if you have emphysema at an unusually early age (45 years or younger). Ask about your family's medical history. If you have close family members who have AAT deficiency, you're more likely to have the condition. Diagnostic Tests A genetic test is the most certain way to check for AAT deficiency. This test will show whether you have altered AAT genes. A blood test also may be used. This test checks the levels of AAT protein in your blood. If the AAT levels are a lot lower than normal, it's likely that you have AAT deficiency. Lung-Related Tests If you have a lung disease related to AAT deficiency, your doctor may recommend pulmonary function tests and high-resolution computed tomography (CT) scanning. Pulmonary function tests show an obstructive pattern with decreased FEV1 and increased volumes. High-resolution CT scanning uses x rays to create detailed pictures of sections of the body. CT scans show whether you have emphysema and how severe it is How Is Alpha-1 Antitrypsin Deficiency Treated? Alpha-1 antitrypsin (AAT) deficiency has no cure. However, the lung diseases linked to this inherited condition have many treatments. Most of these treatments are the same as the ones given to people who have lung diseases without AAT deficiency. If the patient has emphysema for example the treatment is the same; bronchodilators and low flow oxygen. Flu and pneumococcus (noo-mo-KOK-us) vaccines to protect you from diseases that could make your condition worse. Pulmonary rehabilitation (rehab). This involves treatment by a team of experts at a special clinic. In rehab, you learn how to manage your condition and function at your best. Extra oxygen if needed. A lung transplant. You may need a transplant if your lung is so badly damaged that it severely affects your breathing. If you have a good chance of surviving the transplant surgery, you may be a candidate for it. Augmentation therapy is a type of treatment given only to people who have AAT-related lung diseases. This therapy involves getting infusions of the AAT protein. This raises the level of the protein in your blood and lungs. Not enough research has been done to show whether this type of therapy works. However, some suggest that this therapy may slow the development of AAT deficiency in people who don't have severe disease. Anemia Anemia is a deficiency of red blood cells (RBCs) and/or hemoglobin. This results in a reduced ability of blood to transfer oxygen to the tissues, causing tissue hypoxia. Since all human cells depend on oxygen for survival, varying degrees of anemia can have a wide range of clinical consequences. Hemoglobin (the oxygen-carrying protein in the red blood cells) has to be present to ensure adequate oxygenation of all tissues and organs. The three main classes of anemia include excessive blood loss (acutely such as a hemorrhage or chronically through low-volume loss), excessive blood cell destruction (hemolysis) or deficient red blood cell production (ineffective hematopoiesis).Anemia is the most common disorder of the blood. There are several kinds of anemia, produced by a variety of underlying causes. Anemia goes undetected in many people, and symptoms can be vague. Most commonly, people with anemia report a feeling of weakness or fatigue in general or during exercise, general malaise and sometimes poor concentration. People with more severe anemia often report dyspnea (shortness of breath) on exertion. Very severe anemia prompts the body to compensate by increasing cardiac output, leading to palpitations and sweatiness, and to heart failure. When Anemia is known, relieve the work of the heart by supplying Pathology Review Page 31 high concentrations of oxygen (DO NOT LOOK AT THE SPO2; even if it says 100%, if the hemoglobin is low give the patient oxygen!). Other symptoms include pallor (pale skin, mucosal linings and nail beds) is often a useful diagnostic sign in moderate or severe anemia, but it is not always apparent. Pica, the consumption of non-food such as dirt, paper, wax, grass and hair, may be a symptom of iron deficiency, although it occurs often in those who have normal levels of hemoglobin.Chronic anemia may result from renal failure, as the kidneys produce erthyropoetin; the hormone that stimulates production of RBC’s. Diagnosis Generally, clinicians request complete blood counts in the first batch of blood tests in the diagnosis of an anemia. Apart from reporting the number of red blood cells and the hemoglobin level, the automatic counters also measure the size of the red blood cells by flow cytometry, which is an important tool in distinguishing between the causes of anemia. In modern counters, four parameters (RBC Count, hemoglobin concentration, MCV and RDW) are measured, allowing others (hematocrit, MCH and MCHC) to be calculated, and compared to values adjusted for age and sex. Some counters estimate hematocrit from direct measurements. For adult men, a hemoglobin level less than 13.0 g/dl is diagnostic of anemia, and for adult women, the diagnostic threshold is below 12.0 g/dl. Possible complications Anemia diminishes the capability of individuals who are affected to perform physical activities. This is a result of one's muscles being forced to depend on anaerobic metabolism. The lack of iron associated with anemia can cause many complications, including hypoxemia, brittle or rigid fingernails, cold intolerance, impaired immune function, and possible behavioral disturbances in children. Hypoxemia resulting from anemia can worsen the cardio-pulmonary status of patients with pre-existing chronic pulmonary disease. Brittle or rigid fingernails may be a result of abnormal thinness of nails due to insufficient iron supply. Cold intolerance occurs in one in five patients with iron deficiency anemia, and becomes visible through numbness and tingling. Impaired immune functioning leading to increased likelihood of sickness is another possible complication. Treatments for anemia There are many different treatments for anemia and the treatment depends on severity and the cause.Iron deficiency from nutritional causes is rare in non-menstruating adults (men and postmenopausal women). The diagnosis of iron deficiency mandates a search for potential sources of loss such as gastrointestinal bleeding from ulcers or colon cancer. Mild to moderate iron deficiency anemia is treated by iron supplementation with ferrous sulfate or ferrous gluconate. Vitamin C may aid in the body's ability to absorb iron. Vitamin supplements given orally (folic acid) or subcutaneously (vitamin b-12) will replace specific deficiencies. In anemia of chronic disease, anemia associated with chemotherapy, or anemia associated with renal disease, some clinicians prescribe recombinant erythropoietin, epoetin alfa, to stimulate red cell production.In severe cases of anemia, or with ongoing blood loss, a blood transfusion may be necessary. Aspiration Pneumonia (also see Pneumonia) Aspiration pneumonia is bronchopneumonia that develops due to the entrance of foreign material that enter the bronchial tree, usually oral or gastric contents (including food, saliva, or nasal secretions). Depending on the acidity of the aspirate, a chemical pneumonitis can develop, and bacterial pathogens (particularly anaerobic bacteria) may add to the inflammation. Aspiration pneumonia often develops as a result of an impaired or relaxed airway from such things as stroke, drug overdose or loss of consciousness. Causes Aspiration pneumonia is often caused by an incompetent swallowing mechanism, such as occurs in some forms of neurological disease (a common cause being strokes) or while a person is intoxicated. An iatrogenic cause is during general anaesthesia for an operation and patients are therefore instructed to be nil per os (NPO) for at least four hours before surgery.Whether aspiration pneumonia represents a true bacterial infection or a chemical inflammatory process remains the subject of significant controversy. Both causes may present with similar symptoms. Pathology Review Page 32 Location The location is often gravity dependent, and depends on the patient position. Generally the right middle and lower lung lobes are the most common sites of infiltrate formation due to the larger caliber and more vertical orientation of the right mainstem bronchus. Patients who aspirate while standing can have bilateral lower lung lobe infiltrates. The right upper lobe is a common area of consolidation in alcoholics who aspirate in the prone position. Depending on the acidity of the aspirate, a chemical pneumonitis can develop, and bacterial pathogens (particularly anaerobic bacteria) may add to the inflammation. Treatment Treat pneumonitis with antibiotics and aggressive bronchial hygiene. Prevent aspiration with the placement of a secured airway. Case Scenario (aspiration pneumonia) A patient arrives in the ER unconscious and suspected of overdosing. The RT is called for possible aspiration. Upon assessment the patient has shallow respirations with a rate of 6. Breath sounds reveal coarse rhonchus bilaterally. The patient has no gag reflex. What would you do? Easy. Suction particulate out of oropharynx and then nasotracheally. Intubate for airway, and mechanically ventilate for shallow respirations. Recommend placement of a NG tube, wean to extubate depending on CXR, ABG, and labs. Prepare to sedate if drug reversal is given; or prepare to extubate depending on severity of symptoms. Aspergillus The types of diseases caused by Aspergillus are varied, ranging from an allergy-type illness to lifethreatening generalised infections. Diseases caused by Aspergillus are called aspergillosis. The severity of aspergillosis is determined by various factors but one of the most important is the state of the immune system of the person. Allergic bronchopulmonary aspergillosis (ABPA) This is a condition, which produces an allergy to the spores of the Aspergillus moulds. It is quite common in asthmatics; up to 5% of adult asthmatics might get this at some time during their lives. ABPA is also common in cystic fibrosis patients, as they reach adolescence and adulthood. The symptoms are similar to those of asthma: intermittent episodes of feeling unwell, coughing and wheezing. Some patients cough up brown-colored plugs of mucus. The diagnosis can be made by X-ray or by sputum, skin and blood tests. In the long term ABPA can lead to permanent lung damage (fibrosis) if untreated. The treatment is with steroids by aerosol or mouth (prednisolone), especially during attacks. Itraconazole (an oral antifungal drug) is useful in reducing the amount of steroids required in those needing medium or high doses. This is beneficial as steroids have side-effects like thinning of the bones (osteoporosis) and skin and weight gain, especially when used for a long time. It is not known whether patients with ABPA not on steroids (or on low doses) benefit in some way. Aspergilloma and chronic pulmonary aspergillosis This is a very different disease also caused by the Aspergillus mould. The fungus grows within a cavity of the lung, which was previously damaged during an illness such as tuberculosis or sarcoidosis. Any lung disease which causes cavities can leave a person open to developing an aspergilloma. The spores penetrate the cavity and germinate, forming a fungal ball within the cavity. In some people, cavities in the lung are formed by Aspergillus, and no fungal ball is present. The fungus secretes toxic and allergic products, which may make the person feel ill. The person affected may have no symptoms (especially early on). Weight loss, chronic cough, feeling rundown and tired are common symptoms later. Coughing of blood (haemoptysis) can occur in up to 50-80% of affected people. The diagnosis is made by X-rays, scans of lungs and blood tests. Treatment depends on many factors including whether the patient is coughing blood and how much lung disease there is. Those with no symptoms may need no treatment. Oral itraconazole (usually 400 mg daily) helps symptoms in many patients but rarely kills the fungus in the cavity. A new alternative is voriconazole, which is at least as effective as itraconazole. Sometimes surgical removal is possible, especially if the patient is coughing blood. Surgery is difficult however, and therefore is best reserved for Pathology Review Page 33 single lung cavities. Sometimes other antifungal drugs (especially amphotericin B) can be injected directly into the cavity by a tube, which is put into position under local anaesthesia. *People with weakened immune systems are at highest risk of mortality. Atlectasis Atelectasis is defined as a state in which the lung, in whole or in part, is collapsed or without air. It is a condition where the alveoli are deflated, as distinct from pulmonary consolidation. Caused by multiple factors such as: pulmonary edema, mucus plug, ARDS/RDS, post-surgical atelectasis; tumors, flail chest, pnuemothorax and poor inspiratory effort. Treatment includes positive pressure with IPPB, IPV, Mechanical ventilation, Bronchoscopy, BiPAP, PEEP or with less invasive means such as incentive spirometry, PEP, and EZPAP. Case Scenario (Atlectasis) You are called to a post op bowel resection. The patient is awake and alert with mild inspiratory efforts, complains of pain on inspiration. On the IS ordered patient achieves 500 cc times 10 breaths as her best effort. On room air Spo2 is 88% on a 45 y/o patient with no pulmonary disease. Her cough is weak and non productive. What would you do? This is easy. Check post op chest x-ray. Note atelectasis if any yet. Place on low flow oxygen check Spo2 Q-shift and check breath sounds and cough often. Check breath sounds, should be diminished bibaisler. Switch to more invasive IPPB or BiPAP the I.S. is not cutting it! Pathology Review Page 34 Asbestosis Asbestosis is a chronic inflammatory medical condition affecting the parenchymal tissue of the lungs. It occurs after long-term, heavy exposure to asbestos, e.g. in mining, and is therefore regarded as an occupational lung disease. Sufferers have severe dyspnea (shortness of breath) and are at an increased risk regarding several different types of lung cancer. The primary symptom of asbestosis is generally the slow onset of shortness of breath on exertion. In severe, advanced cases, this may lead to respiratory failure. Coughing is not usually a typical symptom, unless the patient has other, concomitant respiratory tract diseases. People with extensive occupational exposure to the mining, manufacturing, handling or removal of asbestos are at risk of developing asbestosis. There is also an increased risk of lung cancer and mesothelioma. Asbestosis and lung cancer require prolonged exposure to asbestos. However, cases of mesothelioma have been documented with even 1-3 months of exposure, and only indirect exposure (through air ventilation system.) Most cases of asbestosis do not present until 5-10 years after exposure to the material. Pathogenesis Asbestosis is the scarring of lung tissue (around terminal bronchioles and alveolar ducts) resulting from the inhalation of asbestos fibers. When such fibers reach the alveoli in the lung, the foreign bodies (asbestos fibers) cause the activation of the lung's local immune system and provoke an inflammatory reaction. This inflammatory reaction can be described as chronic rather than acute, with a slow ongoing progression of the immune system in an attempt to eliminate the foreign fibres. Macrophages phagocytose (ingest) the fibers and stimulate fibroblasts to deposit connective tissue. Due to the asbestos fibres' natural resistance to digestion, the macrophage will die off, releasing certain cytokines and attracting further lung macrophages and fibrolastic cells to lay down fibrous tissue, which eventually forms a fibrous mass. The result is interstitial fibrosis. The fibrotic scar tissue causes alveolar walls to thicken, which reduces elasticity and gas diffusion, reducing oxygen transfer to the blood as well as the removal of carbon dioxide. Asbestosis presents as a restrictive lung disease. The total lung capacity (TLC) may be reduced through alveolar wall thickening. In the more severe cases, the drastic reduction in lung function due to the stiffening of the lungs and reduced TLC may induce right-sided heart failure (cor pulmonale). Treatment There is no curative treatment. Oxygen therapy at home is often necessary to relieve the shortness of breath. Supportive treatment of symptoms includes respiratory physiotherapy to remove secretions from the lungs by postural drainage, chest percussion, and vibration. Nebulized medications to thin secretions may be prescribed. AIDS HIV / AIDS and Lung Disease / Respiratory Infections Opportunistic infections invade the body of persons with HIV disease. Because the respiratory system is "open" to the environment, taking in air and any infectious material in the air, the lungs are the most susceptible to attack. Opportunistic Lung Diseases and Respiratory Infections Pneumocystis Jiroveci Pneumonia ; formerly known as Pneumocystic Carinii Pneumonia (PCP) This is the most common respiratory infection seen in HIV patients. Tuberculosis (TB) - HIV disease can not only allow for new active tuberculosis infection, but it can also trigger latent tuberculosis (tuberculosis infection) to become active (tuberculosis disease). Mycobacterium Avium Complex (MAC) Bacterial Pneumonia - This is a common killer of HIV positive persons Viral Pneumonia Candiasis - This is a common fungal infection that affects the general population, however it is more severe and more difficult to treat in persons with AIDS Pathology Review Page 35 Pneumocystis pneumonia (PCP) is a form of pneumonia caused by the yeast-like fungus, Pneumocystis jirovecii (Jirovecii is pronounced "yee row vet zee eye"). The causal agent was originally described as a protozoan and spelled P. jiroveci and prior to then was classified as a form of Pneumocystis carinii, a name still in common usage. It is relatively rare in people with normal immune systems but common among people with weakened immune systems, such as premature or severely malnourished children, the elderly, and especially AIDS patients, in whom it is most commonly observed today. PCP can also develop in patients who are taking immunosuppressant medications (e.g. patients who have undergone solid organ transplantation) and in patients who have undergone bone marrow transplantation. Symptoms of PCP include fever, nonproductive cough, shortness of breath (especially on exertion), weight loss and night sweats. There is usually not a large amount of sputum with PCP unless the patient has an additional bacterial infection. The fungus can invade other visceral organs, such as the liver, spleen and kidney, but only in a minority of cases.The risk of pneumonia due to Pneumocystis jirovecii increases when CD4 levels are less than 200 cells/μl. In these immunosuppressed individuals the manifestations of the infection are highly variable. The disease attacks the interstitial, fibrous tissue of the lungs, with marked thickening of the alveolar septa and alveoli and leading to significant hypoxia which can be fatal if not treated aggressively; ergo, LDH levels increase and gas exchange is compromised. Oxygen is less able to diffuse into the blood, leading to hypoxia. Hypoxia, along with high arterial carbon dioxide (CO2) levels, stimulates ventilation, thereby causing dyspnea. Diagnosis The diagnosis can be confirmed by the characteristic appearance of the chest x-ray which shows widespread pulmonary infiltrates, and an arterial oxygen level (pO2) strikingly lower than would be expected from symptoms. The diagnosis can be definitively confirmed by pathologic identification of the causative organism in induced sputum or bronchial washings obtained by bronchoscopy with coloration by toluidine blue or immunofluorescence assay, which will show characteristic cysts. Pneumocystis infection can also be diagnosed by immunofluorescent or histochemical staining of the specimen, and more recently by molecular analysis of PCR products comparing DNA samples. Notably, simple molecular detection of Pneumocystis jirovecii in lung fluids does not mean that a person has Pneumocystis pneumonia or infection by HIV. The fungus appears to be present in healthy individuals also in the general population. * RT treatment usually involves mechanical ventilation; typically the patient is on pressure control as this type of pneumonia often leads to ARDS. Oxygen is high, rates are high, Peep is high and the patient is usually sedated. Nebupent may be given to combat PCP. Obtain frequent ABGS, CBC, CXR and Sputum culture Pathology Review Page 36 Abruptio Placenta Abruptio placentae (ie, placental abruption) refers to separation of the normally located placenta after the 20th week of gestation and prior to birth. Pathophysiology: Bleeding into the decidua basalis leads to separation of the placenta. Hematoma formation further separates the placenta from the uterine wall, causing compression of these structures and compromise of blood supply to the fetus. Retroplacental blood may penetrate through the thickness of the uterine wall into the peritoneal cavity, a phenomenon known as Couvelaire uterus. The myometrium in this area becomes weakened and may rupture with increased intrauterine pressure during contractions. A myometrium rupture immediately leads to a lifethreatening obstetrical emergency. Severity of fetal distress correlates with the degree of placental separation. In near-complete or complete abruption, fetal death is inevitable unless an immediate cesarian delivery is performed. Patients usually present with the following symptoms: Vaginal bleeding 80%; Abdominal or back pain and uterine tenderness - 70%; Fetal distress - 60%; Abnormal uterine contractions (eg, hypertonic, high frequency) - 35%; Idiopathic premature labor - 25%; Fetal death - 15% Physical: Placental abruption is mainly a clinical diagnosis based on findings of vaginal bleeding, abdominal pain, uterine tenderness, uterine contractions, and fetal distress. Severe uterine pain and tenderness with mild vaginal bleeding in a patient with hypertension (HTN) indicates placental abruption. Classification of placental abruption is based on extent of separation (ie, partial vs complete) and location of separation (ie, marginal vs central). Clinical characteristics include the following: Class 0 is asymptomatic. Diagnosis is made retrospectively by finding an organized blood clot or a depressed area on a delivered placenta. Class 1 is mild and represents approximately 48% of all cases. Characteristics include the following: No vaginal bleeding to mild vaginal bleeding; Slightly tender uterus; Normal maternal BP and heart rate; No coagulopathy; No fetal distress Class 2 is moderate and represents approximately 27% of all cases. Characteristics include the following: No vaginal bleeding to moderate vaginal bleeding; Moderate-to-severe uterine tenderness with possible tetanic contractions; Maternal tachycardia with orthostatic changes in BP and heart rate; Fetal distress; Hypofibrinogenemia (ie, 50-250 mg/dL) Class 3 is severe and represents approximately 24% of all cases. Characteristics include the following: No vaginal bleeding to heavy vaginal bleeding; Very painful tetanic uterus; Maternal shock; Hypofibrinogenemia (ie, <150 mg/dL); Coagulopathy; Fetal death Causes: Maternal hypertension - Most common cause of abruption, occurring in approximately 44% of all cases; Maternal trauma (eg, motor vehicle accidents [MVA], assaults, falls) - Causes 1.5-9.4% of all cases; Cigarette smoking; Alcohol consumption; Cocaine use; Short umbilical cord; Sudden decompression of the uterus (eg, premature rupture of membranes, delivery of first twin); Retroplacental fibromyoma; Retroplacental bleeding from needle puncture (ie, postamniocentesis); Advanced maternal age; Idiopathic (probable abnormalities of uterine blood vessels and decidua) Pathology Review Page 37 Prehospital Care: Provide emergency care at the advanced life support (ALS) level to all patients with suspected placental abruption. This care includes the following: Continuous monitoring of vital signs; Continuous, high-flow, supplemental oxygen; One or 2 large-bore IV lines with normal saline (NS) or lactated Ringer (LR) solution; Monitor amount of vaginal bleeding ; Monitoring of fetal heart; Treatment of hemorrhagic shock, if needed Emergency Department Care: ED care depends on stage of gestation and severity of symptoms. Closely observe the patient; Administer supplemental oxygen. Perform fetal monitoring. Administer IV fluids. Perform aggressive fluid resuscitation to maintain adequate perfusion, if needed. Monitor vital signs and urine output. Crossmatch 4 units of packed red blood cells. Transfuse, if necessary. Perform amniotomy to decrease intrauterine pressure, extravasation of blood into the myometrium, and entry of thromboplastic substances into the circulation. Immediately deliver the fetus by cesarean delivery if the mother or fetus becomes unstable. Treatment of coagulopathy or disseminated intravascular coagulation (DIC) may be necessary. Some degree of coagulopathy occurs in about 30% of severe cases of placental abruption. The best treatment for DIC as a complication of placental abruption is immediate delivery. Deterrence/Prevention: Treat maternal hypertension. Prevent maternal trauma/domestic violence. Prevent smoking and substance abuse. Diagnose placental abruption at an early stage in high-risk groups (eg, maternal hypertension, maternal trauma, association with domestic violence, smoking habit, substance abuse, advanced maternal age, premature ruptured membranes, uterine fibromyomas, amniocentesis). Complications: Maternal complications Fetal complications Hemorrhagic shock Hypoxia Coagulopathy/DICUterine Anemia Rupture Growth retardation Renal failure CNS anomalies Ischemic necrosis of distal organs (eg, Fetal death hepatic, adrenal, pituitary) Bronchiectasis What Is Bronchiectasis? Bronchiectasis is a chronic lung disease that usually results from chronic infections or other conditions (such as Cystic Fibrosis) that injures the walls of the airways. This injury is the beginning of a cycle in which your airways slowly lose their ability to clear out mucus by becoming overstretched losing their compliance. The mucus builds up and creates an environment in which bacteria can grow. This leads to repeated serious lung infections. Each infection causes more damage to your airways leading to chronic dilation of a part of the bronchus. The patient becomes a CO2 retainer and has chronic hypoxemia as a result of impaired gas exchange. Bronchiectasis can affect just one section of one of your lungs or many sections of both lungs. It may lead to heart failure as your heart attempts to pump more blood to the lung; over time right heart hypertrophy develops leading to Cor Pulmonale. Bronchiectasis usually begins in childhood, but symptoms may not appear until months or even years after you have started having repeated lung infections. There are two types of Bronchiectasis: Congenital Bronchiectasis usually affects infants and children. It results from a problem in the development of the lungs in the fetus. Acquired Bronchiectasis occurs in adults and older children. It is more common. What Causes Bronchiectasis? Bronchiectasis is caused by injury to the lower airways. This injury may be caused by another disease, including: Pathology Review Page 38 Cystic fibrosis, which leads to almost half of the cases of Bronchiectasis in the United States. Severe pneumonia. Whooping cough (uncommon because most people are now vaccinated against it). Tuberculosis (TB) and other similar infections. Immunodeficiency disorders, such as HIV infection and AIDS. Allergic bronchopulmonary aspergillosis, an allergic reaction to a fungus called aspergillus that causes swelling in the airways. Kartagener's Syndrome, a rare inherited disease that involves the cilia. Other disorders that affect the function of the cilia. Blockage of your airways by a growth or a noncancerous tumor Blockage of your airways by something you inhaled—for example, a piece of a toy or a peanut that you inhaled when you were a child Fungal infections. What Are the Signs and Symptoms of Bronchiectasis? The most common signs and symptoms are: Daily cough, over months or years Weight loss Daily production of large amounts of Fatigue fetid (smelly) mucus Sinus drainage Repeated lung infections (on antibiotics Collapsed lung frequently) Heart failure, if the disease advances to Shortness of breath affect all parts of your airways Wheezing Brain abscess Chest pain (pleurisy) hemoptysis How Is Bronchiectasis Diagnosed? There is no one specific test for Bronchiectasis. Even in its later stages, the signs of the disease are similar to those of other conditions, so those conditions must be ruled out before a diagnosis can be made. Your doctor may suspect Bronchiectasis if you have a daily cough that produces large amounts of mucus. Identify any underlying causes that need to be treated Rule out other causes of your symptoms Determine the amount of damage to your lungs The most commonly used tests to diagnose Bronchiectasis are: Chest x ray. A chest x ray takes a picture of your heart and lungs. It can show infection and scarring of your airway walls. Computed tomography (CT) scan. This test provides a computer generated image of your airways and other tissue in your lungs. It has more detail than a regular chest x ray. A CT scan is the defining test for Bronchiectasis. It can show how much damage has been done to the airways and where the damage is. Blood tests. These tests can show if you have a disease or condition that can lead to Bronchiectasis. They can also show if you have an infection or low levels of certain infectionfighting blood cells. Sputum culture. Sputum contains mucus and often pus, blood, or bacteria. Laboratory tests of a sample of your sputum can show if you have bacteria, fungi, or tuberculosis. PFT showing obstructive disease Sweat test or other tests for cystic fibrosis. How Is Bronchiectasis Treated? The goals of treatment are to: Treat any underlying conditions and respiratory infections Help remove mucus from your lungs Prevent complications Pathology Review Page 39 Early diagnosis and treatment of Bronchiectasis are important. The sooner your doctor can start treating any underlying conditions that may be causing the Bronchiectasis, the better the chances of preventing further damage to your lungs. Medications The main medicines used to treat bronchiectasis are: Antibiotics are the main treatment for the repeated respiratory infections that Bronchiectasis causes. Doctors usually prescribe oral antibiotics to treat these infections. These vary depending on type of infection though Tobramycin is very popular and used for pseudomonas infections. Bronchodilators have minimal affect but are still optimized. Corticosteroids help reduce inflammation in your lungs; used as a maintenance drug. Mucus thinners, such as acetylcysteine, loosen the mucus. Expectorants help loosen the mucus in your lungs. They often come in combination with decongestants, which may provide additional relief. You do not need a prescription for them. Saline nasal washes help control sinusitis. Chest Physical Therapy CPT is also called chest clapping or percussion. It involves pounding your chest and back over and over with your hands or a device to loosen the mucus from your lungs so that you can cough it up. You should do CPT for Bronchiectasis three or four times each day. CPT is often called postural drainage. This means that you sit or lie on your stomach with your head down while you do CPT. This lets gravity and force help drain the mucus from your lungs. Some people find CPT difficult or uncomfortable to do. Several devices have been developed that may help with CPT. The devices include: An electric chest clapper, known as a mechanical percussor. A removable inflatable therapy vest that uses high-frequency air waves to force the mucus that is deep in your lungs toward the upper airways so you can cough it up. A "flutter" device, a small handheld device that you breathe out through. It causes vibrations that dislodge the mucus. A positive expiratory pressure mask that creates vibrations that help break the mucus loose from the airway walls. Several breathing techniques may also help loosen some of the mucus so you can cough it up. These techniques include: Forced expiration technique (FET)—forcing out a couple of breaths or huffs and then doing relaxed breathing Active cycle breathing (ACB)—FET with deep breathing exercises that can loosen the mucus in your lungs Depending on how serious your condition is, your doctor may also recommend: Oxygen therapy. Surgery to remove a section of your lung. Doctors usually do this only if other treatments have not helped and only one part of your lung is affected. If you have major bleeding, your doctor may recommend either surgery to remove the bleeding part of your lung or a procedure to control the bleeding Case Scenario (Bronchiectasis) A patient with Cystic Fibrosis is diagnosed with Bronchiectasis by chest x-ray. The patient developed a productive fetid cough with yellow sputum. The patient has difficulty coughing up sputum; spo2 on room air was 89%, RR 26, BS decreased with bilateral rhonchi. What treatment modality would you place this patient on? Low flow oxygen (remember he has COPD) Obtain sputum C/S, ABG if ventilation is impaired, CXR and CBC Aggressive bronchial hygiene therapy (CPT, PEP/Flutter device with postural drainage) Mucolytics (Dornase Alfa or Mucomyst); Bronchodilators and Antibiotics according to sputum analysis Pathology Review Page 40 Bronchopulmonary Dysplasia Babies who are born prematurely or who experience respiratory problems shortly after birth are at risk for bronchopulmonary dysplasia (BPD), sometimes called chronic lung disease. Although most infants fully recover from BPD and have few long-term health problems as a result, BPD can be a serious condition requiring intensive medical care. A child is not born with BPD. It is something that develops as a consequence of prematurity and progressive lung inflammation; usually secondary to RDS. What Is BPD? Bronchopulmonary dysplasia involves abnormal development of lung tissue. It is characterized by inflammation and scarring in the lungs. It develops most often in premature babies, who are born with underdeveloped lungs (RDS). "Dysplasia" means abnormal changes in the structure or organization of a group of cells. The cell changes in BPD take place in the smaller airways and lung alveoli, making breathing difficult and causing problems with lung function. Along with asthma and cystic fibrosis, BPD is one of the most common chronic lung diseases in children. Children with extremely low birth weight (less than 2.2 pounds or 1,000 grams) are most at risk for developing BPD. Although most of these infants eventually outgrow the more serious symptoms, in rare cases BPD - in combination with other complications of prematurity - can be fatal. What Causes BPD? The majority of BPD cases occur in premature infants, usually those who are born at 34 weeks' gestation or before and weigh less than 4.5 pounds (2,000 grams). These babies are more likely to be affected by a condition known as infant respiratory distress syndrome (RDS) or hyaline membrane disease, which occurs as a result of tissue damage to the lungs from being on a mechanical ventilator for a significant amount of time. Although mechanical ventilation is essential to their survival, over time the pressure from the ventilation and excess oxygen intake can injure a newborn's delicate lungs, leading to RDS. Almost half of all extremely low birth weight infants will develop some form of RDS. If symptoms of RDS persist, then the condition will be considered BPD if a baby is oxygen dependent at 36 weeks' postconceptional age. BPD also can arise from other adverse conditions that a newborn's fragile lungs have difficulty coping with, such as trauma, pneumonia, and other infections. All of these can cause the inflammation and scarring associated with BPD, even in a full-term newborn or, very rarely, in older infants and children. Among babies who are premature and have a low birth weight, white male infants seem to be at greater risk for developing BPD, for reasons unknown to doctors. Genetics may contribute to some cases of BPD as well. Diagnosis and Treatment of BPD Important factors in diagnosing BPD are prematurity, infection, mechanical ventilator dependence, and oxygen exposure. BPD is typically diagnosed if an infant still requires additional oxygen and continues to show signs of respiratory problems after 28 days of age (or past 36 weeks' postconceptional age). Chest Xrays may be helpful in making the diagnosis. In babies with RDS, the X-rays may show lungs that look like ground glass. In babies with BPD, the X-rays may show lungs that appear spongy. No available medical treatment can immediately cure bronchopulmonary dysplasia. Treatment is geared to support the breathing and oxygen needs of infants with BPD and to enable them to grow and thrive. Babies first diagnosed with BPD receive intense supportive care in the hospital, usually in a newborn intensive care unit (NICU) until they are able to breathe well enough on their own without the support of a mechanical ventilator. Some babies also may receive jet ventilation, a continuous low-pressure ventilation that is used to minimize the lung damage from ventilation that contributes to BPD. Not all hospitals use this procedure to treat BPD, but some hospitals with large NICUs do. Infants with BPD are also treated with different kinds of medications that help to support lung function. These include bronchodilators (such as albuterol) to help keep the airways open and diuretics (such as furosemide) to reduce the buildup of fluid in the lungs. Antibiotics are sometimes needed to fight bacterial infections because babies with BPD are more likely to develop pneumonia. Part of a baby's treatment may involve the administration of surfactant, a natural lubricant that improves breathing function. Babies with RDS who have not yet been diagnosed with BPD may have disrupted surfactant production, so administering natural or synthetic Pathology Review Page 41 surfactant may reduce the chance that BPD develops. In addition, babies sick enough to be hospitalized with BPD may need feedings of high-calorie formulas through a gastric tube inserted into the stomach to ensure they get enough calories and nutrients and start to grow.In severe cases, babies with BPD cannot use their gastrointestinal systems to digest food. These babies require intravenous (IV) feedings - called TPN, or total parenteral nutrition - made up of fats, proteins, sugars, and nutrients. These are given through a small tube that is inserted into a large vein through the baby's skin. Even after a baby leaves the hospital, he or she may require continued medication, breathing treatments, or even oxygen at home. Although most children are weaned from supplemental oxygen by the end of their first year, a few with serious cases may need a ventilator for several years or even their entire lives (although this is rare). Improvement for any baby with BPD is gradual. Some infants will be slow to improve; others may not recover from the condition if their lung disease is very severe. Lungs continue to grow for 5-7 years, and there can be subtle abnormal lung function even at school age, although the majority of children function well. Many babies diagnosed with BPD will recover close to normal lung function, but this takes time. Scarred, stiffened lung tissue will always have poor function. However, as infants with BPD grow, new healthy lung tissue can form and grow, and may eventually take over much of the work of breathing for diseased lung tissue. Complications of BPD After coming through the more critical stages of BPD, some infants still have longer-term complications. They are often more susceptible to respiratory infections such as influenza, respiratory syncytial virus (RSV), and pneumonia. When they come down with an infection, they tend to get sicker than most children do. Another respiratory complication of BPD includes excess fluid buildup in the lungs, known as pulmonary edema, which makes it more difficult for air to travel through the airways. Occasionally, kids with a history of BPD may also develop complications of the circulatory system, such as pulmonary hypertension in which the pulmonary arteries - the vessels that carry blood from the heart to the lungs become narrowed and cause high blood pressure. However, this is relatively uncommon and a late complication. Effects of the medications they may need to take include dehydration and low sodium levels from diuretics. Kidney stones, hearing problems, and low potassium and calcium levels can result from long-term furosemide use. Infants with BPD often grow more slowly than other babies and have difficulty gaining weight. They tend to lose weight when they are sick. Premature infants with severe BPD also have a higher incidence of cerebral palsy. (Chronic) Bronchitis 'Bronchitis' is an inflammation of the bronchi in the lungs. Acute bronchitis is usually caused by viruses or bacteria and may last several days or weeks. Chronic bronchitis is not necessarily caused by infection and is generally part of a syndrome called chronic obstructive pulmonary disease (COPD); it is defined clinically as a persistent cough that produces sputum (phlegm) and mucus, for at least three months in two consecutive years. Pathology Review Page 42 Signs and symptoms Bronchitis may be indicated by an expectorating cough (also known as a productive cough), shortness of breath (dyspnea) and wheezing. Occasionally chest pains, fever, and fatigue or malaise may also occur. Mucus is normally green or yellowish green. Symptoms are usually associated with the fact that the patient is not able to generate a lot of expiratory flow due to air trapping from COPD; this mixed with paralyzed and damaged cilia make coughing up sputum difficult (produces “smokers cough” which is a loud frequent hacking cough). Diagnosis A physical examination will often reveal decreased intensity of breath sounds, wheeze (rales) and prolonged expiration. Most doctors rely on the presence of a persistent dry or wet cough as evidence of bronchitis. A variety of tests may be performed in patients presenting with cough and shortness of breath: Pulmonary Function Tests (PFT) (or spirometry) must be performed in all patients presenting with chronic cough. An FEV1/FVC ratio below 0.7 that is not fully reversible after bronchodilator therapy indicates the presence of COPD that requires more aggressive therapy and carries a more severe prognosis than simple chronic bronchitis (though almost all chronic bronchitis is associated with another COPD disease). A chest X-ray that reveals hyperinflation; collapse and consolidation of lung areas would support a diagnosis of pneumonia. Some conditions that predispose to bronchitis may be indicated by chest radiography. A sputum sample showing neutrophil granulocytes (inflammatory white blood cells) and culture showing that has pathogenic microorganisms such as Streptococcus spp. A blood test would indicate inflammation (as indicated by a raised white blood cell count and elevated C-reactive protein). Neutrophils infiltrate the lung tissue, aided by damage to the airways caused by irritation. Damage caused by irritation of the airways leads to inflammation and leads to neutrophils being present Mucosal hypersecretion is promoted by a substance released by neutrophils Further obstruction to the airways is caused by more goblet cells in the small airways. This is typical of chronic bronchitis Although infection is not the reason or cause of chronic bronchitis it is seen to aid in sustaining the bronchitis. Pathology Review Page 43 Treatment Antibiotics For acute exacerbations of chronic bronchitis, if antibiotics are used a meta-analysis found that "amoxicillin/clavulanic acid, macrolides, second-generation or third-generation cephalosporins, and quinolones" may be more effective. Though the underlying cause (smoking) is the real problem., rendering most antibiotics useless. Bronchodilators For acute exacerbations of chronic bronchitis, a clinical practice guideline by the American College of Physicians found that bronchodilators may help; though slightly. Corticosteroids For acute exacerbations of chronic bronchitis, a clinical practice guideline by the American College of Physicians found that corticosteroids may help; again only slightly. Smoking cessation; most effective treatment! In emphysema, chronic inflammation damages the walls of the alveoli — tiny air spaces in your lungs. Chronic bronchitis is a permanent inflammation and thickening of the walls of your bronchial tubes. Eventually, the tubes narrow, causing shortness of breath and a constant cough that brings up large amounts of mucus. Other factors that increase your risk of bronchitis include: Low resistance. This may result from another acute illness, such as a cold, or from a chronic condition that compromises your immune system. Older adults, infants and young children also have greater vulnerability to infection. Gastroesophageal reflux disease (GERD). Stomach acids that persistently back up into your esophagus may cause a chronic cough, usually through a reflex mechanism. Exposure to certain irritants on the job. You run the risk of developing occupational bronchitis if you work around certain lung irritants, such as grains or textiles, or are exposed to chemical fumes from ammonia, strong acids, chlorine, hydrogen sulfide, sulfur dioxide or bromine. The cough associated with occupational bronchitis may be dry (nonproductive). Occupational bronchitis usually clears up when you're no longer exposed to these substances. Pathology Review Page 44 Bronchiolitis Bronchiolitis is a common illness of the respiratory tract caused by an infection that affects the tiny airways, called the bronchioles that lead to the lungs. As these airways become inflamed, they swell and fill with mucus, making breathing difficult. most often affects infants and young children because their small airways can become blocked more easily than those of older kids or adults typically occurs during the first 2 years of life, with peak occurrence at about 3 to 6 months of age is more common in males, children who have not been breastfed, and those who live in crowded conditions Day-care attendance and exposure to cigarette smoke also can increase the likelihood that an infant will develop bronchiolitis. Although it's often a mild illness, some infants are at risk for a more severe disease that requires hospitalization. Conditions that increase the risk of severe bronchiolitis include prematurity, prior chronic heart or lung disease, and a weakened immune system due to illness or medications. Kids who have had bronchiolitis may be more likely to develop asthma later in life, but it's unclear whether the illness causes or triggers asthma, or whether children who eventually develop asthma were simply more prone to developing bronchiolitis as infants. Studies are being done to clarify the relationship between bronchiolitis and the later development of asthma. Bronchiolitis is usually caused by a viral infection, most commonly respiratory syncytial virus (RSV). RSV infections are responsible for more than half of all cases of bronchiolitis and are most widespread in the winter and early spring. Other viruses associated with bronchiolitis include rhinovirus, influenza (flu), and human metapneumovirus. Signs and Symptoms The first symptoms of bronchiolitis are usually the same as those of a common cold: stuffiness runny nose mild cough mild fever These symptoms last a day or two and are followed by worsening of the cough and the appearance of wheezes (high-pitched whistling noises when exhaling). Sometimes more severe respiratory difficulties gradually develop, marked by: rapid, shallow breathing flaring of the nostrils a rapid heartbeat irritability, with difficulty sleeping and signs of fatigue or lethargy drawing in of the neck and chest with each breath, known as retractions The child may also have a poor appetite and may vomit after coughing. Less commonly, babies, especially those born prematurely, may have episodes where they briefly stop breathing (this is called apnea) before developing other symptoms. In severe cases, symptoms may worsen quickly. A child with severe bronchiolitis may tire from the work of breathing and have poor air movement in and out of the lungs due to the clogging of the small airways. The skin can turn blue (called cyanosis), which is especially noticeable in the lips and fingernails. The child also can become dehydrated from working harder to breathe, vomiting, and taking in less during feedings. Contagiousness The infections that cause bronchiolitis are contagious. The germs can spread in tiny drops of fluid from an infected person's nose and mouth, which may become airborne via sneezes, coughs, or laughs, and also can end up on things the person has touched, such as used tissues or toys. Infants in child-care centers have a higher risk of contracting an infection that may lead to bronchiolitis because they're in close contact with lots of other young children. Duration Cases of bronchiolitis typically last about 12 days, but kids with severe cases can cough for weeks. The illness generally peaks on about the second to third day after the child starts coughing and having difficulty breathing and then gradually resolves. Pathology Review Page 45 Professional Treatment Fortunately, most cases of bronchiolitis are mild and require no specific professional treatment. Antibiotics aren't useful because bronchiolitis is caused by a viral infection, and antibiotics are only effective against bacterial infections. Medication may sometimes be given to help open a child's airways. Those who are moderately or severely ill may need to be hospitalized, watched closely, and given fluids and humidified oxygen. Rarely, in very severe cases, some babies are placed on ventilators to help them breathe until they start to get better. *Test for RSV by doing a nasopharngeal wash. To give Ribavirin (anti-viral for RSV) you must give it using the SPAG device to prevent the caregiver from being infected. The Small Particle Aerosol Generator Model-2 (SPAG-2) is indicated for administration of VIRAZOLE®(ribavarin for inhalation solution, USP) only. VIRAZOLE® is indicated in the treatment of carefully selected hospitalized infants and young children with severe lower respiratory tract infections due to respiratory syncytial virus (RSV). Cystic Fibrosis What Is Cystic Fibrosis? Cystic fibrosis (CF) is an inherited disease of your mucus and sweat glands. It affects mostly your lungs, pancreas, liver, intestines, sinuses, and sex organs. Normally, mucus is watery. It keeps the linings of certain organs moist and prevents them from drying out or getting infected. But in CF, an abnormal gene causes mucus to become thick and sticky. The mucus builds up in your lungs causing mucus plugs. This makes it easy for bacteria to grow and leads to repeated serious lung infections as well as atelectasis. Over time, these infections can cause serious damage to your lungs (from inflammation). The thick, sticky mucus can also block tubes, or ducts, in your pancreas. As a result, digestive enzymes that are produced by your pancreas cannot reach your small intestine. These enzymes help break down the food that you eat. Without them, your intestines cannot absorb fats and proteins fully. This causes: Nutrients leave your body unused, and you can become malnourished. Your stools become bulky. You may not get enough vitamins A, D, E, and K. You may have intestinal gas, a swollen belly, and pain or discomfort. The abnormal gene also causes your sweat to become extremely salty. As a result, when you perspire, your body loses large amounts of salt. This can upset the balance of minerals in your blood. The imbalance may cause you to have a heat emergency. CF can also cause infertility (mostly in men). The symptoms and severity of CF vary from person to person. Some people with CF have serious lung and digestive problems. Other people have more mild disease that doesn't show up until they are adolescents or young adults. Respiratory failure is the most common cause of death in people with CF. Other Names for Cystic Fibrosis Pathology Review Page 46 CF Mucoviscidosis of the pancreas Cystic fibrosis of the pancreas Pancreas fibrocystic disease Fibrocystic disease of the pancreas Pancreatic cystic fibrosis Mucoviscidosis What Causes Cystic Fibrosis? Cystic fibrosis (CF) is caused by a defect in a gene called the cystic fibrosis transmembrane conductance regulator (CFTR) gene. This gene makes a protein that controls the movement of salt and water in and out of the cells in your body. In people with CF, the gene does not work effectively. This causes the thick, sticky mucus and very salty sweat that are the main features of CF. Each of us inherits two CFTR genes, one from each parent. Children who inherit an abnormal CFTR gene from each parent will have CF. Children who inherit an abnormal CFTR gene from one parent and a normal CFTR gene from the other parent will not have CF. They will be CF carriers. When two CF carriers have a baby, the baby has a: One in four chance of inheriting two abnormal CFTR genes and having CF. One in four chance of inheriting two normal CFTR genes and not having CF or being a carrier. Two in four chance of inheriting one normal CFTR gene and one abnormal CFTR gene. The baby will not have CF but will be a CF carrier like its parents. Who Is At Risk for Cystic Fibrosis About 30,000 people in the United States have cystic fibrosis (CF). It affects both males and females. It affects people from all racial and ethnic groups but is most common among Caucasians whose ancestors came from northern Europe. CF is one of the most common inherited diseases among Caucasians. What Are the Signs and Symptoms of Cystic Fibrosis? Most of the symptoms of cystic fibrosis (CF) are caused by the thick, sticky mucus. The most common symptoms include: Frequent coughing that brings up thick sputum Frequent bouts of bronchitis and pneumonia. They can lead to inflammation and permanent lung damage. Salty-tasting skin. Pathology Review Page 47 Dehydration. Infertility (mostly in men). Ongoing diarrhea or bulky, foul-smelling, and greasy stools. Huge appetite but poor weight gain and growth. This is called "failure to thrive." It is a result of chronic malnutrition because you do not get enough nutrients from your food. Stomach pain and discomfort caused by too much gas in your intestines. CF can also lead to other medical problems, including: Sinusitis. The sinuses are air-filled spaces behind your eyes, nose, and forehead. They produce mucus and help keep the lining of your nose moist. When the sinuses become swollen, they get blocked with mucus and can become infected. Most people with CF develop sinusitis. Bronchiectasis is a lung disease in which the bronchial tubes, or large airways in your lungs, become stretched out and flabby over time and form pockets where mucus collects. The mucus provides a breeding ground for bacteria. This leads to repeated lung infections. Each infection does more damage to the bronchial tubes. If not treated, bronchiectasis can lead to serious illness, including respiratory failure. Pancreatitis. Pancreatitis is inflammation in the pancreas that causes pain. Episodes of intestinal blockage, especially in newborns. Nasal polyps, or growths in your nose, that may require surgery. Clubbing. Clubbing is the widening and rounding of the tips of your fingers and toes. It develops because your lungs are not moving enough oxygen into your blood stream. Collapsed lung. This is also called pneumothorax. Rectal prolapse. Frequent coughing or problems passing stools may cause rectal tissue from inside you to move out of your rectum. Liver disease due to inflammation or blocked bile ducts. Diabetes. Gallstones. Low bone density because you do not get enough Vitamin D. How Is Cystic Fibrosis Diagnosed? First, your doctor will obtain a detailed medical and family history and perform a thorough physical examination. Next, your doctor may order some tests to ensure an accurate diagnosis. The sweat test is the most useful test for diagnosing cystic fibrosis (CF). It measures the amount of salt in your sweat. For this test, doctors rub a small amount of a chemical called pilocarpine on your arm or leg. They then attach an electrode to this spot. The electrode provides a mild electric current that produces sweat. This may cause tingling or a feeling of warmth. They then cover the area with a gauze pad or filter paper and wrap in plastic. After 30 to 40 minutes, they remove the plastic so the sweat that collected on the pad or paper can be analyzed. The test is usually done twice. High salt levels mean CF. Other tests: Blood tests to look for an abnormal CF PFT’s= obstructive pattern gene or other things that indicate CF. Sputum cultures. Chest x ray. It can show scarring from inflammation in your lungs. Sinus x ray. How Is Cystic Fibrosis Treated? There still is no cure for cystic fibrosis (CF), but treatments have improved greatly in recent years. The goals of CF treatment are to: Prevent and control infections in your Prevent blockages in your intestines. lungs. Provide adequate nutrition. Loosen and remove the thick, sticky mucus from your lungs. Treatment for Lung Problems Antibiotics for infections of the airways Exercise Chest physical therapy Bronchieal hygiene Pathology Review Page 48 Most people with CF have ongoing, low-grade lung infections. Sometimes, these infections become so serious that you may need to be hospitalized. Antibiotics are the primary treatment. You may be given several different types of antibiotics. The choice of antibiotics depends on: The strains of bacteria involved How serious your condition is Your previous history of antibiotic use The different types of antibiotics include: Oral antibiotics for relatively mild airway infections. Inhaled antibiotics, such as tobramycin (to-bra-MI-sin). They may be used alone or with oral antibiotics. Intravenous antibiotics for severe infections or when none of the oral antibiotics work. Antibiotics, such as azithromycin (az-ith-roe-MYE-sin), that also reduce inflammation. Chest physical therapy Chest physical therapy (CPT) is also called chest clapping or percussion. It involves pounding your chest and back over and over again to dislodge the mucus from your lungs so that you can cough up the mucus. CPT for cystic fibrosis should be done three to four times each day. Exercise Aerobic exercise helps: Loosen the mucus Encourage coughing to clear the mucus Improve your overall physical condition If you exercise regularly, you may be able to cut back on your chest therapy. Check with your doctor before doing this. Other medications Anti-inflammatory medications may help reduce the inflammation in your lungs that is caused by ongoing infections. These medications include: Inhaled or, sometimes, oral steroids. Steroids are the most effective anti-inflammatory medicines. Ibuprofen, a type of nonsteroidal, anti-inflammatory medicine. It may slow the progress of CF in young children with mild symptoms. Bronchodilators, which are inhaled drugs that relax the muscles around the airways so that the airways can open up. They should be taken just before CPT to help clear mucus. Mucus-thinning drugs reduce the stickiness of mucus in your airways. They include: Human DNase (Dornase Alfa), a drug that loosens the mucus in your lungs. It may lead to shorter hospital stays. Acetylcysteine and saline. Hypertonic saline, a solution of very salty sterile water taken by nebulizer two times a day, can help clear mucus and improve lung function. Some doctors are now giving it to select patients over 6 years old. Oxygen Therapy If the level of oxygen in your blood is too low, you may need oxygen therapy. Oxygen is usually given through nasal prongs or a mask. Lung Transplantation Surgery to replace one or both of your lungs with healthy lungs from a human donor may help you. Some of the factors that determine whether you can undergo lung transplantation include: The type of bacteria in your lungs Your age and weight The medications you are taking Whether you have other medical conditions, including osteoporosis How well your lungs are functioning Pathology Review Page 49 Management of Digestive Problems Nutritional therapy can improve your growth and development, strength, and exercise tolerance. It may also make you strong enough to resist some lung infections. Nutritional therapy includes a well-balanced, high-calorie diet that is low in fat and high in protein. As part of your nutritional therapy, your doctor may: Prescribe oral pancreatic enzymes to help you digest fats and proteins and absorb more vitamins. The enzymes should be taken in capsule form before every meal, including snacks. Recommend supplements of vitamins A, D, E, and K to replace the fat-soluble vitamins that your intestines cannot absorb. Recommend that you use a feeding tube, called a gastrostomy (gas-TROS-to-me) tube or T-tube, to add more calories at night while you are sleeping. The tube is placed in your stomach. Before you go to bed each night, you attach a bottle with a nutritional solution to the entrance of the tube. It feeds you while you sleep. Other treatments for the digestive problems caused by CF may include: Enemas and mucus-thinning medications to treat intestinal blockages Medicines that reduce stomach acid and help the oral pancreatic enzymes work better *RT intervention: Lots of Bronchial Hygiene. Be aware that these treatments lasts up to 25 minutes each! You are giving a bronchodilator, steroid, antibiotic and mucolytic, plus CPT; flutter/PEP device and breathing exercises. Most CF kids are used to the routine and will be able to manage their own treatments (Most visit CF clinics on a weekly or daily basis). Just be aware of increasing sputum production and declining vitals; and know when to suggest CXR, ABG, CBC and C/S. Croup The term croup does not refer to a single illness, but rather a group of conditions involving inflammation of the upper airway that leads to a cough that sounds like a bark, particularly when a child is crying. Most croup is caused by viruses, but similar symptoms may occasionally be caused by bacteria or an allergic reaction. The viruses most commonly involved are parainfluenza virus (accounting for most cases), adenovirus, respiratory syncytial virus, influenza, and measles. Most children with viral croup are between the ages of 3 months and 5 years old. Croup is most likely to occur during the winter months and early spring, and symptoms are most severe in children younger than 3 years of age. Most croup due to viruses is mild and can be treated at home, though rarely viral croup can be severe and even life-threatening. Some children are more prone to developing croup, especially those who were born prematurely or with narrowed upper airways. The term spasmodic croup refers to a condition similar to viral croup, except that there are no accompanying symptoms of an infection. The cough frequently begins at night with a sudden onset. The Pathology Review Page 50 child usually has no fever with spasmodic croup. The symptoms are treated the same for either form of croup. Signs and Symptoms Croup is characterized by a loud cough that may sound like the barking of a seal and may be accompanied by fast or difficult breathing and sometimes a grunting noise or wheezing while breathing. At first, a child may have cold symptoms like a stuffy or runny nose for a few days and may also have fever. As the upper airway (the lining of the windpipe and the voice box) becomes progressively inflamed and swollen, the child may become hoarse, with a harsh, barking cough. If the upper airway becomes swollen to the point where it is partially blocked off, it becomes even more difficult for a child to breathe. This happens with severe croup. With severe croup, there may be a high-pitched or squeaking noise when breathing in (this is called stridor). A child will tend to breathe very fast, and the stomach or the skin between the child's ribs may seem to pull in during breathing. The child may also appear pale or bluish around the mouth because he is not getting enough oxygen. Symptoms of croup often worsen at night and when the child is upset or crying. In addition to the effects on the upper airway, the infections that cause croup can result in inflammation further down the airway, including the bronchi (breathing tubes) and the lungs. Diagnosis Doctors can usually diagnose croup by looking for the telltale barking cough and stridor, as well as the onset of symptoms. They will also check the child for fever, cold symptoms (like a runny nose), or a recent viral illness, and ask questions to find out if the child has a prior history of croup or upper airway problems. If the child's croup is severe and slow to respond to treatment, a neck X-ray may also be taken to rule out any other reasons for the breathing difficulty, such as a foreign object lodged in the throat or epiglottitis (an inflammation of the epiglottis, the flap of tissue that covers the windpipe). Typical findings on an X-ray if a child has croup includes the top of the airway narrowing (SUPRAGLOTTIC SWELLING) to a point, called a steeple sign. Treatment Most, though not all, cases of viral croup are mild. Breathing in cool mist will relieve many of the symptoms; if only temporarily. Racemic epinephrine may be given to reduce swelling or a steroid may be given. These are temporary fixes, Croup usually only lasts a few days. Case Scenario (Croup) A mother brings her child in to the ER for complaints of cough and fever. Upon assessment you note a barking seal like cough. The patient’s BS are relatively clear though diminished. The patient’s spo2 on room air is 95%, RR 36, and HR 156. The patient is awake/alert and the mother states that the symptoms have been progressing and getting worse for the last 3 days. Pathology Review Page 51 What would you do? This kid is not in any immediate danger. If it were epiglotitis the kid would not be awake and alert. This is what you do: Keep the kid calm, the more worked up he/she is the more they cough Give cool aerosol, room air is fine, unless Spo2 is low. If that doesn’t work give Racemic epi- but note once you give this you have to keep the kid in the ER for at least 90 minutes (most ER’s want these non acute kids out ASAP); or give a steroid like Decadron Tell the mother to buy a humidifier, and that symptoms will reside in a few days, and not to worry! Cerebrovascular Accident (CVA or Stroke) Stroke or cerebrovascular accident (CVA) is the clinical designation for a rapidly developing loss of brain functions due to a disturbance in the blood vessels supplying blood to the brain. This phenomenon can be due to ischemia (lack of blood supply) caused by thrombosis or embolism, or due to a hemorrhage. Stroke is a medical emergency and can cause permanent neurological damage, complications and death if not promptly diagnosed and treated. It is the third leading cause of death and the leading cause of adult disability in the United States and Europe. Risk factors for stroke include advanced age, hypertension (high blood pressure), previous stroke or transient ischaemic attack, diabetes, high cholesterol, cigarette smoking, atrial fibrillation, migraine with aura, and thrombophilia (a tendency to thrombosis). In clinical practice, blood pressure is the most important modifiable risk factor of stroke. Treatment of stroke is occasionally with thrombolysis ("clot buster"), but usually with supportive care (physiotherapy and occupational therapy) and secondary prevention with antiplatelet drugs (aspirin and often dipyridamole), blood pressure control, statins and anticoagulation (in selected patients).[5] Classification Strokes can be classified into two major categories: ischemic and hemorrhagic. Ischemia is due to interruption of the blood supply, while hemorrhage is due to rupture of a blood vessel or an abnormal vascular structure. 80% of strokes are due to ischemia; the remainder is due to hemorrhage. Ischemic stroke In an ischemic stroke, blood supply to part of the brain is decreased, leading to dysfunction and necrosis of the brain tissue in that area. Thrombotic stroke In thrombotic stroke, a thrombus (blood clot) usually forms around atherosclerotic plaques. Since blockage of the artery is gradual, onset of symptomatic thrombotic strokes is slower. A thrombus itself (even if non-occluding) can lead to an embolic stroke (see below) if the thrombus breaks off, at which point it is called an "embolus". Thrombotic stroke can be divided into two types depending on the type of vessel the thrombus is formed on. High risk: atrial fibrillation and paroxysmal atrial fibrillation, rheumatic disease of the mitral or aortic valve disease, artificial heart valves, known cardiac thrombus of the atrium or vertricle, sick sinus syndrome, sustained atrial flutter, recent myocardial infarction, chronic myocardial infarction together with ejection fraction <28 percent, symptomatic congestive heart failure with ejection fraction <30 percent, dilated cardiomyopathy, Libman-Sacks endocarditis, Marantic endocarditis, infective endocarditis, papillary fibroelastoma, left atrial myxoma and coronary artery bypass graft (CABG) surgery Low risk/potential: calcification of the annulus (ring) of the mitral valve, patent foramen ovale (PFO), atrial septal aneurysm, atrial septal aneurysm with patent foramen ovale, left ventricular aneurysm without thrombus, isolated left atrial "smoke" on echocardiography (no mitral stenosis or atrial fibrillation), complex atheroma in the ascending aorta or proximal arch Pathology Review Page 52 Hemorrhagic stroke CT scan showing an intracerebral hemorrhage. Intracranial hemorrhage is the accumulation of blood anywhere within the skull vault. A distinction is made between intra-axial hemorrhage (blood inside the brain) and extra-axial hemorrhage (blood inside the skull but outside the brain). Intra-axial hemorrhage is due to intraparenchymal hemorrhage or intraventricular hemorrhage (blood in the ventricular system). The main types of extra-axial hemorrhage are epidural hematoma (bleeding between the dura mater and the skull), subdural hematoma (in the subdural space) and subarachnoid hemorrhage (between the arachnoid mater and pia mater). Most of the hemorrhagic stroke syndromes have specific symptoms (e.g. headache, previous head injury). Intracerebral hemorrhage (ICH) is bleeding directly into the brain tissue, forming a gradually enlarging hematoma (pooling of blood). It generally occurs in small arteries or arterioles and is commonly due to hypertension, trauma, bleeding disorders, amyloid angiopathy, illicit drug use (e.g. amphetamines or cocaine), and vascular malformations. The hematoma enlarges until pressure from surrounding tissue limits its growth, or until it decompresses by emptying into the ventricular system, CSF or the pial surface. A third of intracerebral bleed is into the brain's ventricles. ICH has a mortality rate of 44 percent after 30 days, higher than ischemic stroke or even the very deadly subarachnoid hemorrhage. Signs and symptoms Stroke symptoms typically develop rapidly (seconds to minutes). The symptoms of a stroke are related to the anatomical location of the damage; nature and severity of the symptoms can therefore vary widely. Ischemic strokes usually only affect regional areas of the brain perfused by the blocked artery. Hemorrhagic strokes can affect local areas, but often can also cause more global symptoms due to bleeding and increased intracranial pressure. On the basis of the history and neurological examination, as well as the presence of risk factors, a doctor can rapidly diagnose the anatomical nature of the stroke (i.e. which part of the brain is affected), even if the exact cause is not yet known. In most cases, the symptoms affect only one side of the body (unilateral). The defect in the brain is usually on the opposite side of the body (depending on which part of the brain is affected). However, the presence of any one of these symptoms does not necessarily suggest a stroke, since these pathways also travel in the spinal cord and any lesion there can also produce these symptoms. Diagnosis Stroke is diagnosed through several techniques: a neurological examination, CT scans (most often without contrast enhancements) or MRI scans, Doppler ultrasound, and arteriography. The diagnosis of stroke itself is clinical, with assistance from the imaging techniques. Imaging techniques also assist in determining the subtypes and cause of stroke. There is yet no commonly used blood test for the stroke diagnosis itself, though blood tests may be of help in finding out the likely cause of stroke. [8] RESPIRATORY INVOLVEMENT: With Ischemic stroke, respiratory involvement depends on the severity of injury. At the very least, the stroke victim with have difficulty swallowing as their epiglottis and vocal cords become either temporarily or permanently paralyzed. This may require the RT to manage the airway for aspiration risks. With hemorrhagic stroke; the RT will most likely be ventilating the patient, as blood in the brain usually Pathology Review Page 53 destroys the rhythmic capabilities of it. You will most likely hyperventilate for the first 24-48 hours to help reduce ICP and swelling until surgery is done or it is determined to be ineffective. These types of injuries may also lead to an impairment of the patient’s cough reflex requiring bronchial hygiene techniques and tracheotomy maintenance. CHF What is congestive heart failure? Congestive heart failure (CHF) is a condition in which the heart's function as a pump to deliver oxygen rich blood to the body is inadequate to meet the body's needs. Congestive heart failure can be caused by 1) diseases that weaken the heart muscle, 2) diseases that cause stiffening of the heart muscles, or 3) diseases that increase oxygen demand by the body tissue beyond the capability of the heart to deliver. Many diseases can impair the pumping action of the ventricles. For example, the muscles of the ventricles can be weakened by heart attacks or infections (myocarditis). The diminished pumping ability of the ventricles due to muscle weakening is called systolic dysfunction. Diseases such as hemochromatosis can cause stiffening of the heart muscles and impair the ventricles' capacity to relax and fill. This is referred to as diastolic dysfunction. Additionally, in some patients, although the pumping action and filling capacity of the heart may be normal, abnormally high oxygen demand by the body's tissues (for example, in patients with hyperthyroidism) may make it difficult for the heart to supply an adequate blood flow (called high output heart failure). Congestive heart failure can affect many organs of the body. For example, the weakened heart muscles may not be able to supply enough blood to the kidneys, which then begin to lose their normal ability to excrete salt (sodium) and water. This diminished kidney function can cause to body to retain more fluid. The lungs may become congested with fluid (pulmonary edema) and the person's ability to exercise is decreased. Fluid may likewise accumulate in the liver, thereby impairing its ability to rid the body of toxins and produce essential proteins. The intestines may become less efficient in absorbing nutrients and medicines. Over time, untreated congestive heart failure will affect virtually every organ in the body. What causes congestive heart failure? Many disease processes can impair the pumping efficiency of the heart to cause congestive heart failure. In the United States, the most common causes of congestive heart failure are coronary artery disease, high blood pressure (hypertension), longstanding alcohol abuse, and disorders of the heart valves. Less common causes include viral infections of the stiffening of the heart muscle, thyroid disorders, disorders of the heart rhythm, and many others. It should also be noted that in patients with underlying heart disease, taking certain medications can lead to the development or worsening of congestive heart failure. This is especially true for those drugs that can cause sodium retention or affect the power of the heart muscle. Examples of such medications are the commonly used nonsteroidal antiinflammatory drugs (NSAIDs), which include ibuprofen (Motrin and others) and naproxen (Aleve and others) as well as certain steroids, some diabetic medication, and some calcium channel blockers. What are the symptoms of congestive heart failure? The symptoms of congestive heart failure vary among individuals according to the particular organ systems involved and depending on the degree to which the rest of the body has "compensated" for the heart muscle weakness. An early symptom of congestive heart failure is fatigue. While fatigue is a sensitive indicator of possible underlying congestive heart failure, it is obviously a nonspecific symptom that may be caused by many other conditions. The person's ability to exercise may also diminish. Patients may not even sense this decrease and they may subconsciously reduce their activities to accommodate this limitation. As the body becomes overloaded with fluid from congestive heart failure, swelling (edema) of the ankles and legs or abdomen may be noticed. In addition, fluid may accumulate in the lungs, thereby causing shortness of breath, particularly during exercise and when lying flat. In some instances, patients are awakened at night, gasping for air. Some may be unable to sleep unless sitting upright. The extra fluid in the body may cause increased urination, particularly at night. Accumulation of fluid in the liver and intestines may cause nausea, abdominal pain, and decreased appetite Pathology Review Page 54 Medications Angiotensin Converting Enzyme (ACE) Inhibitors These medications block the formation of angiotensin II, a hormone with many potentially adverse effects on the heart and circulation in patients with heart failure. Possible side effects of these drugs include a nagging, dry cough, low blood pressure, worsening kidney function and electrolyte imbalances, and rarely, true allergic reactions. Examples of ACE inhibitors include captopril (Capoten), enalapril (Vasotec), lisinopril (Zestril, Prinivil), benazepril (Lotensin), and ramipril (Altace). For those patients who are unable to tolerate the ACE inhibitors, an alternative group of drugs, called the angiotensin receptor blockers (ARBs), may be used. These drugs act on the same hormonal pathway as the ACE inhibitors, but instead block the action of angiotensin II at its receptor site directly. Examples of this class of medications include losartan (Cozaar), candesartan (Atacand), telmisartan (Micardis), valsartan (Diovan), and irbesartan (Avapro). Beta–blockers Certain hormones, such as epinephrine (adrenaline), norepinephrine, and other similar hormones, act on the beta receptor's of various body tissues and produce a stimulative effect. The effect of these hormones on the beta receptors of the heart is a more forceful contraction of the heart muscle. Beta–blockers are agents that block the action of these stimulating hormones on the beta receptors of the body's tissues. Beta–blockers can also generally not be used in people with certain significant diseases of the airways (e.g., asthma, emphysema) or very low resting heart rates. While carvedilol (Coreg) has been the most thoroughly studied drug in the setting of congestive heart failure (and remains the only beta–blocker with FDA approval for the treatment of congestive heart failure). Digoxin Digoxin stimulates the heart muscle to contract more forcefully. It also has other actions, which are incompletely understood, that improve congestive heart failure symptoms and can prevent further heart failure. However, a recent large–scale randomized study failed to demonstrate any effect of digoxin on mortality. It is useful for many patients with significant congestive heart failure symptoms, even though long term survival may not be affected. Potential side effects include nausea, vomiting, heart rhythm disturbances, kidney dysfunction, and electrolyte abnormalities. These side effects, however, are generally a result of toxic levels in the blood and can be monitored by blood tests. The dose of digoxin may also need to be adjusted in patients with significant kidney impairment. Diuretics Diuretics are often an important component of the treatment of congestive heart failure to prevent or alleviate the symptoms of fluid retention. These drugs help keep fluid from building up in the lungs and other tissues by promoting the flow of fluid through the kidneys. Diuretics remain key in preventing deterioration of the patient's condition thereby requiring hospitalization. When hospitalization is required, diuretics are often administered intravenously because the ability to absorb oral diuretics may be impaired. Potential side effects of diuretics include dehydration, electrolyte abnormalities, particularly low potassium levels, hearing disturbances, and low blood pressure. Examples of various classes of diuretics include furosemide (Lasix), hydrochlorothiazide, bumetanide (Bumex), torsemide (Demadex), and metolazone (Zaroxolyn). BIPAP= used to displace pulmonary edema caused by CHF. Fluid is forced to the walls of the airways allowing for increased surface area and better gas exchange. BiPAP is used until diuretics are able to remove excess fluid thus avoiding the need for invasive mechanical ventilation. Bronchodilators have no effect on pulmonary edema and associated wheezing; crackles will be noted with CHF as well as cardiac wheezes. Pathology Review Page 55 Case Scenario (CHF) You arrive to ER for a patient in respiratory distress. The 56 y/o patient is on a NRB mask with a SPO2 of 90%, RR 34, BS bilateral crackles on inspiration; pink frothy secretions are protruding from the patient’s mouth; BP is elevated; patient is diaphoretic, has dyspnea and is very orthopneic. Patient is unable to speak due to SOB; upon quick medical hx you note patient has a record of CHF and has been non compliant with their lassix at home. What do you do? Well you know what’s wrong with the patient. The patient is on a NRB and only saturating 90%, they are tachypneic and BS reveals pulmonary edema. Grab a BiPAP immediately and place on general settings of lets say 15/5; rate 12, 100% and adjust per patient need. Obtain a ABG about an hour after BiPAP. If the patient looses consciousness you may consider intubation. Breathing treatments will do nothing for this patient unless an underlying pulmonary problem exists. However, the doc will probably order them anyways. All it will do is add more flow which is good, but will increase HR, which is not good. Suggest lassix and wean off BiPAP as tolerated. Coronary Artery Disease (CAD) What Is Coronary Artery Disease? Coronary artery disease (CAD) is a condition in which plaque builds up inside the coronary arteries. These arteries supply your heart muscle with oxygen-rich blood. Plaque is made up of fat, cholesterol calcium, and other substances found in the blood. When plaque builds up in the arteries, the condition is called atherosclerosis. Atherosclerosis Figure A shows a normal artery with normal blood flow. Figure B shows an artery with plaque buildup. Plaque narrows the arteries and reduces blood flow to your heart muscle. It also makes it more likely that blood clots will form in your arteries. Blood clots can partially or completely block blood flow. Pathology Review Page 56 Overview When your coronary arteries are narrowed or blocked, oxygen-rich blood can't reach your heart muscle. This can cause angina or a heart attack. Angina is chest pain or discomfort that occurs when not enough oxygen-rich blood is flowing to an area of your heart muscle. Angina may feel like pressure or squeezing in your chest. The pain also may occur in your shoulders, arms, neck, jaw, or back. A heart attack occurs when blood flow to an area of your heart muscle is completely blocked. This prevents oxygen-rich blood from reaching that area of heart muscle and causes it to die. Without quick treatment, a heart attack can lead to serious problems and even death. Over time, CAD can weaken the heart muscle and lead to heart failure and arrhythmias. Heart failure is a condition in which your heart can't pump enough blood throughout your body. Arrhythmias are problems with the speed or rhythm of your heartbeat. What Causes Coronary Artery Disease? Research suggests that coronary artery disease (CAD) starts when certain factors damage the inner layers of the coronary arteries. These factors include: Smoking High amounts of certain fats and cholesterol in the blood High blood pressure High amounts of sugar in the blood due to insulin resistance or diabetes When damage occurs, your body starts a healing process. Excess fatty tissues release compounds that promote this process. This healing causes plaque to build up where the arteries are damaged. Over time, the plaque may crack. Blood cells called platelets (PLATE-lets) clump together to form blood clots where the cracks are. This narrows the arteries more and worsens angina or causes a heart attack. The buildup of plaque in the coronary arteries may start in childhood. Over time, this buildup can narrow or completely block some of your coronary arteries. This reduces the flow of oxygen-rich blood to your heart muscle. Respiratory Involvement: Be aware that CAD is the primary cause of MI. You will need to supply O2 to these patients to take the workload off the heart. You will also do frequent EKG’s to assess cardiac rhythm. If a MI does occur they may undergo a CABG thus involving respiratory directly as after surgery they will be on a ventilator. Cor Pulmonale Cor pulmonale is a medical term used to describe a change in structure and function of the right ventricle of the heart as a result of a respiratory disorder. Right ventricular hypertrophy or RVH is the predominant change in chronic cor pulmonale although in acute cases dilation dominates. Both hypertrophy and dilation are the result of increased right ventricular pressure. Dilation is essentially a stretching of the ventricle, the immediate result of increasing the pressure in an elastic container. Ventricular hypertrophy is an adaptive response to a long-term increase in pressure. Additional muscle grows to allow for the increased contractile force required to move the blood against greater resistance.To be classified as cor pulmonale, the cause must originate in the pulmonary circulation system. Two major causes are vascular changes as a result of tissue damage (e.g. disease, hypoxic injury, chemical agents etc.), and chronic hypoxic pulmonary vasoconstriction. RVH due to a systemic defect is not classified as cor pulmonale. Left untreated, cor pulmonale can lead to right-heart failure and death. Causes Acute: o Massive pulmonary o Exacerbation of chronic cor embolization pulmonale Chronic: o COPD o Pierre Robin Sequence o Loss of lung tissue following o End stage Pneumoconiosis trauma or surgery Pathology Review Page 57 Treatment Elimination of the cause is the most important intervention. Diuretics for RVF, In pulmonary embolism, thrombolysis (enzymatic dissolution of the blood clot) is advocated if there is dysfunction of the right ventricle. In COPD, long-term oxygen therapy may improve cor pulmonale. Cor pulmonale may lead to congestive heart failure (CHF), with worsening of respiration due to pulmonary edema, swelling of the legs due to peripheral edema and painful congestive hepatomegaly (enlargement of the liver due to tissue damage as explained in the Complications section. This situation requires diuretics (to decrease strain on the heart), sometimes nitrates (to improve blood flow), phosphodiesterase inhibitors such as sildenafil or tadalafil and occasionally inotropes (to improve heart contractility). CHF is a negative prognostic indicator in cor pulmonale. Congenital Diaphragmatic Hernia What is a diaphragmatic hernia? A diaphragmatic hernia is a birth defect, which is an abnormality that occurs before birth as a fetus is forming in the mother's uterus. An opening is present in the diaphragm (the muscle that separates the chest cavity from the abdominal cavity). With this type of birth defect, some of the organs that are normally found in the abdomen move up into the chest cavity through this abnormal opening. Pathology Review Page 58 There are two types of diaphragmatic hernia: Bochdalek hernia A Bochdalek hernia involves an opening on the left side of the diaphragm. The stomach and intestines usually move up into the chest cavity. Morgagni hernia A Morgagni hernia involves an opening on the right side of the diaphragm. The liver and intestines usually move up into the chest cavity. What causes a diaphragmatic hernia? As a fetus is growing in its mother's uterus before birth, different organ systems are developing and maturing. The diaphragm forms between the 7th and 10th week of pregnancy. The esophagus (the tube that leads from the throat to the stomach), the stomach, and the intestines are also developing at this time. In a Bochdalek hernia, the diaphragm may not develop properly, or the intestine may become trapped in the chest cavity as the diaphragm is forming. In a Morgagni hernia, the tendon that should develop in the middle of the diaphragm does not develop properly. In both cases, normal development of the diaphragm and the digestive tract does not occur. Diaphragmatic hernia is a multifactorial condition, which means that "many factors," both genetic and environmental, are involved. It is thought that multiple genes from both parents, as well as a number of environmental factors that scientists do not yet fully understand, contribute to diaphragmatic hernia. In cases where it is the only health problem in a baby, the chance for diaphragmatic hernia to happen again in a future pregnancy is two percent or 2 in 100 chances. This means that there is a 98 percent chance that it would not be seen in a future pregnancy. Why is a diaphragmatic hernia of concern? The lungs are developing at the same time as the diaphragm and the digestive system. A diaphragmatic hernia allows abdominal organs to move into the chest cavity, instead of remaining in the abdomen as they are developing. With the heart, lungs, and abdominal organs all taking up space in the chest cavity, the lungs do not have space to develop properly. This underdevelopment of the lungs is called pulmonary hypoplasia. A diaphragmatic hernia is a life-threatening illness. When the lungs do not develop properly during pregnancy, it can be difficult for the baby to breathe after birth. Healthy lungs have millions of small air sacs (alveoli), which resemble a balloon filled with air. With pulmonary hypoplasia: there are fewer air sacs than normal. the air sacs that are present are only able to partially fill with air. the air sacs deflate easily due to a lack of a lubricating fluid called surfactant. When these conditions are present, the baby is unable to take in enough oxygen to stay healthy. The intestines also may not develop properly, especially if they are not receiving enough blood supply while they are developing. A good blood supply is necessary for the intestines to develop correctly, and to be healthy and function properly. What are the symptoms of a diaphragmatic hernia? The symptoms of a Bochdalek diaphragmatic hernia are often observable soon after the baby is born. The following are the most common symptoms of a Bochdalek diaphragmatic hernia. However, each child may experience symptoms differently. Symptoms may include: difficulty breathing abnormal chest development, with one side being larger than the other fast breathing abdomen that appears caved in fast heart rate (concave) cyanosis (blue color of the skin ) A baby born with a Morgagni hernia may or may not show any symptoms.The symptoms of diaphragmatic hernia may resemble other conditions or medical problems. Always consult your baby's physician for a diagnosis. How is a diaphragmatic hernia diagnosed? After birth, your baby's physician will perform a physical examination. A chest x-ray is done to look at the abnormalities of the lungs, diaphragm, and intestine. A blood test known as an arterial blood gas is often performed to evaluate the baby's breathing ability. Other tests that may be performed include: Pathology Review Page 59 blood test for chromosomes (to determine if there is a genetic problem) ultrasound of the heart (echocardiogram) Treatment for a diaphragmatic hernia: Specific treatment will be determined by your baby's physician based on the following: when the problem is diagnosed (during your baby's tolerance for specific pregnancy or after birth) medications, procedures, or therapies your baby's overall health and medical your opinion or preference history the severity of the problem Treatment may include: neonatal intensive care A diaphragmatic hernia is a life-threatening illness and requires care in a neonatal intensive care unit (NICU). Babies with diaphragmatic hernia are often unable to breathe effectively on their own because their lungs are underdeveloped. Most babies will need to be placed on a breathing machine called a mechanical ventilator to help their breathing. ECMO Some infants may need to be placed on a temporary heart/lung bypass machine called ECMO if they have severe problems. ECMO does the job that the heart and lungs would be doing: putting oxygen in the bloodstream and pumping blood to the body. ECMO may be used temporarily while a baby's condition stabilizes and improves. surgery When the baby's condition has improved, the diaphragmatic hernia will be repaired with an operation. The stomach, intestine, and other abdominal organs are moved from the chest cavity back to the abdominal cavity. The hole in the diaphragm is repaired. Many babies will need to remain in the NICU for a while after surgery. Although the abdominal organs are now in the right place, the lungs still remain underdeveloped. The baby will usually need to have breathing support for a period of time after the operation. Once the baby no longer needs help from a breathing machine (ventilator), he/she may still need oxygen and medications to help with breathing for weeks, months, or years. Cardiac Tamponade Cardiac tamponade is the compression of the heart caused by blood or fluid accumulation in the space between the myocardium (the muscle of the heart) and the pericardium (the outer covering sac of the heart). Causes In this condition, blood or fluid collects within the pericardium. This prevents the ventricles from expanding fully, so they cannot adequately fill or pump blood. Cardiac tamponade is often associated with pericarditis caused by bacterial or viral infections. Heart surgery, dissecting aortic aneurysm (thoracic), wounds to the heart, end-stage lung cancer, and acute MI can all lead to cardiac tamponade. Other potential causes include heart tumors, kidney failure, recent heart attack, recent open heart surgery, recent invasive heart procedures, radiation therapy to the chest, hypothyroidism and systemic lupus erythematosus. Symptoms o Radiating to the neck, shoulder, back or abdomen Anxiety, restlessness o Sharp, stabbing Discomfort, sometimes relieved by o Worsened by deep breathing sitting upright or leaning forward. or coughing Difficulty breathing Swelling of the abdomen or other areas Rapid breathing Skin pale, gray or blue Fainting, light-headedness Palpitations Chest pain Pulse, weak or absent Pathology Review Page 60 Drowsiness Low blood pressure Dizziness Exams and Tests Although there are no specific laboratory tests that diagnose tamponade, echocardiogram is first choice to help establish the diagnosis. The heart will often sound uncharacteristically faint during examination with a stethoscope. Peripheral pulses may be weak or absent. Neck veins may be distended but the blood pressure may be low.The heart rate may be over 100 (normal is 60 to 100 beats per minute), and breathing may be rapid (faster than 12 breaths in an adult per minute). The blood pressure may fall (pulsus paradoxical) when the person inhales deeply. Fluid in the pericardial sac may show on: o Chest x-ray o Echocardiogram o Chest CT or MRI of chest o Coronary angiography (may show other changes also) ECG changes include low voltage wave forms Treatment Cardiac tamponade is an emergency condition that requires hospitalization. Treatment is aimed at saving the patient's life, improving heart function, relieving symptoms, and treating the tamponade. This is usually accomplished with pericardiocentesis (a procedure to drain the fluid around the heart) or by cutting and removing part of the pericardium (pericardial window). Fluids are the initial treatment to maintain normal blood pressure until pericardiocentesis can be performed. Medications that increase blood pressure may also help sustain the patient's life until the fluid is drained. The patient may be given oxygen, this reduces the workload on the heart by decreasing tissue demands for blood flow. The cause of the tamponade must be identified and treated. Treatment of the cause may include medications, such as antibiotics, and surgical repair of injury. Cutting or removing part of the pericardium (surgical pericardiectomy, also known as pericardial window) may be required for most tamponade patients. Central Sleep Apnea Central sleep apnea is when you stop breathing during sleep. It is caused by problems with how the brain controls breathing. This is not the more common obstructive sleep apnea, which is caused by obesity or other problems and involves loud snoring. Central sleep apnea usually occurs in people who are seriously ill. For example, it can occur in people with a variety of severe and life-threatening lower brain stem lesions. The brainstem controls breathing. As a result, any disease or injury affecting this area may result in problems with normal breathing during sleep or when awake. Conditions that can cause central sleep apnea include: Bulbar poliomyelitis Encephalitis affecting the brainstem Neurodegenerative illnesses Stroke affecting the brainstem Other causes include complications of cervical spine surgery, secondary radiation in the region of the cervical spine, severe arthritis and degenerative changes in the cervical spine or the base of the skull, or primary hypoventilation syndrome. There is a form of central sleep apnea that commonly occurs in people with congestive heart failure. Finally, idiopathic central sleep apnea is when the apnea is not associated with another disease. Symptoms The primary symptom is when you temporarily stop breathing, especially during sleep. People with apnea of unknown cause may awaken frequently and complain of insomnia. If a neurological condition is causing the apnea, it may also produce other symptoms. These include difficulty swallowing, change in voice, Pathology Review Page 61 weakness, or numbness throughout the body, depending on the underlying disease and what parts of the nervous system it has affected. Exams and Tests Lung and breathing studies All-night polygraphic sleep monitoring MRI Tests to diagnose an underlying medical condition Treatment If central sleep apnea is due to heart failure, the goal is to treat the heart failure itself. In the case of idiopathic apnea or brainstem problems, nasal CPAP may be used to allow breathing. Choanal Artresia Choanal atresia is a narrowing or blockage of the nasal airway by tissue. It is a congenital condition, meaning it is present at birth. Causes The cause of choanal atresia is unknown, but is thought to occur when the thin tissue seperating the nasal and oral spaces during fetal development persists. The condition is the most common nasal abnormality seen in the newborn infant, affecting about 1 in 7,000 live births. Newborns with this condition are known as an "obligate nose breathers." This means the baby must breathe through its nose because the oral airway is not yet developed enough to allow for frequent mouth breathing. In fact, almost the only time an infant does not breathe through its nose is when crying. Choanal atresia may be affect one or both sides of the nasal airway. It is often associated with other congenital problems. Choanal atresia blocking both sides (bilateral) of the nose causes acute breathing problems with cyanosis and breathing failure. Infants with bilateral choanal atresia may need resuscitation at delivery. Blockage on only one side causes less severe problems. Choanal atresia is generally recognized shortly after birth while the infant is still in the hospital. Symptoms Difficulty breathing following birth, which may result in cyanosis (bluish discoloration), unless infant is crying Inability to nurse and breathe at same time Chest retraction unless breathing through mouth or crying Inability to pass a catheter through each side of the nose into the throat Persistent one-sided nasal blockage or discharge Exams and Tests A physical examination may show an obstruction of the nose. Tests that may be done include: CT Endoscopy of the nose Sinus x-ray Treatment The immediate concern is to resuscitate the baby if necessary. An airway may need to be placed so that the infant can breathe. In some cases, intubation or tracheostomy may be needed. An infant can learn to mouth breathe, which can delay the need for immediate surgery. Surgery to remove the obstruction cures the problem. It may be delayed if the infant can tolerate mouth breathing. Possible surgical approaches include through the nose (transnasal) and through the mouth (transpalatal). Coarctation of the Aorta Aortic coarctation is a narrowing of part of the aorta (the major artery leading the heart). It is a type of birth defect. Coarctation means narrowing. Causes The aorta carries blood from the heart to the vessels that supply the body with blood and nutrients. If part of the aorta is narrowed, it is hard for blood to pass through the artery. People with this condition Pathology Review Page 62 often have high blood pressure in the upper body and arms (or one arm) and low blood pressure in the lower body and legs. Aortic coarctation is more common in persons with certain genetic disorders, such as Turner's syndrome. However, it can also be due to birth defects of the aortic valve, such as a bicuspid aortic valve. Aortic coarctation occurs in approximately 1 out of 10,000 people. It is usually diagnosed in children or adults under age 40. Symptoms Symptoms depend on how much blood can flow through the artery. In severe cases, symptoms are seen when the baby is very young. In milder cases, symptoms may not develop until the child has reached adolescence. Symptoms include: Dizziness or fainting Leg cramps with exercise Shortness of breath Hypertension (high blood pressure) with exercise Pounding headache Decreased ability to exercise Chest pain Cold feet or legs Nosebleed Exams and Tests The health care provider will perform a physical exam and take your blood pressure in your arms and legs. Your pulse will be checked. The pulse in the femoral (groin) area is weaker than the carotid (neck) pulse. Sometimes, the femoral pulse may not be felt at all. The doctor will use a stethoscope to listen to your heart and check for murmurs. People with aortic coarctation have a harsh murmur that can be heard from the back. Coarctation is often discovered during a newborn's first examination or a well-baby exam. Taking the pulses in an infant is an important part of the examination since there may not be any other symptoms or findings until the child is older. Tests to diagnose this condition may include: Chest x-ray Chest CT ECG MRI of the chest Echocardiography Cardiac catheterization and aortography Doppler ultrasound of the aorta Both Doppler ultrasound and cardiac catheterization can be used to see if there are any differences in blood pressure in different areas of the aorta. Treatment Surgery is usually recommended. The narrowed part of the aorta will be removed. If the problem area was small, the two free ends of the aorta may be re-connected. This is called anastomosis. If a large part of the aorta was removed, a Dacron graft (a synthetic material) is used to fill the gap In some cases, balloon angioplasty may be done instead of surgery. Pathology Review Page 63 Carbon Monoxide Posioning Carbon monoxide poisoning occurs after the inhalation of carbon monoxide gas. Carbon monoxide is colorless, odorless, tasteless, and non-irritating, making it difficult for people to detect.Carbon monoxide is a significantly toxic gas with poisoning being the most common type of fatal poisoning in many countries. Symptoms of mild poisoning include headaches, vertigo, and flu-like effects; larger exposures can lead to significant toxicity of the central nervous system and heart. Carbon monoxide can also have severe effects on the fetus of a pregnant woman. The mechanisms by which carbon monoxide produces toxic effects are not yet fully understood, but hemoglobin, myoglobin, and mitochondrial cytochrome oxidase are thought to be compromised. Treatment largely consists of administering 100% oxygen or hyperbaric oxygen therapy. Common sources of CO that may lead to poisoning include house fires, furnaces or heaters, wood-burning stoves, motor vehicle exhaust, and propane-fueled equipment such as portable camping stoves. Symptoms The earliest symptoms, especially from low level exposures, are often non-specific and readily confused with other illnesses, typically flu-like viral syndromes, depression, chronic fatigue syndrome, and migraine or other headaches. This often makes the diagnosis of carbon monoxide poisoning difficult. If suspected, the diagnosis can be confirmed by measurement of blood carboxyhemoglobin. The main manifestations of poisoning develop in the organ systems most dependent on oxygen use: the central nervous system and the heart. The clinical manifestations include tachycardia and hypertension, and central nervous system symptoms such as headache, dizziness, confusion, convulsions, and unconsciousness. CO poisoning may also produce myocardial ischemia, atrial fibrillation, pneumonia, pulmonary edema, hyperglycemia, muscle necrosis, acute renal failure, skin lesions, visual and auditory problems, and respiratory arrest. One of the major concerns following CO poisoning is the severe neurological manifestations that may occur days or even weeks after an acute poisoning. Common problems encountered are difficulty with higher intellectual functions and short-term memory, dementia, irritability, gait disturbance, speech disturbances, parkinson-like syndromes, cortical blindness, and depression (depression can occur in those accidentally exposed). Levels of oxygen available for tissue use are decreased. This situation is described as CO shifting the oxygen dissociation curve to the left. Blood oxygen content is actually increased in the case of carbon monoxide poisoning; because all the oxygen is in the blood, none is being given to the tissues, and this causes tissue hypoxic injury. However, despite CO affecting oxygen availability, other mechanisms may contribute to the crucial effects of CO poisoning. A sufficient exposure to carbon monoxide can reduce the amount of oxygen taken up by the brain to the point that the victim becomes unconscious, and can suffer brain damage or even death from hypoxia. The Pathology Review Page 64 brain regulates breathing based upon carbon dioxide levels in the blood, rather than oxygen levels, so a victim can succumb to hypoxia without ever noticing anything up to the point of collapse. Hemoglobin acquires a bright red color when converted to carboxyhemoglobin, so a casualty of CO poisoning is described in textbooks as looking pink-cheeked and healthy. However, this "classic" cherryred appearance is not always seen. *PLACE PATIENT ON 100% O2 and watch for signs of burns. Obtain a ABG with Co-OX. Diabetes Mellitus Diabetic Ketoacidosis Diabetic ketoacidosis is a complication of diabetes. It is caused by the buildup of by-products of fat breakdown, called ketones. This occurs when glucose is not available as a fuel source for the body, and fat is used instead. Causes People with diabetes lack enough insulin, a hormone the body uses to process glucose (a simple sugar) for energy. When glucose is not available, body fat is broken down instead. The by-products of fat metabolism are ketones. When fat is metabolized, ketones build up in the blood and "spill" over into the urine. A condition called ketoacidosis develops when the blood becomes more acidic than body tissues. Blood glucose levels rises (usually higher than 300 mg/dL) because the liver produces glucose to try to combat the problem, but the cells cannot take up that glucose without insulin. Diabetic ketoacidosis may lead to the initial diagnosis of type 1 diabetes, as it is often the first symptom that causes the person to come to medical attention. It can also be the result of increased insulin needs in someone already diagnosed with type 1 diabetes. Infection, trauma, heart attack, or surgery can lead to diabetic ketoacidosis in such cases. People with type 2 diabetes usually develop ketoacidosis only under conditions of severe stress. Not following the prescribed diet and treatment is usually the cause when episodes are repeated. ymptoms Frequent urination or frequent thirst for a day or more Fatigue Nausea and vomiting Muscular stiffness or aching Mental stupor that may progress to coma Rapid breathing Exams and Tests Low blood pressure Rapid heart rate Signs of dehydration High blood glucose (above 300 mg/dL) Fruity breath (breath odor) Headache Decreased consciousness Breathing difficulty while lying down Low blood pressure Decreased appetite Abdominal pain Presence of glucose and ketones in urine by home or office testing Serum potassium (may be elevated) Serum amylase (may be elevated) Arterial blood gas (reveals pH of less than 7.3) This disease may also alter the results of the following tests: Urine pH Serum phosphorus Sodium - urine Serum magnesium Serum sodium CSF collection Potassium - urine CO2 Treatment The goal of treatment is to correct the elevated blood glucose level by giving additional insulin, and to replace fluids lost through excessive urination and vomiting. A person with diabetes may be able to Pathology Review Page 65 recognize the early warning signs and make appropriate corrections at home, before the condition progresses. If ketoacidosis is severe, hospitalization is required to control the condition. Insulin replacement will be given, fluid and electrolytes will be replaced, and the cause of the condition (such as infection) will be identified and treated. * Get a ABG to assess Bicarb, patient will be breathing fast, you do not need to give oxygen, their oxygen will be high. No other respiratory intervention is needed. Suggest giving Bicarb then Insulin. Eclampsia Eclampsia is the occurence of seizures (convulsions) in a pregnant woman. The seizures are unrelated to brain conditions and usually happen after the 20th week of pregnancy. See also: Preeclampsia Causes The cause of eclampsia is not well understood. Researchers believe a person's genes, diet, blood vessels, and neurological factors may play a role. However, no theories have yet been proven. Eclampsia follows preeclampsia, a serious complication of pregnancy marked by high blood pressure, weight gain, and protein in the urine. It is difficult to predict which women with preeclampsia will go on to have seizures. Women with very high blood pressure, headaches, vision changes, or abnormal blood tests have severe preeclampsia and are at high risk for seizures. The rate of eclampsia is approximately 1 out of 2000 to 3000 pregnancies. The following increase a woman's chance for preeclampsia: First pregnancies Multiple pregnancies (twins, triplets, etc.) Teenage pregnancies History of diabetes, hypertension, or Being 35 or older renal (kidney) disease Being African-American Symptoms Seizures Unconsciousness Severe agitation Muscle aches and pains Symtoms of preeclampsia include swelling of hands and face, gaining more than 2 pounds per week, headache, vision problems, and stomach pain. See preeclampsia. Exams and Tests The health care provider will perform a physical exam and rule out other possible causes of seizures. Blood pressure and breathing rate will be checked and monitored. Blood tests may be performed to check: Uric acid Creatinine (to evaluate kidney function) Liver function Platelet count Treatment A woman with eclampsia should be continously monitored. Delivery is the treatment of choice for eclampsia in a pregnancy over 28 weeks. For pregnancies less than 24 weeks, the start of labor is recommended, although the baby may not survive. Prolonging pregnancies in which the woman has eclampsia results in danger to the mother and infant death in approximately 87% of cases. Women may be given medicine to prevent seizures (anticonvulsant). Magnesium sulfate is a safe drug for both the mother and the baby. Medication may be used to lower the high blood pressure. The goal is to manage severe cases until 32-34 weeks and mild cases until 36 weeks of the pregnancy have passed. The condition is then relieved with the delivery of the baby. Delivery may be induced if blood pressure stays high despite medication. *Important due to the fact that woman must deliver early= means you will have to deal with a premature baby with premature lungs! Pathology Review Page 66 Empyema Empyema is a collection of pus in the cavity between the lung and the membrane that surrounds it (pleural space). Causes Empyema is caused by an infection that spreads from the lung and leads to an accumulation of pus in the pleural space. The infected fluid can build up to a quantity of a pint or more, which puts pressure on the lungs, causing shortness of breath and pain. Risk factors include recent pulmonary (lung) conditions including bacterial pneumonia, lung abscess, thoracic surgery, trauma or injury to the chest, or rarely, a needle inserted through the chest wall to draw off fluid in the pleural space (thoracentesis). Symptoms Dry cough General discomfort, uneasiness, or ill feeling (malaise) Fever and chills Unintentional weight loss Excessive sweating, especially night sweats Chest pain, which worsens on deep inhalation (inspiration) Exams and Tests The health care provider may note abnormal findings, such as decreased breath sounds or a friction rub, when listening to the chest with a stethoscope (auscultation). Tests may include the following: Chest x-ray Pleural fluid gram stain and culture Thoracentesis CT scan of chest Treatment The goal of treatment is to cure the infection and remove the collection of pus from the lung. Antibiotics are prescribed to control the infection. A doctor will place a chest tube to completely drain the pus. A surgeon may need to perform a procedure to peel away the lining of the lung (decortication) if the lung does not expand properly. End Stage Renal Disease End-stage kidney disease is a complete or near complete failure of the kidneys to function to excrete wastes, concentrate urine, and regulate electrolytes. Also called End-stage renal disease (ESRD). Causes End-stage kidney disease occurs when the kidneys are no longer able to function at a level that is necessary for day to day life. It usually occurs as chronic renal failure worsens to the point where kidney function is less than 10% of normal. At this point, the kidney function is so low that without dialysis or kidney transplantation, complications are multiple and severe, and death will occur from accumulation of fluids and waste products in the body. The kidney is responsible for maintenance of electrolytes, blood pressure, pH balance, stimulation of RBC production and waste removal. Kidney failure will lead to fluid retention causing heart complications; it will cause metabolic acidosis from build up of uremic acid, it will cause anemia from inability to produce ethryopoetin, it will cause muscle fatigue from electrolyte imbalance and hypertension. Symptoms Unintentional weight loss Greatly decreased urine output Nausea or vomiting No urine output General ill feeling Easy bruising or bleeding Fatigue May have blood in the vomit or stools Headache Decreased alertness o drowsiness, somnolence, Frequent hiccups lethargy Generalized itching Pathology Review Page 67 o confusion, delirium Skin may appear yellow or brown o coma Nail abnormalities Muscle twitching or cramps Decreased sensation in the hands, feet, Seizures or other areas Increased skin pigmentation Exams and Tests The patient usually has a long history of chronic kidney failure, which has progressed. The person may have required dialysis to control chronic renal failure. The urine volume may decrease or urine production may stop totally. Signs of complications commonly are present. Creatinine and BUN levels are chronically high. Creatinine clearance is very low. Treatment Dialysis or kidney transplantation are the only treatments for ESRD. The physical condition of the person and other factors determines which of these is used. Other treatments of chronic kidney failure may continue but are unlikely to work without dialysis or transplantation. Current therapy includes aggressive treatment of high blood pressure with an ACE inhibitor or an angiotensin receptor blocker. Associated diseases that cause or result from chronic renal failure must be controlled. Hypertension (high blood pressure), congestive heart failure, urinary tract infections, kidney stones, obstructions of the urinary tract, glomerulonephritis, and other disorders should be treated. Blood transfusions and medications such as iron and erythropoietin may be needed to control anemia. Fluids may be restricted to an amount nearly equal to the volume of urine produced. Dietary restrictions may slow the build-up of wastes in the bloodstream and control associated symptoms such as nausea and vomiting. Restrictions include a lowprotein diet, with high carbohydrate levels to make up for the lost calories. Salt, potassium, phosphorus, and other electrolytes may be restricted. Ventilator modalities: Watch for fluid overload and pulmonary edema. This may require the use of PEEP. If sepsis if present, you may switch to pressure control as Sepsis often leads to ARDS. Epiglottitis Epiglottitis is inflammation of the cartilage that covers the trachea (windpipe); characterized by subglottic swelling. An acute condition requiring immediate response by placement of an airway. Causes Epiglottitis is a life-threatening disease. The epiglottis is the piece of cartilage at the back of the tongue that closes off the windpipe when swallowing. Without an epiglottis, food could enter the airways, and you would cough or choke after swallowing. Epiglottitis causes swelling of the epiglottis. Breathing problems increases rapidly as the epiglottis swells. Epiglottitis can get rapidly worse.Inflammation of the epiglottis is usually caused by the bacteria Haemophilus influenzae (H. influenzae), although it may be caused by other bacteria or viruses. Upper respiratory infections can lead to epiglottitis. Medicines or Pathology Review Page 68 diseases that weaken the immune system can make adults more prone to epiglottitis. Epiglottitis is most common in children between 2 and 6 years old. Rarely, epiglottitis can occur in adults, and it may be easily overlooked in such patients.The occurrence of epiglotti has decreased steadily in the United States since the H. influenzae type B (Hib) vaccine became a routine childhood immunization in the late 1980s. Symptoms Epiglottitis begins with a high fever and sore throat. Other symptoms may include: Drooling Voice changes (hoarseness) Difficulty swallowing Chills, shaking Difficulty breathing (patient may need Cyanosis (blue skin coloring) to sit upright and lean slightly forward to breathe) Noisy breathing (stridor) Exams and Tests Epiglottitis is a medical emergency. Seek immediate medical help. Do not use a tongue depressor (tongue blade) to try to examine the throat at home, as this may make the condition worse. The health care provider will examine the larynx (voice box) using either a small mirror held against the back of the throat or a viewing tube called a laryngoscope. This procedure, called laryngoscopy, may show a swollen and red epiglottis. Anesthesia may be needed to do a laryngoscopy if a breathing tube is necessary. Tests used to diagnose epiglottitis may include: Blood culture or throat culture -- may show H. influenzae or other bacteria CBC -- may show a high number of white blood cells Neck x-rays -- may show a swollen epiglottis Treatment The patient will be admitted to the hospital, usually an intensive care unit (ICU). Treatment may include methods to help the patient breathe, including: Humidified oxygen (oxygen that has been moistened) Breathing tube ( intubation) Other treatments may include: Fluids given by IV Antibiotics to treat the infection Corticosteroids to decrease the swelling of the throat Case Scenario (Epiglottitis) An 2 year old arrives in the ER with sudden onset of barking cough/stridor. Upon assessment the child is lethargic with shallow respirations, drooling and desaturating on room air. What do you do? This kid is not here for Croup. The onset of symptoms was sudden (within a few hours), he is lethargic and breathing badly. INTUBATE NOW! Don’t waste time getting a neck x-ray or doing a ABG. Try not to stimulate child and insert a airway ASAP either by ETT or trach. Once on vent check cuff pressures frequently and get neck x-ray, extubate once swelling goes down. Give steroids for swelling. Pathology Review Page 69 Ventilator Modalities: No specific ventilator setting is required. Be sure to check cuff pressure of ETT frequently to assess if swelling is subsiding or not (this is also done with burn victims). Emphysema Emphysema is caused by loss of elasticity (increased compliance) of the lung tissue, from destruction of structures supporting the alveoli, and destruction of capillaries feeding the alveoli. The result is that the small airways collapse during exhalation (although alveolar collapsibility has increased), leading to an obstructive form of lung disease (airflow is impeded and air is generally "trapped" in the lungs in obstructive lung diseases). Symptoms include shortness of breath on exertion (typically when climbing stairs or inclines, and later at rest), hyperventilation, and an expanded chest. Emphysema patients are sometimes referred to as "pink puffers". This is because emphysema sufferers may hyperventilate to maintain adequate blood oxygen levels. Hyperventilation explains why mild emphysema patients do not appear cyanotic as chronic bronchitis (another COPD disorder) sufferers often do; hence they are "pink puffers" (able to maintain almost normal blood gases through hyperventilation and not "blue bloaters" (cyanosis; inadequate oxygen in the blood). However, any severely chronically obstructed (COPD) respiratory disease will result in hypoxia (decreased blood partial pressure of oxygen) and hypercapnia (increased blood partial pressure of Carbon Dioxide), called Blue Bloaters. Blue Bloaters are so named as they have almost normal ventilatory drive (due to decreased sensitivity to carbon dioxide secondary to chronic hypercapnia), are plethoric (red face/cheeks due to a polycythemia secondary to chronic hypoxia) and cyanotic (due to decreased hemoglobin saturation). Clinical signs Clinical signs at the fingers include cigarette stains (although actually tar) and asterixis (metabolic flap) at the wrist if they are carbon dioxide retainers. Examination of the face reveals a ruddy complexion (if there is a secondary polycythemia), pursed-lipped breathing, and central cyanosis. Examination of the chest reveals increased percussion notes (particularly over the liver) and a difficult to palpate apex beat (all due to hyperinflation), decreased breath sounds, audible expiratory wheeze, as well as signs of fluid overload (seen in advanced disease) such as pitting peripheral edema. The patient will have a “barrel chest” and prolonged expiratory time. The patient will complain of SOB and have different grades of dyspnea according to disease progression. Diagnosis Diagnosis is by spirometry (lung function testing), including diffusion testing. Findings will often demonstrate a decrease in FEV1 but an increase in Total Lung Capacity (TLC). Diffusion tests such as DLCO will show a decreased diffusion capacity. Other investigations might include X-rays, high resolution spiral chest CT-scan, bronchoscopy (when other lung disease is suspected, including malignancy), blood tests, and increased pulse. It might also be under the category of Alpha-1 Antitrypsin Deficiency, AAT. A way to help AAT is to put more into the blood flow and eat more protein. Pathophysiology Pathology of lung showing centrilobular emphysema characteristic of smoking. When toxins such as smoke are breathed into the lungs, the particles are trapped and cause a localized inflammatory response. Chemicals released during the inflammatory response can break down the walls of alveoli. This leads to significant modifications of lung architecture, characterized mainly by intrapulmonary air collections and confining parenchyma collapse; as far as the process proceeds expiratory bronchial obstruction and thoracic cage expansion also occur, with the clinical picture of COPD. Pathology Review Page 70 Mainly decreased is the ability to exude carbon dioxide due to ventilation deficit and, in the more serious cases, oxygen uptake is also impaired. The activity of another molecule called alpha 1-antitrypsin normally neutralizes the destructive action of one of these damaging molecules. After a prolonged period, hyperventilation becomes inadequate to maintain high enough oxygen levels in the blood. The body compensates by vasoconstricting appropriate vessels. This leads to pulmonary hypertension, which places increased strain on the right side of the heart, the one that pumps deoxygenated blood to the lungs, and it often fails. The failure causes the heart muscle to thicken to pump more blood. Eventually, as the heart continues to fail, it becomes larger and blood backs up in the liver. Prognosis and treatment Emphysema is an irreversible degenerative condition. The most important measure that can be taken to slow the progression of emphysema is for the patient to stop smoking and avoid all exposure to cigarette smoke and lung irritants. Pulmonary rehabilitation can be very helpful to optimize the patient's quality of life and teach the patient how to actively manage his or her care. Emphysema is also treated by supporting the breathing with anticholinergics, bronchodilators and (inhaled or oral) steroid medication, and supplemental oxygen as required. Lung volume reduction surgery (LVRS) can improve the quality of life for certain carefully selected patients. It can be done by several different methods, some of which are minimally invasive. The only known "cure" for emphysema is a lung transplant, although few patients are strong enough physically to survive the surgery. The combination of a patient's age, oxygen deprivation and the side-effects of the medications used to treat emphysema cause damage to the kidneys, heart and other organs. Transplants also require the patient to take an anti-rejection drug regime which suppresses the immune system possibly leading to microbial infection of the patient. Emphysema can be classified into two types: Primary and Secondary. Primary emphysema can be further subdivided into panacinary and centroacinary types. Panacinary emphysema is related to the destruction of alveoli, because of an inflammation or deficiency of alfa-1-antitripsine. This type of emphysema is found more in young adults that do not have chronic bronchitis. Centroacinary emphysema is related with destruction of terminal bronchioli muchosis, as a result of chronic bronchitis. This is found mostly in elderly people with a long history of smoking. Clinical Scenario You are called to ER to assess a patient with SOB and productive cough. You immediately recognize the patient as they are frequent flyer at your facility. The patient is a known to have COPD. Assessment reveals: HR 122, RR 24, SpO2 on RA 86%, BS expiratory wheezes, decreased and mild rhonchi. The patient has a barrel chest, clubbed fingers, and flattened diaphragms on CXR. What might you do to relieve this patient’s SOB? Apply low flow oxygen with NC or high flow with low concentrations with a Venturi mask, you may give a bronchodilator and anticholinergic; you might instruct the patient on pursed lip breathing and diaphragmatic breathing. Order QID or Q4 treatments until symptoms are closer to baseline. Obtain an ABG for CO2 and O2 assessment. Ventilate as needed with Bipap according to Co2 level. Obtain a CXR, CBC, EKG and C/S. Have the patient do a PFT once more stable to assess progression on COPD. Possible Ventilator modalities: Supply plenty of flow if on VC. Use >60 LPM to assure proper exhalation time. Use PEEP to augment autopeep. Note changes with PIP, patient’s with COPD have the tendency to blow blebs and have spontaneous pneumothorax due to the over stretched alveoli. Adjust sensitivity to meet patient’s flow demand. Do not over correct the patient’s ABG; before weaning make sure patient’s ABG is close to their normal base line. In other words do not try to blow off retainers CO2 with excessive ventilation. Endocarditis This is caused by an inflammation of the inner lining of the heart from bacteria. The bacterium often enters the circulation from contaminated needles from drug abusers. As the valves of the heart do not actually receive any blood supply of their own, defense mechanisms (such as white blood cells) cannot Pathology Review Page 71 enter. So if an organism (such as bacteria) establishes a hold on the valves, the body cannot get rid of them. The bacteria can destroy valves leading to blood regurgitation and pulmonary edema. The RT intervention would come in the form of positive pressure for severe pulmonary edema and oxygen as well as EKG and assessment of ventilation. Flail Chest A flail chests occurs when a segment of the thoracic wall breaks under extreme stress and becomes detached from the rest of the chest wall. It is a serious, life-threatening chest injury often associated with underlying pulmonary injury and is most commonly seen in cases of significant blunt trauma. This typically occurs when three or more ribs are fractured in two or more places, allowing that segment of the thoracic wall to displace and move independently of the rest of the chest wall. Flail chest can also occur when ribs are fractured proximally in conjunction with disarticulation of costochondral cartilages distally. For the condition to occur, generally there must be a significant force applied over a large surface of the thorax to create the multiple anterior and posterior rib fractures. Rollover and crushing injuries most commonly break ribs at only one point– for flail chest to occur a significant impact is required, breaking the ribs in two or more places. Presentation The characteristic paradoxical motion of the flail segment occurs due to pressure changes associated with respiration that the rib cage normally resists: During normal inspiration, the diaphragm contracts and intercostal muscles push the rib cage out. Pressure in the thorax decreases below atmospheric pressure, and air rushes in through the trachea. However, a flail segment will not resist the decreased pressure and will appear to push in while the rest of the rib cage expands. During normal expiration, the diaphragm and intercostal muscles relax, allowing the abdominal organs to push air upwards and out of the thorax. However, a flail segment will also be pushed out while the rest of the rib cage contracts. The constant motion of the ribs in the flail segment at the site of the fracture is exquisitely painful, and, untreated, the sharp broken edges of the ribs are likely to eventually puncture the pleural sac and lung, which may be fatal. Treatment Good analgesia including intercostal blocks, avoiding narcotic analgesics as much as possible. This allows much better ventilation, with improved tidal volume, and increased blood oxygenation. Positive ventilation, meticulosly adjusting the ventilator settings to avoid barotrauma. Chest tubes as required. Adjustment of position to make the patient most comfortable and provide relief of pain. Flail Chest with paradoxical breathing Pathology Review Page 72 Foreign Body Airway Obstruction (FBAO) Acute upper airway obstruction may have any of a number of causes including viral and bacterial infections, fire or inhalation burns, chemical burns and reactions, allergic reactions, foreign bodies, and trauma. The blockage can be complete or partial. Mild obstruction may result in air hunger, while more severe obstruction can lead to cyanosis, confusion, or unconsciousness. Complete obstruction, if not corrected, leads to rapid suffocation and death. Symptoms of acute upper airway obstruction are dramatic and easy to diagnose. The individual may suddenly begin having difficulty breathing or be unable to breathe at all. Cyanosis, unconsciousness, and death may rapidly follow a large or total obstruction. Common causes of acute upper airway obstruction include: Foreign bodies -- In adults, foreign bodies are usually inhaled bits of food. In children, peanuts are the most commonly inhaled food, but hot dogs are the most common food item associated with choking deaths, because of their size and shape. Children also commonly choke on many nonfood items, such as balloon fragments, buttons, coins, and small toys. Acute allergic reactions in which the trachea or throat swell closed are a common cause of airway obstruction. The classic example is an allergic reaction to a bee sting, which frequently takes the form of an anaphylactic reaction. Other common causes include allergy reaction to peanuts, antibiotics (penicillin), and blood pressure medications (ACE inhibitors). Croup may lead to a fairly rapid decrease in airway diameter and marked respiratory distress. Epiglottitis can cause rapid airway obstruction as the epiglottis swells and occludes the airway. Retropharyngeal abscess is a rapidly expanding abscess can block the airway and cause respiratory distress. Peritonsillar abscess is similar to retropharyngeal abscess. Symptoms vary depending on the cause, but some symptoms are common to all types of obstruction. Choking Panic Gasping for air Cyanosis Wheezing, crowing, whistling, or other Changes in consciousness unusual breathing noises indicating Unconsciousness breathing difficulty Agitation or fidgeting Exams and Tests Physical examination may show decreased breath sounds. Tests are usually not necessary, but may include x-rays, bronchoscopy, and laryngoscopy. Treatment If the victim has a complete obstruction and is unable to speak or breathe, the Heimlich maneuver may be lifesaving. Treatment depends on the cause of the blockage. Objects lodged in the airway may be removed with a laryngoscope or bronchoscope. A tube may be inserted into the airway (endotracheal tube or nasotracheal tube). Sometimes an opening is made directly into the airway (tracheostomy or cricothyrotomy). Guillain- Barre Syndrome Guillain-Barre syndrome is a serious disorder that occurs when the body's defense (immune) system mistakenly attacks part of the nervous system. This leads to nerve inflammation that causes muscle weakness, which continues to get worse. Guillain-Barre syndrome is an autoimmune disorder. The exact cause of Guillain-Barre syndrome is unknown. The syndrome may occur at any age, but is most common in people of both sexes between the ages 30 and 50. It often follows a minor infection, usually a respiratory infection or gastrointestinal infection. Usually, signs of the original infection have disappeared before the symptoms of Guillain-Barre begin. Guillain-Barre syndrome causes inflammation that damages parts of nerves. This nerve damage causes tingling, muscle weakness, and paralysis beginning in the legs progressing upward in the body (Ground to Brain). The inflammation usually affects the nerve's covering (myelin sheath). Such damage is called demyelination. Demyelination slows nerve signaling. Damage to other parts of the nerve can cause the nerve to stop working. Pathology Review Page 73 Guillain-Barre syndrome may occur along with viral infections such as: Mononucleosis AIDS Herpes simplex It may also occur with other medical conditions such as systemic lupus erythematosus or Hodgkin's disease. Some people may get Guillain-Barre syndrome after a bacterial infection, certain vaccinations (such as rabies and swine flu), and surgery. Symptoms Symptoms of Guillain-Barre get worse very quickly. It may take only a few hours to reach the most severe symptoms. Muscle weakness or the loss of muscle function (paralysis) affects both sides of the body. If the muscle weakness starts in the legs and then spreads to the arms, it is called ascending paralysis. Patients may notice tingling, foot or hand pain, and clumsiness. As the loss of muscle function gets worse, the patient may need breathing assistance. o In mild cases, weakness or Muscle weakness or loss of muscle paralysis may not occur function (paralysis) o Weakness begins in the feet Uncoordinated movement and legs and may move up to Sensation changes the arms and head Numbness, decreased sensation o May get worse over 24 to 72 Tenderness or muscle pain (may be a hours cramp-like pain) o May start in the arms and Blurred vision move downward Difficulty moving face muscles o May begin in the arms and Clumsiness and falling legs at the same time Palpitations (sensation of feeling o May occur in the nerves of the heartbeat) head only Muscle contractions Emergency symptoms (seek immediate medical help): Difficulty swallowing Drooling Difficulty breathing Breathing, may temporarily stop Can't take a deep breath Fainting Exams and Tests A history of increasing muscle weakness and paralysis may indicate Guillain-Barre syndrome, especially if there was a recent illness. A medical exam may show muscle weakness and problems with involuntary (autonomic) body functions such as blood pressure and heart rate. There may be signs of decreased breathing (caused by paralysis of the breathing muscles), and a decrease in certain arm and leg reflexes. The following tests may be ordered: NCV (nerve conduction velocity) shows nerve damage. EMG tests the electrical activity in muscles. It may shows that nerves do not react properly to stimulation. CSF (cerebrospinal fluid) may have increased levels of protein without an increase in white blood cells. ECG may show heart problems in some cases. Vital Capacity and MIP to assess respiratory muscle strength Treatment There is no cure for Guillain-Barre syndrome. However, many treatments are available to help reduce symptoms, treat complications, and speed up recovery. A method called plasmapheresis is used to remove a person's blood and replace it with intravenous (IV) fluids or donated blood that is free of Pathology Review Page 74 proteins called antibodies. High-dose immunoglobulin therapy (IVIg) is another procedure used to reduce the severity and length of Guillain-Barre symptoms. Other treatments are directed at preventing complications. Proper body positioning or a feeding tube may be used to prevent choking during feeding. Blood thinners may be used to prevent blood clots. Pain is treated aggressively with anti-inflammatory medicines and narcotics, if needed. Breathing modalities such as IPPB, BiPAP and mechanical ventilation The patient is usually trached at some point and bronchial hygiene will need to be performed and well as frequent VC/MIP and ABG analysis to assess ventilation. CXR will show under inflation NORMAL VC LEVEL: above 65 ml/kg; at 30 most people need CPT do to poor cough; at 25 most people need IS or IPPB for atlectasis; at 12-15 most patients need mechanical ventilation Gastroschisis Gastroschisis is a birth defect in which an infant's intestines stick out of the body through a defect on one side of the umbilical cord. Gastroschisis is a type of hernia. Babies with this condition have a hole in the abdominal wall, usually on the right side of the umbilical cord. The child's intestines can be easily seen. The condition is similar to an omphalocele in appearance. An omphalocele, however, is a birth defect in which the infant's intestine or other abdominal organs stick out of the belly button area. Symptoms Lump in the abdomen Intestine sticks through the abdominal wall near the umbilical cord Problems with movement and absorption in the gut due to the unprotected intestine being exposed to irritating amniotic fluid Exams and Tests Physical examination of the infant is sufficient for the health care provider to diagnose gastroschisis. The mother may have shown signs indicating excessive amniotic fluid (polyhydramnios). Prenatal ultrasonography often identifies the gastroschisis. Treatment If identified before birth, mothers with gastroschisis need special monitoring to make sure the unborn baby remains and healthy. Plans should be made for careful delivery and immediate management of the problem after birth. Treatment for gastroschisis is surgery. A surgeon will put the bowel back into the abdomen and close the defect, if possible. If the abdominal cavity is too small, a mesh sack is stitched around the borders of the defect and the edges of the defect are pulled up. Over time, the herniated intestine falls back into the abdominal cavity, and the defect can be closed. Other treatments for the baby include nutrients by IV and antibiotics to prevent infection. The baby's temperature must be carefully controlled, since the exposed intestine allows a lot of body heat to escape. RT involvement: The RT will most likely intubate the patient pre operatively after birth. The baby will have flattened diaphragms due to displacement of the intestines, this may affect breathing. Gastrointestinal bleeding A GI bleed refers to any bleeding that starts in the gastrointestinal tract, which extends from the mouth to the large bowel. The amount of bleeding can range from nearly undetectable to acute, massive, and life threatening. Bleeding may come from any site along the gastrointestinal tract, but is often divided into: Upper GI bleeding: The upper gastrointestinal (GI) tract is located between the mouth and outflow tract of the stomach. Lower GI bleeding: The lower GI tract is located from the outflow tract of the stomach to the anus (small and large bowel included). Considerations Pathology Review Page 75 Gastrointestinal bleeding can range from microscopic bleeding (the amount of blood is so small that it can only be detected by laboratory testing) to massive bleeding (pure blood is passed).It is important to be aware of gastrointestinal bleeding, because it may point to many significant diseases and conditions. Prolonged microscopic bleeding can lead to massive loss of iron, causing anemia. Acute, massive bleeding can lead to hypovolemia, shock, and death. Causes Some of the possible causes of gastrointestinal bleeding include: ulcer, hemorrhoids, crohns disease, espohageal varices, ischemic bowel, post surgery, placental abruption, and many others. RT involvement: The RT will provide O2 for Anemia and possibly perform cardiopulmonary resuscitation for severe hypovolemic shock. Goodpasture Syndrome Goodpasture syndrome is a disease that affects the kidneys and lungs. It usually involves rapidly progressive kidney failure that develops in days to weeks along with lung disease (cough, shortness of breath, and blood in the sputum).Some forms of the disease involve just the lung or kidney, not both. Goodpasture syndrome is an autoimmune disorder. This means your body makes antibodies that attack your own body tissues. In this case, antibodies form against a certain type of protein called collagen. The collagen is present in the alveoli (tiny air sacs in the lungs) and in the glomeruli (the filtering units of the kidney). These antibodies are called anti-glomerular basement membrane antibodies (or anti-GBM antibodies). Sometimes the disorder is triggered by a viral respiratory infection or by inhaling hydrocarbon solvents. In such cases, the immune system may attack organs or tissues because it mistakes them for these viruses or foreign chemicals. The antibody attack leads to bleeding in the air sacs, which causes shortness of breath, cough, and bloody sputum. It also causes inflammation in the glomeruli of the kidney, which causes blood in urine (hematuria), protein in the urine (proteinuria), or kidney failure. Symptoms Bloody urine Difficulty breathing after exertion Dark colored urine Weakness Decreased urine output Nausea and vomiting Foamy urine Chest pain Cough with bloody sputum (coughing Pale skin up blood) Exams and Tests During a physical examination, the health care provider will usually discover that the patient has high blood pressure. The patient usually has signs of fluid overload, such as swelling, gallop rhythms of the heart, and crackle sounds in the lungs. The crackles may also be from blood in the air sacs. Urinalysis shows blood and protein in Arterial blood gas analysis may show the urine. Abnormal red blood cells low oxygen in the blood. may be seen. Lung biopsy shows damaged alveoli. BUN and creatinine levels are elevated. Kidney biopsy shows damaged Chest x-ray shows diseased alveoli. glomeruli. Anti-GBM antibody levels are elevated. Treatment The main goal is to remove the circulating antibodies from the blood. An early diagnosis is very important. The patient's outlook is much worse if the kidneys are already severely damaged when treatment begins. Plasmapheresis is a procedure where blood plasma is removed from the circulation and replaced by fluid, protein, or donated plasma. This helps to make sure that harmful antibodies are removed. Antiinflammatory and cytotoxic agents (such as prednisone or cyclophosphamide) may be needed. If kidney failure becomes severe, dialysis may be needed to substitute for the kidney's normal functioning. Kidney Pathology Review Page 76 transplantation may be performed in patients who suffer irreversible loss of kidney function. The nephrologist (a specialist physician) would usually wait for the levels of circulating anti-GBM antibodies to drop before proceeding with the transplant. VENT Settings: most likely the patient will require lots of PEEP meaning they will need PCV mode and high O2. Sedation is often given as well as EPI to control pulmonary bleeding. Hepatic encephalopathy Hepatic encephalopathy is brain and nervous system damage that occurs as a complication of liver disorders. It causes different nervous system symptoms including changes in reflexes, changes in consciousness, and behavior changes that can range from mild to severe. Hepatic encephalopathy is caused by disorders affecting the liver. These include disorders that reduce liver function (such as cirrhosis or hepatitis) and conditions where blood circulation does not enter the liver. The exact cause of hepatic encephalopathy is unknown. When the liver cannot properly metabolize and turn poisons into harmless substances in the body, these poisons build up in the bloodstream. One substance believed to be particularly harmful to the central nervous system is ammonia, which is produced by the body when proteins are digested. Ammonia is normally made harmless by the liver. Many other substances may also accumulate in the body if the liver is not working well. They add to the damage done to the nervous system. THE PATIENT WILL HAVE METABOLIC ACIDOSIS; if severe enough the patient will become comatose and airway management may need to be implemented. In people with otherwise stable liver disorders, hepatic encephalopathy may be triggered by gastrointestinal bleeding, eating too much protein, infections, renal disease, procedures that bypass blood past the liver, and electrolyte abnormalities (especially a decrease in potassium). A potassium decrease may result from vomiting, or treatments such as paracentesis or taking diuretics. Hepatic encephalopathy may also be triggered by any condition that results in alkalosis, low oxygen levels in the body, use of medications that suppress the central nervous system (such as barbiturates or benzodiazepine tranquilizers), surgery, and sometimes by co-occurring illness. Disorders that mimic or mask symptoms of hepatic encephalopathy include alcohol intoxication, sedative overdose, complicated alcohol withdrawal, Wernicke-Korsakoff syndrome, subdural hematoma, meningitis, and metabolic abnormalities such as low blood glucose. Symptoms Changes in mental state, Decreased self-care ability consciousness, behavior, personality Deterioration of handwriting or loss of o Forgetfulness other small hand movements o Confusion, disorientation Muscle tremors o Delirium Muscle stiffness o Dementia Seizures (rare) o Changes in mood Speech impairment o Decreased alertness, daytime Uncontrollable movement sleepiness Dysfunctional movement o Decreased responsiveness Agitation o Coma Blood tests may be nonspecific, or may show liver failure. Blood chemistry may show low albumin, high bilirubin, or other abnormalities. Serum ammonia levels are usually high. Prothrombin time may be prolonged and not correctable with Vitamin K. CT scan of the head may be normal, or may show general atrophy (loss of tissue). EEG (a reading of electrical activity in the brain) shows abnormalities. Treatment Hepatic encephalopathy is an acute medical condition that may become a medical emergency. Hospitalization is required. The goals of treatment include life support, elimination or treatment of the causes, and removal or neutralization of ammonia and other toxins. Life support may include support of Pathology Review Page 77 breathing or blood circulation, particularly if coma develops. The brain may swell, which can be lifethreatening. Sedatives, tranquilizers, and any other medications that are broken down or released by the liver should be avoided if possible. Medications containing ammonium (including certain antacids) should also be avoided. Other medications and treatments may be recommended, with variable results HELLP Syndrome HELLP syndrome occurs in approximately 10% of pregnant women with pre-eclampsia or eclampsia. Preeclampsia may be mild or severe. Severe cases involve high blood pressure and protein in the urine and can progress to seizures (eclampsia). Severe cases are life-threatening to both mother and fetus. HELLP syndrome is associated with more serious cases. It may occur long before a pregnancy reaches term (for example, HELLP at 30 weeks gestation). Many women have high blood pressure and are diagnosed with pre-eclampsia before they get HELLP syndrome. However, in some cases, HELLP symptoms are the first warning of pre-eclampsia and the condition is misdiagnosed as hepatitis, gallbladder disease, idiopathic thrombocytopenic purpura, or thrombotic thrombocytopenic purpura. Symptoms Progressive nausea and vomiting Upper abdominal pain Headache Vision problems Exams and Tests During a physical examination, the doctor may discover upper abdominal tenderness, especially in the right upper quadrant. The liver may be enlarged. Liver function tests (liver enzyme tests) may be elevated. Red blood cell and platelet counts may be low. Treatment The main treatment is to deliver the baby as soon as possible, even if premature, since liver function in the mother gets worse very quickly. Problems with the liver can be harmful to both mother and child. NOTE TO RT: PREMATURE BABY = YOU NEED TO DO SOMETHING! Hemothorax Hemothorax is a collection of blood in the space between the chest wall and the lung (the pleural cavity). The most common cause of hemothorax is chest trauma. Hemothorax can also occur in patients with lung or pleural cancer, or in patients with a defect of the blood clotting mechanism. The condition is also commonly linked with thoracic or heart surgery, and can also occur in patients who suffer pulmonary infarction (death of lung tissue). In blunt chest trauma, a rib may lacerate lung tissue or an artery, causing blood to collect in the pleural space. In penetrating chest trauma, a weapon such as a knife or bullet lacerates the lung. A large hemothorax is often a cause of shock in a trauma victim. Hemothorax may also be associated with pneumothorax (air trapped in the pleural cavity). Depending on the amount of blood or air in the pleural cavity, a collapsed lung can lead to respiratory and hemodynamic failure (tension pneumothorax). Hemothorax can also be a complication of tuberculosis. Symptoms Chest pain Rapid heart rate Shortness of breath Anxiety Respiratory failure Restlessness Pathology Review Page 78 The goal of treatment is to stabilize the patient, stop the bleeding, and remove the blood and air in the pleural space. A chest tube is inserted through the chest wall to drain the blood and air. It is left in place for several days to re-expand the lung. The cause of the hemothorax should be also treated. In trauma patients, depending on the severity of the injury, chest tube drainage is often all that is necessary, and surgery is often not required. Infant Respiratory Distress Syndrome Respiratory distress syndrome (RDS) is defined as a condition of prematurity whereas the surfactant in the alveoli has yet to reach maturity. Normal gestational age is 40 weeks, RDS usually occurs before about 34 weeks (34-35 weeks is when surfactant matures naturally). Without surfactant, alveoli have no surface area causing respiratory distress which include symptoms like retractions, grunting, tachypnea and nasal flaring. Most infants who develop RDS show signs of breathing problems at birth or within the next few hours. RDS is one of the most common lung disorders in premature babies. It affects about 10 of every 100 premature infants in the United States, or about 40,000 babies, each year. In fact, nearly all babies born before 28 weeks of pregnancy develop RDS. What Causes Respiratory Distress Syndrome? A lack of surfactant in a premature baby's lungs causes respiratory distress syndrome (RDS). Surfactant maintains stability of the alveoli. The transition from fetal life (lungs not in use) to external life becomes difficult as the neonate has to overcome tremendous resistance to make up for absent FRC. Other possible causes of RDS: If you have diabetes mellitus Cesarean delivery Stress during delivery, especially hemorrhage (a large blood loss) Infection Some infants born at term develop RDS because they have abnormal genes for surfactant. What Are the Signs and Symptoms of Respiratory Distress Syndrome? Signs and symptoms of respiratory distress syndrome (RDS) usually appear at birth or within the next few hours. They include: Rapid, shallow breathing apnea Sharp pulling in of the chest below the A collapsed lung or scattered atlectasis. ribs with each breath taken in Leakage of air from the lung into the (retractions) chest cavity due to tearing of premature ac membrane as the Grunting sounds during exhalation (breathing against a half closed glottis neonate inhales/exhales to produce a back pressure and FRC) Bronchopulmonary dysplasia, Flaring of the nostrils during breathing (to produce a back pressure or PEEP) Pathology Review Page 79 Bleeding in the brain, which can lead to ROP delayed mental development, mental Kidney failure, only in the most severe retardation, and cerebral palsy. cases. Sepsis, an infection of the bloodstream. Necrotizing enterocolitis, a disease of the bowel Bleeding in the lung. How Is Respiratory Distress Syndrome Diagnosed? Chest x ray. Shows areas of collapse (noted as ground glass appearance) ABG: Will show respiratory acidosis, possibly combined due to perfusion problems Echocardiogram. This test uses sound waves to create a moving picture of the heart. An echocardiogram is used to rule out congenital heart defects as the cause of the breathing problems. Main diagnoses done so by gestational age, prenatal care including steroid administration before birth and by physical assessment and symptoms How Is Respiratory Distress Syndrome Treated? RDS presents quickly and those neonates are placed in the NICU for treatment. Surfactant may be given once the neonate is intubated to expand collapsed alveoli and stimulate surfactant growth. Specific RT interventions include the use of NCPAP, mechanical ventilation and HFV/HFO. Mechanical ventilation is always PCV due to the low compliance; PIP start around 20-25 cmH2O and 25-30 for more premature infants. SIMV rates of 20-40 used, and O2 kept below 40% if possible to prevent ROP. NCPAP is used if at possible in replace of the ventilator to reduced to damage caused by mechanical ventilation in the alveoli from the constant opening and closing of a non compliant lung. When this damage worsens the option of HFV/HFO come in to play to maintain the lung at dead space volume (using AMP, MAP and Frequency) while maintaining gas exchange thus avoiding complications of opening and closing the lungs. Surfactant Replacement Therapy The baby is given surfactant until his or her lungs have developed enough to start making their own surfactant. The machine pushes the surfactant directly into the baby's lungs. Surfactant may be given right after birth in the delivery room to try to prevent or treat RDS. It can be given two to four more times over the next few days, until the baby is able to breathe on his or her own. Idiopathic Pulmonary Fibrosis Pulmonary fibrosis is defined as a process of inflammation in the alveolar sac that leads to scar tissue formation. This impedes gas exchange. As the lung tissue becomes thicker, the ability to move oxygen into your bloodstream lessens, causing hypoxemia and hypoxia. Idiopathic simply means that the root cause is unknown. However most PF occurs as a result of chronic inhalation of irritate gas or gas particles. IPF varies from person to person. In some people, the lung tissue quickly becomes thick and stiff. In others, the process is much slower, and in some people, the condition stays the same for years. There is no cure Pathology Review Page 80 for IPF yet. Many people live only about 3 to 5 years after diagnosis. The most common cause of death related to IPF is respiratory failure. Other causes include: Pulmonary arterial hypertension Heart failure Pulmonary embolism Pneumonia Lung cancer What Causes Idiopathic Pulmonary Fibrosis? Doctors don’t know what causes idiopathic pulmonary fibrosis (IPF). They think that something inside or outside of the lungs attacks the lungs again and again over time. These attacks injure the lungs and cause scarring in the tissue inside and between the air sacs. This makes it harder for oxygen to pass through the air sac walls into the bloodstream. Possible causes: Cigarette smoking Viral infections, including Epstein Barr virus (which causes mononucleosis), influenza A virus, hepatitis C virus, HIV-a virus, and herpes virus 6 Exposure to environmental pollutants, including inorganic dust (silica and hard metal dusts), organic dust (bacteria and animal proteins), and gases and fumes Use of certain medicines, including: o Nitrofurantoin and sulfasalazine, which are antibiotics used mostly to treat urinary tract infections, ulcerative colitis, and rheumatoid arthritis o Medicines to keep your heartbeat regular (for example, amiodarone or propranolol) o Chemotherapy or cancer medicines, such as methotrexate, cyclophosphamide, and bleomycin Your genes may also play a role in the development of IPF. Some families have at least two members with IPF. Scientists have found that 9 out of 10 people with IPF also have gastroesophageal reflux disease (GERD). As a result, some scientists think that some people who have GERD may breathe in tiny drops of acid from their stomachs on a regular basis. The acid may then injure the lungs and lead to IPF. What Are the Signs and Symptoms of Idiopathic Pulmonary Fibrosis? The signs and symptoms of idiopathic pulmonary fibrosis (IPF) develop over time. They may not even begin to appear until the disease has done serious damage to your lungs. Once they do appear, they are likely to become worse over time.The most common signs and symptoms are: Shortness of breath. This is usually the main symptom. At first, you may be short of breath only during exercise. Over time, you are likely to feel breathless even at rest. A dry, hacking cough that doesn’t get better when you treat it with over-the-counter cough medicines. Over time, you may have repeated bouts of coughing that you can't control. Rapid, shallow breathing Gradual, unintended weight loss Fatigue (tiredness) or malaise (a general feeling of being unwell) Aching muscles and joints Enlargement of the ends of the fingers or toes, which is called clubbing IPF may lead to other medical conditions, including: collapsed lung, lung infections, blood clots in the lungs, and lung cancer. How Is Idiopathic Pulmonary Fibrosis Diagnosed? Idiopathic pulmonary fibrosis (IPF) produces the same kind of scarring and symptoms as some other lung diseases. This makes it hard to diagnose. Medical History (the following is obtained) age history of legal and illegal drug use history of smoking Other medical conditions that you have that can trigger an immune system Things in the air at your job or response elsewhere that could irritate your lungs family's medical history hobbies Pathology Review Page 81 How long you've had symptoms Diagnostic Tests There is no single test for diagnosing IPF. Chest X Ray: It can show shadows that suggest scar tissue. Many people with IPF have normal chest x rays at the time they’re diagnosed. High-Resolution Computerized Tomography: High-resolution computerized tomography (HRCT) scan is an x ray that provides sharper and more detailed images than a regular chest x ray. It can show scar tissue and how much lung damage you have. This test can help your doctor spot IPF at an early stage or rule it out. Lung Function Tests: Will show restrictive patterns ABG: Show hypoxemia and respiratory acidosis depending on advancement of illness TB test: used to rule out TB as cause of respiratory distress Exercise testing is used to find out how well your lungs move oxygen and carbon dioxide in and out of your bloodstream when you’re active. During this test, you walk or pedal on an exercise machine for a few minutes. Electrodes attached to your chest and an EKG (electrocardiogram) machine show your heart rate, a blood pressure cuff monitors your blood pressure, and a pulse oximeter attached to a finger or ear lobe shows how much oxygen is in your blood. Lung Biopsy Looking at samples of tissue from several places in your lungs under a microscope is the best way for your doctor to diagnose IPF. A lung biopsy can help your doctor rule out other causes of your condition, such as sarcoidosis, cancer, or infection. It can also show your doctor how far your condition has advanced. Doctors use several different procedures to obtain samples of your lung tissue: Bronchoscopy. Bronchoalveolar lavage during bronchoscopy Video-assisted thoracoscopy. This is the procedure that doctors use most to obtain lung tissue. Your doctor inserts a small, lighted tube with a camera (endoscope) into your chest through small incisions between your ribs. The endoscope provides a video image of the lungs and allows your doctor to collect samples of tissue. This procedure must be done in the hospital, under general anesthesia. Thoracotomy. Your doctor removes a few small pieces of lung tissue through an incision in the chest wall between your ribs. Thoracotomy is done in the hospital under general anesthesia. How Is Idiopathic Pulmonary Fibrosis Treated? The goals of treating idiopathic pulmonary fibrosis (IPF) are to prevent more lung scarring, relieve your symptoms, maintain your ability to be active and get around, keep you healthy, and improve your quality of life. Treatment can’t remove scarring that has already happened. As a result, diagnosing and treating IPF as early as possible, before a lot of scarring has taken place, is very important. Treatment is usually based on your age, medical history, any medical problems you may have, and how much the IPF has advanced. Treatments can include: Medicines to reduce inflammation (swelling) in your lungs and/or prevent more scarring Oxygen therapy Pulmonary rehabilitation Lung transplantation Medicines The main treatment for IPF is medicine to reduce inflammation. Many doctors also add a medicine to suppress your body's immune system. These treatments can prevent further scarring and increase survival time in some people, but they don’t work for everyone. Prednisone The anti-inflammatory medicine that most doctors prescribe is high-dose prednisone, a corticosteroid. You usually take prednisone by mouth every day. Sometimes your doctor may give it to you through a needle or tube inserted into a vein in your arm for several days. After that, you usually take it by mouth. Because prednisone can cause serious side effects, your doctor may prescribe it for only 3 to 6 months at Pathology Review Page 82 first. Then, if it works for you, your doctor may reduce the dose over time and keep you on it for a longer time. Most people who take high-dose prednisone for a long time can have side effects, including difficulty sleeping at night (insomnia), weight gain, acne, and irritability. Using prednisone for a long time also can lead to other conditions, including: High blood pressure. Hyperglycemia (high blood sugar). Cataracts (a cloudy area in the lens in your eyes). Glaucoma (a serious eye condition that can lead to blindness). Anxiety or depression. Osteoporosis (thinning of the skin and bones). Adrenal gland insufficiency (a condition in which the adrenal glands don’t produce enough of certain hormones). This condition should be treated by an endocrinologist—a doctor who specializes in the diagnosis and treatment of the adrenal glands. Prednisone also can cause conditions such as diabetes and glaucoma to get worse. Other Medicines Many doctors prescribe a second medicine with prednisone. Azathioprine (Imuran®) is a medicine that affects your immune system. Most patients take it by mouth every day. Because it can cause serious side effects, your doctor may prescribe it with prednisone for only 3 to 6 months. Then, if you don’t have serious side effects, and the drug combination seems to help you, your doctor may keep you on it long term. The most common side effects of azathioprine include: o Nausea, vomiting, diarrhea, and fever and chills o Anemia and low platelet and white blood cell counts o Liver problems o Pancreatitis or lymphoma (rarely) Cyclophosphamide (Cytoxan®) is another immune system suppressant that doctors use to treat IPF. They usually add it to low doses of prednisone for patients who are getting worse while taking prednisone alone. Many patients who can't take prednisone take cyclophosphamide alone. Most people take cyclophosphamide by mouth every day. Some IPF patients receive it for 3 to 5 days through a needle that is inserted in a vein in the arm. After that, they take it by mouth every day. You usually start on a low dose that’s increased over time. It may take 3 to 6 months before you see any benefits from cyclophosphamide. The most common side effect of cyclophosphamide is a decrease in the number of blood cells that you have. This increases your chances for infection. Your doctor may order blood tests before, during, and after your treatment to see how your blood cells are affected by the drug. Other side effects can include: o Infertility in both men and women. o Nausea, diarrhea, and fatigue (tiredness). o Hair loss. o Bladder irritation. Some people who have taken cyclophosphamide for more than 2 years have developed bladder cancer. If you take cyclophosphamide, you should drink at least 8 glasses of water a day. Your doctor should test your urine at least monthly. Other medicines that may help people with IPF include the following: Influenza and pneumonia vaccinations may help prevent infection and keep you healthy. Cough medicines or oral codeine may relieve coughing. Vitamin D, calcium, and a bone-building drug may help prevent bone loss if you are taking prednisone or another corticosteroid. Anti-reflux therapy may help control gastroesophageal reflux disease. Oxygen Therapy When the amount of oxygen in your blood gets low, you may need oxygen therapy to help reduce your shortness of breath and let you be more active. Oxygen is usually given through nasal prongs or a mask. At Pathology Review Page 83 first, you may need it only during exercise and sleep. As your condition gets worse, you may need it all the time. Pulmonary Rehabilitation Pulmonary rehabilitation is now the standard of care for people with ongoing lung disease. It usually involves treatment by a team of specialists in a specialized clinic. The goal is to teach you how to manage your condition and function at your best. Services usually include: Physical conditioning training Breathing exercises and retraining, so that it takes less energy for you to breathe Anxiety, stress, and depression management Nutritional counseling Support groups Lung Transplantation Early referral for surgery to replace one of your lungs with a healthy lung from a human donor is usually recommended if you: Are younger than 65 Have no other medical problems Are not being helped by medicines Single lung transplantation can improve your quality of life and help you live longer. Complications can include rejection by the body of the transplanted lung and infection. You may have to take medicines for life to reduce the chances that your body will reject the transplanted lung. Because the supply of donor lungs is limited, asking for an evaluation for a transplant as soon as possible is important. Kyphoscoliosis Kyphoscoliosis describes an abnormal curvature of the spine in both a coronal and sagittal plane. It is a combination of kyphosis and scoliosis. This causes a compression on internal organs including the lungs. The patient will have a limited ability to expel sputum do to the misshaped parenchyma. The patient may need assistance from a tracheotomy and mechanical ventilation as this presents as a severe restrictive disease. Severe restrictive ventilatory insufficiency and hypoxaemic-hypercapnic respiratory failure is common among serious cases of kyphoscoliosis. Some patients may be on ventilatory support their entire life as others are weaned as corrective surgical procedures may improve kyphoscoliosis to small degrees. Pathology Review Page 84 Kyphoscoliosis Legionnaires' Disease ;Also called: Legionellosis Legionnaires' disease is a type of pneumonia caused by bacteria. You usually get it by breathing in mist from water that contains the bacteria. The mist may come from hot tubs, showers or air-conditioning units for large buildings. The bacteria does not spread from person to person (common outbreaks occur on cruise ships). Symptoms of Legionnaires' disease include fever, chills, a cough and sometimes muscle aches and headaches. Other types of pneumonia have similar symptoms. Diagnosis is made through history, CXR, physical exam, CBC and sputum culture. The bacteria are more likely to make you sick if you Are older than 65 Smoke Have a lung disease Have a weak immune system Legionnaires' disease is serious and can be life-threatening. However, most people recover with antibiotic treatment Lung Cancer Lung cancer affects more than 200,000 Americans each year. Although cigarette smoking is the main cause, anyone can develop lung cancer. Lung cancer is always treatable, no matter the size, location, or if the cancer has spread.There are two major types of lung cancer: non-small cell and small cell. Non-small cell lung cancer (NSCLC) arises from epithelial cells and is the most common type. Small cell lung cancer begins in the nerve cells or hormone-producing cells of the lung. The term "small cell" refers to the size and shape of the cancer cells as seen under a microscope. It is important to distinguish NSCLC from small cell lung cancer because the two types of cancer are usually treated in different ways. Lung cancer begins when cells in the lung grow out of control and form a lump (also called a tumor, mass, lesion, or nodule). A cancerous tumor is a collection of a large number of cancer cells and appears as a lump within the lung tissues. A lung tumor can begin anywhere in the lung. Once a cancerous lung tumor begins to grow, it may or may not shed cancer cells. Shed cells can be carried away in blood, or float away in the natural fluid, called lymph, that surrounds lung tissue. Lymph flows through tubes called lymphatic vessels that drain into collecting stations called lymph nodes. Lymph nodes are located in the lungs, the center of the chest, and elsewhere in the body. The natural flow of lymph out of the lungs is toward the center of the chest, which explains why lung cancer often spreads there. When a cancer cell leaves its site of origin, and moves into a lymph node or to a far away part of the body through the bloodstream, it is called metastasis. The location and size of the initial lung tumor, and whether it has spread to lymph nodes or more distant sites, determines the stage of lung cancer. The type of lung cancer (NSCLC versus small cell) and stage of the disease (discussed later in Staging) determine what type of treatment is needed. Pathology Review Page 85 Risk Factors and Prevention A risk factor is anything that increases a person's chance of developing cancer. Some risk factors can be controlled, such as smoking, and some cannot be controlled, such as age and family history. Although risk factors can influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do. However, knowing your risk factors and communicating them to your doctor may help you make more informed lifestyle and health-care choices. The following factors can raise a person's risk of developing lung cancer: Tobacco. Most lung cancer occurs in people who smoke. Tobacco smoke damages cells in the lungs, causing the cells to grow abnormally. The risk that smoking will lead to cancer is higher for people who smoke heavily and/or for a long time. Regular exposure to smoke from someone else's cigarettes, cigars, or pipes (called environmental or "secondhand" tobacco smoke) can increase a person's risk of lung cancer even if that person does not smoke. Asbestos. These are hair-like crystals found in many types of rock and are often used as fireproof insulation in buildings. When asbestos fibers are inhaled, they can irritate the lung. Many studies show that the combination of smoking and asbestos exposure is particularly hazardous. People who work with asbestos in jobs (such as shipbuilding, asbestos mining, insulation, or automotive brake repair) and smoke have a higher risk of developing lung cancer. Using protective breathing equipment reduces this risk. Radon. This is an invisible, odorless gas naturally released by some soil and rocks. Exposure to radon has been associated with an increased risk of some types of cancer, including lung cancer. Most hardware stores have kits that test home radon levels, and basements can be ventilated to reduce radon exposure. The most important way to prevent lung cancer is to avoid tobacco smoke. People who never smoke have the lowest risk of lung cancer. People who smoke can reduce their risk of lung cancer by stopping smoking, but their risk of lung cancer will still be higher than people who never smoked. Attempts to prevent lung cancer with vitamins or other treatments have not worked. Beta-carotene, a drug related to vitamin A, has been tested for the prevention of lung cancer. It did not reduce the risk of cancer. In people who continued to smoke, beta-carotene actually increased the risk of lung cancer. Symptoms People with lung cancer may experience the following symptoms. Sometimes people with lung cancer do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom on this list, please talk with your doctor. For people with lung cancer who have no symptoms, their lung cancer can be discovered on a chest x-ray or CT scan performed for some other reason, such as checking for heart disease. Most people with lung cancer are diagnosed when the tumor grows, takes up space, or begins to interfere with nearby structures. A lung tumor may also make fluid that can collect in the lung or the space around the lung. A tumor can push the air out of the lungs and cause the lung to collapse. In this way, a lung tumor can prevent the exchange of oxygen and carbon dioxide by blocking the flow of air into the lungs, or by using up the space normally required for oxygen to come in and carbon dioxide to go out of the lung. Symptoms of lung cancer may include: Fatigue Coughing up phlegm or mucus Cough Hemoptysis (coughing up blood) Shortness of breath Chest pain, if a tumor invades a structure within the chest or involves the lining of the lung Loss of appetite Although lung cancer can metastasize (spread) anywhere in the body, the most common sites of spread are the lymph nodes, lungs, bones, brain, liver, and structures near the kidneys called the adrenal glands. Metastases (spread to more than one area) from lung cancer can cause further breathing difficulties, bone pain, abdominal or back pain, headache, weakness, seizures, and/or speech difficulties. Rarely, a lung tumor can release hormones that result in chemical imbalances, such as low blood sodium levels or high blood calcium.Symptoms such as fatigue, malaise (feeling out-of-sorts or unwell), and loss of appetite Pathology Review Page 86 are not necessarily due to metastases. The presence of cancer anywhere in the body can cause a person to feel unwell in a general way. Loss of appetite can result in weight loss. Fatigue and weakness can further worsen breathing difficulties. RT involvement with lung CA: RT’s frequently assist with bronchoscopy which is used to biopsy lung tissue. RT’s perform bronchial hygiene and airway maintenance of individuals with lung CA. RT’s also assist with patient’s complaining of SOB from lung ca and pleural effusion associated with lung CA. Mesothelioma The tissue that lines your lungs, stomach, heart and other organs is called mesothelium. Mesothelioma is cancer of that tissue. It is a rare but serious type of cancer. It usually starts in the lungs, but can also start in the abdomen or other organs. Most people who develop mesothelioma have worked on jobs where they inhaled asbestos particles. It can take a long time - 30 to 50 years - between being around asbestos and getting the disease. Treatment includes surgery, radiation, chemotherapy or all three. Meconium Aspiration Meconium aspiration occurs when a baby breathes in amniotic fluid containing meconium (the baby's first stools). Before or during labor, the fetus sometimes passes the meconium stool into the amniotic fluid. It is not clearly understood why this happens. It may be a natural event, but it is also thought to be related to fetal distress in some babies. When the thick meconium mixes into the amniotic fluid, it is swallowed and breathed into the airways of the fetus. As the baby takes the first breaths at delivery, meconium particles enter the airways and can be aspirated (inhaled) deep into the lungs leading to pneumonia and sepsis. Meconium is passed into the amniotic fluid in about 5 to 15 percent of births. It usually occurs in babies born at term (37 to 41 weeks) or post-term (after 42 weeks). Meconium particles in the amniotic fluid can block small airways and prevent the exchange of oxygen and carbon dioxide after birth. Some babies have immediate respiratory distress and have to be resuscitated at birth. Others develop respiratory distress within a few hours. Some babies with meconium aspiration need a mechanical ventilator (breathing machine) because of the difficulty breathing. The plugged airways may cause air to be trapped and leak into the tissues in and around the lungs. Infection can also occur causing pneumonia. Although the condition often improves within a few days, severe meconium aspiration, and the respiratory problems it causes, may lead to death in a small number of babies. What are the symptoms of meconium aspiration? Meconium in the amniotic fluid gives the fluid a greenish color. This is called meconium staining. Babies who have been exposed to meconium in the amniotic fluid for a long time may have yellowed skin and nails. The following are the most common symptoms of meconium aspiration. However, each baby may experience symptoms differently. Symptoms may include: Pathology Review Page 87 rapid breathing retractions (pulling in of the chest wall) grunting sounds with breathing cyanosis (blue coloring) overdistended chest Treatment for meconium aspiration (based on the following) the amount and thickness of the meconium the length of time the baby was exposed the degree of respiratory distress At delivery, treatment may include: suctioning of the upper airways (nose, mouth, and throat) suctioning of the lower airways through an endotracheal tube (ET) if the baby is not active at birth (intubate, suction THEN stimulate) supplemental oxygen given by face mask or mechanical ventilator Prevention of meconium aspiration: Early identification of meconium aspiration is essential to preventing severe aspiration problems. A technique called amnioinfusion is sometimes used during labor with meconium-stained amniotic fluid. This procedure uses a small tube inserted into the uterus through the vagina. Sterile fluid is infused through the tube to help dilute the thick meconium. Suctioning of the upper airways as soon as a baby's head is delivered may also help reduce the effects of meconium aspiration. This meconium aspirator is placed at the end of the ETT and used to suction large particles of meconium. Muscular Dystrophy Also called: MD Muscular dystrophy (MD) refers to a group of more than 30 inherited diseases that cause muscle weakness and muscle loss (most common neuromuscular disease). Some forms of MD appear in infancy or childhood, while others may not appear until middle age or later. The different muscular dystrophies vary in who they affect and the symptoms. All forms of MD grow worse as the person's muscles get weaker. Most people with MD eventually lose the ability to breath without the aid of a ventilator. There is no cure for muscular dystrophy. Treatments include physical and speech therapy, orthopedic devices, surgery and medications. Some people with muscular dystrophy have mild cases that worsen slowly. Others cases are disabling and severe. The disease causes muscle weakness and affects the central nervous system, heart, gastrointestinal tract, eyes (causing cataracts) and endocrine (hormone-producing) glands. Although muscle weakness progresses slowly, this symptom can vary greatly, even among members of a single family. Most often muscle weakness doesn't hamper daily living for many years after symptoms first occur. Many people with myotonic dystrophy seem to require more sleep than the average person. Mild mental retardation is present in some people with the disease, and a peculiar emotional and mental "indifference" has also been found to accompany myotonic dystrophy in some patients. Congenital myotonic dystrophy is a rare Pathology Review Page 88 form of the disorder occurring almost exclusively in infants of mothers with the adult form of the disease. At birth, infants can show symptoms of the disease, including severe weakness, difficulty in sucking and swallowing, and impaired breathing. Delayed motor development and mental retardation are common features of congenital myotonic dystrophy. DUCHENNE muscular dystrophy (DMD) is the most common childhood form of muscular dystrophy. Early signs of Duchenne, which usually occur between the ages of 2 and 6, include frequent falling, difficulty getting up from a sitting or lying position, and a waddling gait. Another hallmark is the apparent enlargement of the calf and sometimes other muscles, which is really due to an accumulation of fat and connective tissue in the muscle. A blood sample shows a very high level of creatine kinase (CK), an enzyme that leaks out of damaged muscle. RT involvement: Breathing becomes affected during the later stages of Duchenne, leading to respiratory infections. These are often successfully treated with antibiotics and respiratory therapy. Severe respiratory and heart problems mark the disease's final stages, usually in the boy's teens or early 20s. Typically these patients are trached, on long term ventilators and require bronchial hygiene. In Duchenne muscular dystrophy, posture changes as the child grows. Myasthenia Gravis Myasthenia gravis interferes with messages your nerves send to your muscles. Myasthenia gravis often affects muscles in your head progressing downward. Initial symptoms are trouble with eye and eyelid movement, facial expression and swallowing. Eventually it progresses to the diaphragm affecting ventilation. Myasthenia gravis is caused by a problem in the transmission of nerve signals to your muscles. Normally, nerve endings release a substance that attaches to receptors on your muscles. That tells your muscles to contract. If you have myasthenia gravis, your body's own immune system. In addition to a complete medical and neurological evaluation, a number of tests may be used to establish a diagnosis of MG. A diagnosis can be confirmed in several ways, including the following: Acetylcholine Receptor Antibody -- A blood test for the abnormal antibodies can be performed to see if they are present. Acetylcholine Receptor Antibody testing - Approximately 85% of MG patients have this antibody and, when detected, is a guaranteed diagnosis. Anti-MuSK Antibody testing - a blood test for the remaining 15% of seronegative (SN) MG patients, those who have tested negative for the acetylcholine antibody, 40-70% test positive for the anti-MuSK antibody. The remaining patients have an unidentified antibody causing their MG. Tensilon® test, The edrophonium chloride (Tensilon®) test is performed by injecting this chemical into a vein. Improvement of strength immediately after the injection provides strong support for the diagnosis of MG. Electromyography -- (EMG) studies can provide support for the diagnosis of MG when characteristic patterns are present. Repetitive Nerve Stimulation to check for a post-synaptic defect, Single Fiber EMG, or a muscle biopsy to look for antibodies may also be used. Single Fiber EMG -- studies can provide support for the diagnosis of MG when characteristic patterns are present. Sometimes all of these tests are negative or equivocal in someone whose story and examination still seem to point to a diagnosis of MG. The positive clinical findings should probably take precedence over negative confirmatory tests. Pathology Review Page 89 In most cases of MG, the immune system makes antibodies that recognize the ACh receptor. The antibodies, Y-shaped missiles that normally attack bacteria and viruses, destroy many of the ACh receptors on muscle. Consequently, the muscles response to repeated nerve stimulation declines with time, causing weakness and fatigue. Is It Really MG? Experts say the first step toward getting effective treatment for MG is an accurate diagnosis. Double vision and droopy eyelids might lead a physician to suspect MG. Weakness of the extraocular muscles, those that control movement of the eyes and eyelids, is conspicuously absent in many neuromuscular diseases, but its a common and early symptom of MG. For about 15 percent of people with MG, the disease remains exclusively "ocular" for its entire course, but for most people, it eventually becomes "generalized," involving voluntary muscles throughout the body. If left untreated, weakness often spreads from the extraocular muscles to the rest of the face (including the jaw, throat, and tongue), then to the limbs and trunk, and finally to the respiratory muscles. Weakness that fluctuates during the day and over longer periods, from months to years, is another hallmark of MG. But other diseases can cause similar symptoms, and to the untrained eye, some of these can look a lot like MG. The most reliable indicator of MG is a blood test that reveals antibodies to the Ach. If the blood test for ACh receptor antibodies is negative, the next step is usually electromyography (EMG), which involves using electrodes to measure a muscles response to nerve impulses. Edrophonium (Tensilon), a fast-acting cholinesterase inhibitor, might be given as a test to temporarily improve neuromuscular transmission. Finally, "Its key to distinguish MG from congenital myasthenic syndromes," Drachman says. These diseases related to but different from MG are caused by genetic defects in the ACh receptor and other components of the neuromuscular junction, and thus can share symptoms with MG. Some even respond well to cholinesterase inhibitors, but they arent treatable with immunosuppressant drugs. The Path to Treatment Once a diagnosis of MG is established, both doctor and patient face many decisions regarding treatment. Most of the available medications and procedures have side effects, from mood changes to gastrointestinal problems, and a few have been linked to more serious ailments, including osteoporosis and kidney damage. While cholinesterase inhibitors boost the amount of ACh that reaches muscle, they dont counter the immune systems assault on the ACh receptor. Thus, long-term treatment of MG usually requires immunosuppression. Immunosuppressant Drugs Among these drugs, corticosteroid hormones and azathioprine (Imuran) are the most widely used. Corticosteroids (which include prednisone) are relatively inexpensive and tend to act fast, producing improvement within weeks. But when taken over the long term, they can have many side effects, including weight gain, high blood pressure, cataracts, osteoporosis, and a blunting of mood and memory. Pathology Review Page 90 Other Options Besides this arsenal of drugs, surgeries and other procedures are often successful in treating MG. Thymectomy, the removal of an immune system gland called the thymus. From fetal development through childhood, the thymus produces immune cells called T-cells, but at puberty the gland starts to degenerate. About 15 percent of people with MG have a thymic tumor, called a thymoma, and another 65 percent have abnormal thymic tissue; theres evidence that these abnormalities might trigger the production of antibodies to the ACh receptor. Thymectomy is necessary for treating thymoma, and often recommended for generalized MG. Two other procedures bring about fast, but short-lived relief from MG, and thus are used mostly for myasthenic crisis, a rapid worsening of symptoms that can culminate in respiratory distress. Plasmapheresis, also called plasma exchange, was developed by MDA-funded researchers in the 1970s. It involves the use of an intravenous line to filter the blood and remove ACh receptor antibodies. Intravenous immunoglobulin (IVIG) therapy is essentially an injection of nonspecific antibody (immunoglobulin). Recent studies suggest that it works by dialing down the immune systems production of its own antibodies, much like the negative feedback circuit that tells the thermostat to stop pumping out heat once your house is warm. RT involvement: eventually MG will lead to respiratory failure and the need for mechanical ventilation. Treatment includes the placement of a trachestomy, mechanical ventilation and bronchial hygiene. Before placement of the vent, the RT should assess NIF and MEP Q8-Q12 to assess the patient’s ability to take a deep breath and cough. MRSA / VRE Also called: Methicillin-resistant Staphylococcus aureus MRSA stands for methicillin-resistant Staphylococcus aureus. It causes an infection that is resistant to several common antibiotics. There are two types of infection. Hospital-associated MRSA happens to people in healthcare settings. Community-associated MRSA happens to people who have close skin-toskin contact with others, such as athletes involved in football and wrestling. Infection control is key to stopping MRSA in hospitals. To prevent community-associated MRSA Practice good hygiene Avoid sharing personal items, such as towels, washcloths, razors, or clothes Keep cuts and scrapes clean and covered with a bandage until healed Wash soiled sheets, towels and clothes in hot water with bleach and dry in a Avoid contact with other people’s hot dryer wounds or bandages Pathology Review Page 91 Treatment may include draining the infection and antibiotics. During the past four decades, a type of bacteria has evolved from a controllable nuisance into a serious public health concern. This bacteria is known as methicillin-resistant Staphylococcus aureus, or MRSA. About one-third of people in the world have S. aureus bacteria on their bodies at any given time, primarily in the nose and on the skin. The bacteria can be present but not causing active infection. Of the people with S. aureus present, about 1 percent have MRSA, according to the Centers for Disease Control and Prevention (CDC).Life-threatening MRSA infections can involve anyone, including people living in confined areas or those who have close skin-to-skin contact with others, such as athletes involved in football and wrestling, soldiers kept in close quarters, inmates, childcare workers, and residents of long-term care facilities. MRSA has attracted the attention of the medical research community, illustrating the urgent need to develop better ways to diagnose and treat bacterial infections. vancomycin-resistant enterococci (VRE) Enterococci are a group of gram-negative, round-shaped bacteria that commonly live in the gut, although they can cause infection anywhere in the body. They are resistant to several antibiotics, but in the past, physicians could rely on the drug vancomycin to effectively treat enterococcal infections. In recent decades, however, some enterococci have become resistant to vancomycin. The two main species that cause problems are vancomycin-resistant Enterococcus faecium and vancomycin-resistant Enterococcus faecalis, with E. faecium being the most common. Vancomycin resistance is acquired when a sensitive Enterococcus acquires a special piece of DNA called a plasmid. The new strain is called vancomycinresistant enterococci (VRE). One concern is that VRE appears able to transfer vancomycin resistance to unrelated bacteria such as MRSA (methicillin-resistant Staphylococcus aureus). In addition, VRE organisms are usually resistant to more than one antibiotic. VRE can also be spread from person to person and are an increasing problem in hospitals and chronic-care facilities. Approximately 30% of all enterococcal infections are now caused by vancomycin-resistant strains (VRE). What causes a vancomycin-resistant enterococcal (VRE) infection? VRE can exist in the body without causing infection, in which case a patient is said to be colonized with VRE. Colonization usually occurs in the bowel. If the number of VRE bacteria increases, they can invade the bloodstream or spread locally to cause an abdominal abscess or urinary infection. Once in the bloodstream, VRE can cause meningitis, pneumonia, or infection of a heart valve (endocarditis). VRE may also be introduced directly into an open sore or wound, causing a wound infection. The bacteria produce several substances, including proteases that help them break down the normal barriers between the gut tissue and the bloodstream. Mounier-Kuhn's syndrome (tracheobronchomegaly) Mounier-Kuhn's syndrome (tracheobronchomegaly) is a rare lung disorder characterized by marked widening (dilation) of the windpipe (trachea) and sometimes the larger bronchial tubes (bronchiectasis). This impairs your ability to clear mucus from your lungs. As mucus accumulates in your lungs, it increases your risk of lung infections.The cause of Mounier-Kuhn's syndrome isn't known. Although it may be present at birth (congenital), Mounier-Kuhn's syndrome rarely causes problems before age 20. Signs and symptoms include: Recurrent lung infections Raspy breathing Shortness of breath Productive cough A doctor may make a diagnosis of Mounier-Kuhn's syndrome by: Chest X-ray Computerized tomography (CT) scan of the lungs Pathology Review Page 92 Treatment is usually directed at managing the signs and symptoms and may include: Quitting smoking Antibiotics Postural drainage to clear mucus from the lungs Lung transplant Necrotizing Enterocolitis A gastrointestinal disease that mostly affects premature infants, NEC involves infection and inflammation that causes destruction of the bowel or part of the bowel. Although it affects only one in 2,000 to 4,000 births, or between 1% and 5% of neonatal intensive care unit (NICU) admissions, NEC is the most common and serious gastrointestinal disorder among hospitalized preterm infants. NEC typically occurs within the first 2 weeks of life, usually after milk feeding has begun (at first, feedings are usually given through a tube that goes directly to the baby's stomach). About 10% of babies weighing less than 1,500 grams (3 lbs., 5 oz.) experience NEC. These premature infants have immature bowels, which are sensitive to changes in blood flow and prone to infection. They may have difficulty with blood and oxygen circulation and digestion, which increases their chances of developing NEC. What Causes It? The exact cause of NEC is unknown, but several theories exist. It is thought that the intestinal tissues of premature infants are weakened by too little oxygen or blood flow, and when feedings are started, the added stress of food moving through the intestine allows bacteria that are normally found in the intestine to invade and damage the wall of the intestinal tissues. The damage may affect only a short segment of the intestine, or it may progress quickly to involve a much larger portion. The infant is unable to continue feedings and starts to appear ill if bacteria continues to spread through the wall of the intestines and sometimes into the bloodstream. He may also develop imbalances in the minerals in the blood. In severe cases of NEC, a hole (perforation) may develop in the intestine, allowing bacteria to leak into the abdomen and causing life-threatening infection (peritonitis). Because the infant's body systems are immature, even with quick treatment for NEC there may be serious complications. Other factors seem to increase the risk of developing NEC. Some experts believe that the makeup of infant formula, the rate of delivery of the formula, or the immaturity of the mucous membranes in the intestines can cause NEC. (Babies who are fed breast milk can also develop NEC, but their risk is lower.) Another theory is that babies who have had difficult deliveries with lowered oxygen levels can develop NEC. When there is not enough oxygen, the body sends the available oxygen and blood to vital organs instead of the gastrointestinal tract, and NEC can result. Babies with an increased number of red blood cells (polycythemia) in the circulation also seem to be at higher risk for NEC. Too many red blood cells thicken the blood and hinder the transport of oxygen to the intestines. NEC sometimes seems to occur in "epidemics," affecting several infants in the same nursery. Although this may be due to coincidence, it suggests the possibility that it could in some cases be spread from one baby to another, despite the fact that all nurseries have very strict precautions to prevent the spread of infection. Signs and Symptoms The symptoms of NEC may resemble other digestive conditions. Every infant experiences the symptoms of NEC differently, which may include: poor tolerance to feedings greenish (bile-colored) vomit feedings stay in stomach longer than redness of the abdomen expected increase in stools, or lack of stools decreased bowel sounds bloody stools abdominal distension (bloating) and tenderness More subtle signs of NEC might include apnea (periodic stoppage of breathing), bradycardia (slowed heart rate), diarrhea, lethargy, and fluctuating body temperature. Advanced cases may show fluid in the peritoneal (abdominal) cavity, peritonitis (infection of the membrane lining the abdomen), or shock. Pathology Review Page 93 Diagnosis and Treatment The diagnosis of NEC is usually confirmed by the presence of an abnormal gas pattern as seen on an X-ray. This is indicated by a "bubbly" appearance of gas in the walls of the intestine, large veins of the liver, or the presence of air outside of the intestines in the abdominal cavity. A surgeon may insert a needle into the abdominal cavity to withdraw fluid to determine whether there is a hole in the intestines. The majority of infants with NEC are treated medically, and symptoms resolve without the need for surgery. Treatment includes: stopping feedings nasogastric drainage (inserting a tube through the nasal passages down to the stomach to remove air and fluid from the stomach and intestine) intravenous fluids for fluid replacement and nutrition antibiotics for infection frequent examinations and X-rays of the abdomen The baby's belly size is measured and watched carefully, and periodic blood samples are taken to determine the presence of bacteria. Stools are also checked for blood. If the abdomen is so swollen that it interferes with breathing, extra oxygen or mechanically assisted breathing (a ventilator) is used to help the baby breathe. Most infants who develop NEC recover fully and do not have further feeding problems. In some cases, scarring and narrowing of the bowel may occur and can cause future intestinal obstruction or blockage. Another residual problem may be malabsorption (the inability of the bowel to absorb nutrients normally). This is more common in children who required surgery for NEC and had part of their intestine removed. Obstructive Sleep Apnea and Obesity hypoventilation syndrome (OHS) Sleep apnea is a disorder that commonly affects more than 12 million people in the United States. People with sleep apnea literally stop breathing repeatedly during their sleep, often for a minute or longer and as many as hundreds of times during a single night. Sleep apnea can be caused by either complete obstruction of the airway (obstructive apnea) or partial obstruction (obstructive hypopnea—hypopnea is slow, shallow breathing), both of which can wake one up. There are three types of sleep apnea— obstructive, central, and mixed. Of these, obstructive sleep apnea (OSA) is the most common. Causes The exact cause of OSA remains unclear. The site of obstruction in most patients is the soft palate, extending to the region at the base of the tongue. There are no rigid structures, such as cartilage or bone, in this area to hold the airway open. During the day, muscles in the region keep the passage wide open. But as a person with OSA falls asleep, these muscles relax to a point where the airway collapses and becomes obstructed. When the airway closes, breathing stops, and the sleeper awakens to open the airway. The arousal from sleep usually lasts only a few seconds, but brief arousals disrupt continuous sleep and prevent the person from reaching the deep stages of slumber, such as rapid eye movement (REM) sleep, which the body needs in order to rest and replenish its strength. Once normal breathing is restored, the person falls asleep only to repeat the cycle throughout the night. Risk Factors The primary risk factor for OSA is excessive weight gain. The accumulation of fat on the sides of the upper airway causes it to become narrow and predisposed to closure when the muscles relax. Age is another prominent risk factor. Loss of muscle mass is a common consequence of the aging process. If muscle mass decreases in the airway, it may be replaced with fat, leaving the airway narrow and soft. Men have a greater risk for OSA. Male hormones can cause structural changes in the upper airway. Other predisposing factors associated with OSA include: Anatomic abnormalities, such as a receding chin Enlarged tonsils and adenoids, the main causes of OSA in children Family history of OSA, although no genetic inheritance pattern has been proven Use of alcohol and sedative drugs, which relax the musculature in the surrounding upper airway Pathology Review Page 94 Smoking, which can cause inflammation, swelling, and narrowing of the upper airway Hypothyroidism, acromegaly, amyloidosis, vocal cord paralysis, post-polio syndrome, neuromuscular disorders, Marfan's syndrome, and Down syndrome Nasal congestion Signs and Symptoms The signs and symptoms of OSA result from disruption of the normal sleep architecture. The frequent arousals and the inability to achieve or maintain the deeper stages of sleep can lead to excessive daytime sleepiness, nonrestorative sleep, automobile accidents, personality changes, decreased memory, erectile dysfunction (impotence), and depression.Patients rarely complain about frequent awakenings due to obstruction, but awakenings do occur. Excessive daytime sleepiness may be mild or severe, depending on the severity of the obstruction. Some patients suffering from OSA fall asleep in a nonstimulating environment, such as while reading in a quiet room. Others may fall asleep in a stimulating environment, such as during business meetings, eating, and even while having sex. Patients with OSA often complain of waking up feeling like they had never slept at all. They often feel worse after taking a nap than they did before napping. Decreased alertness places a person at risk in a variety of potentially hazardous situations. Other symptoms of OSA, such as morning headaches and frequent urination during the night, may be caused by apneic events themselves. The physical signs that suggest OSA include loud snoring, witnessed apneic episodes, and obesity. Patients with OSA often say that their only problem is that their bed partner complains about their snoring. A large number of snorers are believed to have OSA. Many times, a sleep partner will witness an apneic event. Hypertension is prevalent in patients with OSA, although the exact relationship is unclear. It has been shown, however, that treating OSA can modestly lower blood pressure. Not everybody who snore has sleep apnea, but if two or more of the above symptoms are present the person should consider consulting a sleep specialist. A high score on the Epworth Sleepiness Scale is also a strong indicator of possible sleep apnea. Complications The most obvious complication arising from OSA is diminished quality of life brought on by chronic sleep deprivation and the symptoms described above. Coronary artery disease, cerebral vascular accidents (strokes), and congestive heart failure are being evaluated to define the exact nature of their connection to OSA. Some linkage between OSA and coronary artery disease and stroke has been demonstrated, although it is still uncertain whether OSA leads to an increased risk of stroke and coronary artery disease or if both OSA and cardiovascular problems are caused by a common problem, such as obesity. Obstructive sleep apnea aggravates congestive heart failure by placing stress on the heart during sleep. There is a high prevalence of OSA in patients with congestive heart failure. Congestive heart failure patients also may have central sleep apnea, a condition in which the brain signals the patient to stop breathing for short periods of time. Diagnosis The primary method for diagnosing OSA at present is to have the patient undergo a sleep study, known as polysomnography. A sleep technician administers and attends the study. To prepare the patient for sleep study, numerous physiological monitors are attached to the patient to record nighttime breathing, brain activity, and physical activity. Several electrodes are pasted to the patient's head to measure brain electrical activity with an electroencephalogram, or EEG. Electrical activity in the brain during the different stages of sleep is distinctly different from that while awake. The EEG allows the physician to see if the patient is reaching all the stages of sleep to the appropriate depth and if the patient is being aroused excessively from these stages.Electrodes are also taped to the skin near the outer edges of the eyes to record data for an electrocculogram (EOG). This tells the examiner where the patient is in rapid eye movement sleep (REM). A device is placed near the patient's nose and mouth to measure airflow. Electrodes are connected to an electromyogram (EMG) and taped or pasted on the patient's chin to detect activity in the jaw muscles. The EMG detects the presence of REM sleep when the jaw muscles relax. Special belts are placed around the patient's chest and abdomen to detect and record the rising and falling movements associated with the respiration. A pulse oximeter, a noninvasive device for measuring Pathology Review Page 95 oxygen content in the blood, is attached to the finger, and electrodes to provide an electrocardiogram (ECG) are attached to the chest to measure heart rate. Various types of instruments, either straps around the feet or electrodes pasted to the lower legs, measure leg movements, which may indicate another sleep disorder called periodic limb movement disorder. Obstructive sleep apnea is diagnosed if the patient has an apnea index greater than 5, that is, has more than five apneic episodes per hour, or a respiratory disturbance index (RDI), the combination of apneas and hypopneas, greater than 10 per hour. In the appropriate clinical setting, sleep apnea can be diagnosed by an RDI between 5 and 10. Experts disagree somewhat on precisely where the diagnostic threshold lies, so a reliable diagnosis needs to be made in the context of the individual. Furthermore, the criteria are even less precise in children, making an individual approach to diagnosis even more important. Clinically speaking, an obstructive apnea is defined as a complete cessation of airflow for more than 10 seconds with persistent respiratory effort. An obstructive hypopnea is defined as a partial reduction in air flow of approximately 30% to 50% with persistent respiratory effort and a reduction in oxygen saturation by at least 3% to 4% and/or an arousal from sleep. Treatment Weight reduction, positional therapy, positive pressure therapy, surgical options, and oral appliances. Weight gain is a significant risk factor for the development of OSA. While sleep apnea usually can be corrected by weight loss, other factors involved in the pathophysiology of OSA, such as anatomic abnormalities, may cause the condition to persist. However, the vast majority of OSA cases can be improved, if not eliminated, with significant weight loss. The amount of weight a patient needs to lose to achieve these benefits varies. Positional therapy can be used to treat patients whose OSA is related to body positioning during sleep. Most people with sleep apnea have worse symptoms if they lie flat on their back during sleep. Indeed, most bed partners know this from experience and often try to make their partner move onto their side during the night to stop their snoring. There are several strategies which can help patients who have mild apnea only when lying on their back. One is to sew or attach a sock filled with tennis balls, length-wise down the back of their pajama top or nightshirt. This makes it uncomfortable for the sleeper to lie on their back, and they usually will move onto their side. Another technique is to use positional pillows to assist in sleeping on the side. Positional therapy has its limits, but it has been tried with success in some patients. Positive Pressure Therapy Positive airway pressure is a very effective therapy for obstructive sleep apnea. It has three forms: continuous positive airway pressure (CPAP), autotitration, and bi-level positive airway pressure. Regardless of the mechanism used it is desirable to use the lowest possible pressure to eradicate the sleep apnea. In most cases, positive airway pressure is easier to tolerate at lower pressures. Every patient requires a different pressure. To determine precisely the individual patient's optimum airway pressure, it is necessary to titrate the pressure to each individual patient during a polysomnogram. A polysomnogram will show not only when the respiratory events have ceased, but also when the arousals from the respiratory events occur. CPAP, the more common of the three therapy modes, usually is administered at bedtime through a nasal or facial mask held in place by Velcro straps around the patient's head. The mask is connected by a tube to a small air compressor about the size of a shoe box. The CPAP machine sends air under pressure through the tube into the mask, where it imparts positive pressure to the upper airways. This essentially "splints" the upper airway open and keeps it from collapsing. Approximately 55% of patients who use CPAP do so on a nightly basis for more than 4 hours. It is the most commonly prescribed treatment for OSA. The advantages of CPAP are that it is very safe and completely reversible. Generally, it is quite well tolerated. The main disadvantage is that it requires active participation every night; that is, the patient must put it on for it to work. Bi-level positive airway pressure is a variation of CPAP. Most of the problems patients experience with CPAP are caused by having to exhale against a high airway pressure. Because the air pressure required to prevent respiratory obstruction is typically less on expiration than on inspiration, bi-level positive airway pressure machines are designed to sense when the patient is inhaling and exhaling and to reduce the Pathology Review Page 96 pressure to a preset level on exhalation. Bi-level positive airway pressure machines usually are used when the patient does not tolerate CPAP or when the patient has more than one respiratory disorder. Oral Appliances Oral appliances used for the treatment of OSA generally come in two categories: mandibular advance devices and tongue-retaining devices. A variety of both types exists. Mandibular advance devices essentially consist of a plastic (or other material) mold of the teeth. Tongue-retaining devices also resemble an athletic mouth guard. The device is like a suction cup and is placed between the upper and lower teeth. Obesity hypoventilation syndrome (OHS) a condition that occurs in obese people, in which poor breathing leads to lower oxygen levels and higher carbon dioxide levels in the blood. The exact cause of OHS in unknown. Most (but not all) patients with the syndrome have a form of sleep apnea. OHS is believed to result from both a defect in the brain's control over breathing, and excessive weight (due to obesity) against the chest wall, which makes it hard for a person to take a deep breath. As a result, the blood has too much carbon dioxide and not enough oxygen. People with OHS are often tired due to sleep loss, poor sleep quality, and chronic hypoxia. Excess (morbid) obesity is the main risk factor. Symptoms The main symptoms of OHS are due to lack of sleep and include: Excessive daytime sleepiness Falling asleep during the day Increased risk for accidents or mistakes at work Depression Symptoms of low blood oxygen level (chronic hypoxia) can also occur, such as shortness of breath or feeling tired after very little effort. Exams and Tests People with OHS are usually very overweight. Symptoms of OHS include: Bluish color in the lips, fingers, toes, or skin (cyanosis) Signs of right-side heart failure (cor pulmonale), such as swollen legs or feet, shortness of breath, or feeling tired after little effort Reddish complexion A short, thick neck and small airway passage in the mouth Tests to confirm OHS include: Sleep study Lung (pulmonary) function Arterial blood gas Pleurisy and Other Disorders of the Pleura Pleurisy (PLUR-is-see) is inflammation (swelling) of the pleura. The pleura is a large, thin sheet of tissue (membrane) that wraps around the outside of your lungs and lines the inside of your chest cavity. Between the layer of the pleura that wraps around your lungs and the layer that lines your chest cavity is a very thin space. This is called the pleural space. Normally it's filled with a small amount of fluid—about 4 teaspoons full. The fluid helps the two layers of the pleura glide smoothly past each other as your lungs breathe air in and out. Pleurisy occurs when the two layers of the pleura become inflamed. Then they rub against each other every time your lungs expand to breathe in air. This can cause sharp pain with breathing. Infections like pneumonia are the most common cause of swelling, or inflammation, of the pleura and pleurisy. Other Disorders of the Pleura Pleural Effusion In some cases of pleurisy, excess fluid builds up in the pleural space. This is called a pleural effusion. The buildup of fluid usually forces the two layers of the pleura apart so they don't rub against each other Pathology Review Page 97 when you breathe. This can relieve your pain. However, a large amount of extra fluid can push the pleura against your lung until the lung, or a part of it, collapses. This can make it hard for you to breathe. In some cases of pleural effusion, the extra fluid gets infected and turns into an abscess. This is called an empyema (em-pi-E-ma). You can develop a pleural effusion if you don't have pleurisy. For example, pneumonia, heart failure, cancer, or a pulmonary embolism can lead to a pleural effusion. To remove a thorencentesis is performed. Pneumothorax Air or gas also can build up in the pleural space. This is called a pneumothorax. It can result from acute lung injury (car accident leading to rib tearing lung or bullet wound) or a lung disease like emphysema (over stretched alveolar sacs bursting). Lung procedures, like surgery, drainage of fluid with a needle, examination of the lung from the inside with a light and a camera, or mechanical ventilation, also can cause it (occurs accidentally by surgeon, usually while placing central lines). The most common symptom is sudden pain in one side of the lung and shortness of breath. A pneumothorax also can put pressure on the lung and cause it to collapse. If the pneumothorax is small, it may go away on its own (or by nitrogen wash out with 100% O2). If it's large, you may need to have a tube placed through your skin and chest wall into the pleural space to remove the air. First a needle decompression is performed to remove air then chest tube placed between 2nd and 3rd rib in the midclavicular line. Hemothorax Blood also can collect in the pleural space. This is called hemothorax (he-mo-THOR-aks). The most common cause is injury to your chest from blunt force or chest or heart surgery. Hemothorax also can occur in people with lung or pleural cancer. Hemothorax can put pressure on the lung and force it to collapse. It also can cause shock, a state in which not enough blood and oxygen reach important organs in the body. What Causes Pleurisy and Other Disorders of the Pleura? Pleurisy Many different conditions can cause pleurisy. Viral infection is the most common cause. Other conditions that can cause pleurisy are: Bacterial infections like pneumonia and Inflammatory bowel disease tuberculosis Familial Mediterranean fever, an inherited condition that often causes Autoimmune disorders like systemic lupus erythematosus and rheumatoid fever and swelling in the abdomen or arthritis lung Lung cancer, including lymphoma Infection from a fungus or parasite Other lung diseases like sarcoidosis, Heart surgery, especially coronary asbestosis, lymphangioleiomyomatosis, artery bypass grafting and mesothelioma Chest injuries Pulmonary embolism, a blood clot in the blood vessels that go into the lungs Pathology Review Page 98 Reactions to certain medicines that can cause a condition similar to systemic lupus erythematosus. These medicines include procainamide, hydralazine, and isoniazid. Pleural Effusion The most common cause of pleural effusion, or fluid in the pleural space, is congestive heart failure. Lung cancer, pneumonia, tuberculosis, and other lung infections also can cause swelling of the pleura and lead to a pleural effusion. Asbestosis, sarcoidosis, and reactions to some medicines also can lead to pleural swelling and pleural effusion. Pneumothorax A pneumothorax, or air in the pleural space, can be caused by lung diseases like chronic obstructive pulmonary disease (COPD), tuberculosis, and acute lung injury. Surgery or a wound or injury to the chest also may lead to a pneumothorax. Hemothorax The most common cause of hemothorax, or blood in the pleural space, is an injury to the chest. Cancer of the lung or pleura and chest or heart surgery also may lead to a hemothorax. Hemothorax also can be a complication of tuberculosis. What Are the Signs and Symptoms of Pleurisy and Other Disorders of the Pleura? Pleurisy The main symptom of pleurisy is a sharp or stabbing pain in your chest that gets worse when you breathe in deeply or cough or sneeze. The pain may stay in one place or it may spread to your shoulder or back. Sometimes it becomes a fairly constant dull ache. Depending on what's causing the pleurisy, you may have other symptoms, such as: Shortness of breath Unexplained weight loss A cough A sore throat followed by pain and swelling in your joints Fever and chills Rapid, shallow breathing Pneumothorax The symptoms of pneumothorax include: Sudden, sharp chest pain that gets Easy fatigue (tiredness) worse when you breathe in deeply or A rapid heart rate cough A bluish color of the skin caused by lack Shortness of breath of oxygen Chest tightness Other symptoms of pneumothorax include flaring of the nostrils; anxiety, stress, and tension; and hypotension (low blood pressure). Hemothorax The symptoms of hemothorax are often similar to those of pneumothorax. They include: Chest pain A rapid heart rate Shortness of breath Anxiety Respiratory failure Restlessness Computerized Tomography (CT) Scan This test provides a computer-generated picture of your lungs that can show pockets of fluid. It also may show signs of pneumonia, a lung abscess, or a tumor. Ultrasound This test uses sound waves to create pictures of your lungs. It may show where fluid is located in your chest. It also can show some tumors. Magnetic Resonance (MR) Scan This test also is called nuclear magnetic resonance (NMR) scanning or magnetic resonance imaging (MRI). It uses powerful magnets and radio waves to show pleural effusions and tumors. Blood Tests Blood tests can show whether you have a bacterial or viral infection, pneumonia, rheumatic fever, a pulmonary embolism, or lupus. Pathology Review Page 99 Arterial Blood Gas Tests and CXR are also taken to Dx Thoracentesis Once your doctor knows whether fluid has built up in the pleural space and where it is, he or she can remove a sample for testing. This test is called thoracentesis (THOR-a-sen-TE-sis). The doctor inserts a small needle or a thin, hollow, plastic tube through the ribs in the back of your chest into your chest wall and draws fluid out of your chest. Possible complications of thoracentesis include: Pneumothorax, or buildup of air in the pleural space, with a collapsed or partially collapsed lung. Sometimes air comes in through the needle or the needle makes a hole in your lung. Usually, a hole will seal itself. But sometimes air can build up around the lung and make it collapse. The doctor may need to use a chest tube to remove the air and let the lung expand again. Pain. Bleeding and bruising where the needle went in. In rare cases, bleeding may occur in or around the lung. The doctor may need to use a chest tube to drain the blood. In some cases, surgery may be needed. Infection where the needle went in. Liver or spleen injury. This is very rare. Fluid Analysis Doctors look at the fluid removed by thoracentesis under a microscope. They look at the chemicals in it and its color, texture, and clearness for signs of infection, cancer, or other conditions that may be causing the buildup of fluid or blood in the pleural space. Biopsy If your doctor thinks that tuberculosis or cancer may have caused the fluid buildup, he or she may want to look at a small piece of the pleura under a microscope. To take a tissue sample, the doctor may: Insert a needle through the skin on your chest to remove a small sample of the outer layer of the pleura. Insert a small tube with a light on the end (endoscope) into tiny cuts in your chest wall so that the doctor can see the pleura. He or she can then snip out small pieces of tissue. This procedure must be done in the hospital under general anesthesia. Snip out a sample of the pleura through a small cut in your chest wall. This is called an open pleural biopsy. It's usually done if the sample from the needle biopsy is too small for an accurate diagnosis. This procedure must be done in the hospital under general anesthesia. How Are Pleurisy and Other Disorders of the Pleura Treated? Goals of Treatment The goals of treatment are to: Remove the fluid, air, or blood from the pleural space Relieve symptoms Treat the underlying condition Remove Fluid, Air, or Blood From the Pleural Space If large amounts of fluid, air, or blood aren't removed from the pleural space, they may put pressure on your lung and cause it to collapse. The procedures used to drain fluid, air, or blood from the pleural space are similar. During thoracentesis, the doctor inserts a needle or a thin, hollow, plastic tube through the ribs in the back of your chest into your chest wall. A syringe is attached to draw fluid out of your chest. This procedure can remove more than 6 cups of fluid at a time. When larger amounts of fluid must be removed, a chest tube may be inserted through your chest wall. The doctor injects a local painkiller into the area of your chest wall outside where the fluid is. He or she will then insert a plastic tube into your chest between two ribs. The tube is connected to a box that suctions the fluid out. A chest x ray is taken to check the tube's position. A chest tube also is used to drain blood and air from the pleural space. This can take several days. The tube is left in place, and you usually stay in the hospital during this time. Pathology Review Page 100 Sometimes the fluid contains pus that is very thick or blood clots. Or it may have formed a hard skin or peel. This makes it harder to drain the fluid. To help break up the pus or blood clots, the doctor may use the chest tube to put certain medicines into the pleural space. These medicines are called fibrinolytics. If the pus or blood clots still don't drain out, you may need surgery. Treating the Underlying Condition Looking at the fluid under a microscope can often tell the doctor what's causing the fluid buildup. Then treatment of the underlying condition can begin. If the fluid is infected, treatment involves antibiotics and draining the fluid. If the infection is tuberculosis or from a fungus, treatment involves long-term use of antibiotics or antifungal medicines. If the fluid is caused by tumors of the pleura, it may build up again quickly after it's drained. Sometimes antitumor medicines will prevent further fluid buildup. If they don't, the doctor may seal the pleural space. This is called pleurodesis. In pleurodesis, the doctor drains all the fluid out of the chest through a chest tube. Then he or she pushes a substance through the chest tube into the pleural space. This substance irritates the surface of the pleura. This causes the two layers of the pleura to squeeze shut so there is no room for more fluid to build up. Chemotherapy or radiation treatment also may be used to reduce the size of the tumors. Pneumonitis Pneumonitis is a general term that refers to inflammation of lung tissue. Pneumonia is one type of pneumonitis caused by an infection. Many other factors can cause pneumonitis, including breathing in animal dander, inhaling small food particles "down the wrong pipe" and receiving radiation therapy to your chest. A cough and difficulty breathing are the most common signs and symptoms of pneumonitis, but having these problems doesn't necessarily mean that you have pneumonitis. Specialized tests are often necessary to make a diagnosis. Medications are used to treat some causes of pneumonitis, but other types of pneumonitis can be cured by simply avoiding the substance that triggers inflammation. Identifying and treating pneumonitis promptly is important, because untreated pneumonitis can lead to lung scarring and permanent difficulty breathing. Signs and symptoms Cough Chills Difficulty breathing A general feeling of being unwell A low-grade fever Other signs and symptoms depend on the type of pneumonitis that you develop. For example, if you breathe in chlorine gas, causing chemical pneumonitis, you may experience a burning feeling in your chest. Chest pain is a feature of radiation-induced pneumonitis. Signs and symptoms of pneumonitis may last anywhere from several hours to days or even longer. Chronic pneumonitis If pneumonitis is left undetected or untreated, you may develop chronic pneumonitis. This is especially common in cases of hypersensitivity pneumonitis, in which you may be continually exposed to an allergen without realizing it. Signs and symptoms of chronic pneumonitis include: Shortness of breath while exercising Loss of appetite Fever Unintentional weight loss Chills In severe cases, you may begin breathing rapidly (tachypnea) and with difficulty in an effort to meet your body's oxygen demands. Causes Pneumonitis occurs when some irritating substance — solid particles, liquids, gases, radiation or bacteria — cause the tiny air sacs to become inflamed. This can hamper the exchange of oxygen and carbon dioxide in the air sacs. There are several types of pneumonitis. They include: Aspiration pneumonitis. Aspiration pneumonitis occurs when you inhale (aspirate) foreign matter into your lungs. Stomach contents, such as ingested food or liquid, are a frequent cause Pathology Review Page 101 of aspiration pneumonitis. Accidental inhalation of small food particles, such as tiny pieces of peanuts or vegetables, while swallowing is common in young children. Chemical pneumonitis is a type of aspiration pneumonitis that develops when you inhale chemicals that are toxic to your lungs. Industrial and household chemicals, such as chlorine gas, ammonia, solvents and pesticides can all cause chemical pneumonitis. Hypersensitivity pneumonitis. This type of pneumonitis is nicknamed "farmer's lung," "mushroom picker's disease" and other colorful names with good reason. Dust from animal dander, molds and plants, all potential allergens, can provoke an inflammatory reaction in your lungs. Symptoms usually develop within six hours of exposure to the allergen. Some people are more susceptible to developing hypersensitivity pneumonitis than are others, although it's not certain why. Radiation pneumonitis. A few people who undergo radiation therapy for lung cancer, breast cancer, leukemia or lymphoma develop this type of pneumonitis. Taking some types of chemotherapy drugs (doxorubicin, cyclophosphamide, bleomycin) during radiation treatment may increase your risk of developing radiation-induced pneumonitis. Drug-induced pneumonitis. A variety of drugs used to treat other conditions can cause pneumonitis. These include certain chemotherapy drugs (bleomycin, methotrexate, carmustine, busulfan, cyclophosphamide), antibiotics (nitrofurantoin, amphotericin B, minocycline), sulfonamides (sulfasalazine, sulfadiazine), nonsteroidal anti-inflammatory drugs (NSAIDs) and the heart medication amiodarone. Risk factors Risk factors for developing aspiration pneumonitis include situations that increase your chances of inhaling solids or liquids, especially stomach contents, into your lungs. Seizures, vomiting and undergoing anesthesia are common causes. Others include: Medical procedures. A tracheostomy, bronchoscopy or other procedure can interfere with the normal function of the opening at the back of your throat (glottis) leading into the trachea. Other disorders. Neurological diseases that can affect your ability to swallow, including multiple sclerosis and Parkinson's disease, or any disorder of the upper gastrointestinal tract, such as gastroesophageal reflux disease (GERD), can lead to aspiration pneumonitis. The incidence of chemical pneumonitis is usually associated with certain occupational exposures. Cleaning with chlorine bleach in a poorly ventilated area, performing certain types of factory work, such as smelting or welding, and exposure to pesticides or solvents increase the risk of chemical pneumonitis. In addition, receiving radiation or chemotherapy or taking some types of drugs increases your risk of developing radiation or drug-induced pneumonitis. Screening and diagnosis To help determine whether you've been exposed to a substance that may have provoked your signs and symptoms, your doctor will ask you questions about your past and current occupations and your environment, including where you work, if you have pets and whether you've been diagnosed with any allergies. You'll undergo a physical exam, including blood tests, and imaging tests such as a chest X-ray or a computerized tomography (CT) scan of your lungs. To distinguish pneumonitis from other lung disorders, you'll likely have one or more of the following tests: Pulmonary function tests. This refers to a series of tests that evaluate how well your lungs are working. A common pulmonary function test, called a spirometry, measures the amount of air that you're able to inhale and exhale in a specific period of time. Your doctor may also measure how efficiently your lungs transfer gases from the air into the bloodstream (diffusion capacity). Bronchoscopy. A bronchoscopy is a procedure used to view your airways and collect samples from your lungs. In order to make you as comfortable as possible during the procedure, your doctor will spray an anesthetic in the back of your mouth and throat and will give you medication through a vein in your arm to help you relax. A flexible tube called a bronchoscope is passed through your mouth or nose and then down into your lungs. Once the tube is in place, your doctor may flush a section of your lung with a saltwater solution (saline) to collect lung cells and Pathology Review Page 102 other materials. This "flushing" procedure is known as a lavage. Your doctor may also insert a tiny needle or brush through the bronchoscope to scrape a sample of cells from the lung tissue (transbronchoscopic lung biopsy). Complications Pneumonitis that goes unnoticed or untreated can cause irreversible lung damage. Normally, air sacs within your lungs stretch and relax with each breath. Chronic inflammation of the thin tissue lining each air sac can make the air sacs scar and become inflexible. This is called pulmonary fibrosis. In severe cases, pulmonary fibrosis can cause heart failure, respiratory failure and death. Treatment If you have hypersensitivity or chemical pneumonitis, avoiding allergens is a common-sense approach to treatment. By eliminating exposure to the allergen or chemical irritating your lungs, you'll likely notice that your symptoms lessen. In severe cases of pneumonitis, treatment may include: Corticosteroids. Drugs that reduce inflammation, such as prednisone, can help relieve the symptoms of pneumonitis. Corticosteroids are usually taken as a pill. These drugs work by suppressing your immune system, reducing inflammation in your lungs. However, corticosteroid use also increases your risk of developing infections and is associated with the thinning of bones (osteoporosis). Antibiotics. Having pneumonitis makes you more susceptible to developing bacterial infections in your lungs. The type of antibiotic you'll receive depends on the bacteria causing the infection. Antibiotic therapy may be given through a vein in your arm or taken as a pill. Oxygen therapy. People who have chronic pneumonitis with fibrosis and can't breathe well usually require oxygen therapy. Oxygen therapy involves wearing a mask or a plastic tubing (cannula) with prongs that fit into your nostrils that provides breathing air rich in oxygen. Some people need oxygen therapy constantly, while others might need it only during exercise or sleep. Pulmonary edema (Cardiogenic and non-cardiogenic) In most cases, heart problems cause pulmonary edema. But fluid can accumulate for other reasons, including pneumonia, exposure to certain toxins and medications, and exercising or living at high elevations. Acute pulmonary edema is a medical emergency requiring immediate care. Although pulmonary edema can sometimes prove fatal, the outlook is often good when you receive prompt treatment for pulmonary edema along with therapy for the underlying problem. Signs and symptoms Depending on the cause, pulmonary edema symptoms may appear suddenly or develop slowly over weeks or months. Signs and symptoms that come on suddenly are usually severe and may include: Extreme shortness of breath or A cough that produces frothy sputum difficulty breathing that may be tinged with blood A feeling of suffocating or drowning Excessive sweating Wheezing or gasping for breath Pale skin Anxiety, restlessness, a sense of Chest pain when pulmonary edema is apprehension caused by coronary artery disease Signs and symptoms that develop more gradually include: Difficulty breathing when you're lying flat as opposed to sitting up. Awakening at night with a breathless feeling. Having more shortness of breath than normal when you're physically active. Significant weight gain when pulmonary edema develops as a result of congestive heart failure, a condition in which your heart pumps too little blood to meet your body's needs. The weight gain is from accumulation of fluid in your body, especially in your legs. Causes Two major airways (bronchi) carry air into your lungs. These airways subdivide into smaller airways (bronchioles) that finally end in clusters of tiny air sacs called alveoli. These air sacs inflate like miniature balloons every time you inhale. Pathology Review Page 103 Wrapped around each air sac are capillaries that connect the arteries and veins in your lungs. The capillaries are so narrow that red blood cells have to pass through them in single file. Each red blood cell absorbs oxygen, while the plasma — the fluid containing the red blood cells — releases carbon dioxide as well as absorbs some of the oxygen. But in certain circumstances, the alveoli fill with fluid instead of air, preventing oxygen from being absorbed into your bloodstream. A number of factors can cause fluid to accumulate in your lungs, but most have to do with your heart (cardiac pulmonary edema). Understanding the relationship between your heart and lungs can help explain why. How your heart works Your heart is composed of two upper and two lower chambers. The upper chambers (the right and left atria) receive incoming blood and pump it into the lower chambers. The lower chambers, the more muscular right and left ventricles, pump blood out of your heart. The heart valves — which keep blood flowing in the correct direction — are gates at the chamber openings. Normally, deoxygenated blood from your body enters the right atrium and flows into the right ventricle, where it's pumped through large blood vessels (pulmonary arteries) to your lungs. There, the blood releases carbon dioxide and picks up oxygen. The oxygen-rich blood then returns to the left atrium through the pulmonary veins, flows through the mitral valve into the left ventricle, and finally leaves your heart through another large artery, the aorta. The aortic valve at the base of the aorta keeps the blood from flowing backward into your heart. From the aorta, the blood travels to the rest of your body. What goes wrong Cardiac pulmonary edema — also known as congestive heart failure — occurs when the left ventricle isn't able to pump out enough of the blood it receives from your lungs. As a result, pressure increases inside the left atrium and then in the pulmonary veins and capillaries, causing fluid to be pushed through the capillary walls into the air sacs. Congestive heart failure can also occur when the right ventricle is unable to overcome increased pressure in the pulmonary artery, which usually results from left heart failure, chronic lung disease or high blood pressure in the pulmonary artery (pulmonary hypertension). Medical conditions that can cause the left ventricle to become weak and eventually fail include: Coronary artery disease. Over time, the arteries that supply blood to your heart can become narrow from fatty deposits (plaques). A heart attack occurs when a blood clot forms in one of these narrowed arteries, blocking blood flow and damaging the portion of your heart muscle supplied by that artery. The result is that the damaged heart muscle can no longer pump as well as it should. Although the rest of your heart tries to compensate for this loss, it's either unable to do so effectively or it's weakened by the extra workload. When the pumping action of your heart is weakened, blood backs up into your lungs, forcing fluid in your blood to pass through the capillary walls into the air sacs. Cardiomyopathy. When your heart muscle is damaged by causes other than blood flow problems, the condition is called cardiomyopathy. Often, cardiomyopathy has no known cause, although it sometimes runs in families. Less common causes include infections (myocarditis), alcohol abuse and the toxic effects of drugs such as heroin and some types of chemotherapy. Because cardiomyopathy weakens the left ventricle — your heart's main pump — your heart may not be able to respond to conditions that require it to work harder, such as a surge in blood pressure or infections. When the left ventricle can't keep up with the demands placed on it, fluid backs up into your lungs. Heart valve problems. In mitral valve disease or aortic valve disease, the valves that regulate blood flow either don't open wide enough (stenosis) or don't close completely (insufficiency). This allows blood to flow backward through the valve. When the valves are narrowed, blood can't flow freely into your heart and pressure in the left ventricle builds up, causing the left ventricle to work harder and harder with each contraction. The increased pressure extends into the left atrium and then to the pulmonary veins, causing fluid to accumulate in your lungs. On the other hand, if the mitral valve leaks, some blood is Pathology Review Page 104 backwashed toward your lung each time your heart pumps. If the leakage develops suddenly, you may develop sudden and severe pulmonary edema. High blood pressure (hypertension). Untreated or uncontrolled high blood pressure causes a thickening of the left ventricular muscle, and accelerates coronary artery disease. Noncardiac pulmonary edema Not all pulmonary edema is the result of heart disease. Fluid may also leak from the capillaries in your lungs' air sacs because the capillaries themselves become more permeable or leaky, even without the buildup of back pressure from your heart. In that case, the condition is known as noncardiac pulmonary edema because your heart isn't the cause of the problem. Some factors that can cause increased capillary permeability leading to noncardiac pulmonary edema are: Lung infections. When pulmonary edema results from lung infections, such as pneumonia, the edema occurs only in the part of your lung that's inflamed. Exposure to certain toxins. These include toxins you inhale — such as chlorine, ammonia or nitrogen dioxide — as well as those that may circulate within your own body. For example, women giving birth may develop pulmonary edema when amniotic fluid reaches the lungs through the veins of the uterus (amniotic fluid embolism). Kidney disease. When your kidneys can't remove waste effectively, excess fluid can build up, causing pulmonary edema. Smoke inhalation. Smoke contains chemicals that damage the membrane between the air sacs and the capillaries, allowing fluid to enter your lungs. Adverse drug reaction. Many drugs — ranging from narcotics such as heroin to diabetes medications and chemotherapy drugs — are known to cause noncardiac pulmonary edema. Acute respiratory distress syndrome (ARDS). This serious disorder occurs when your lungs suddenly fill with fluid and inflammatory blood cells. Many conditions can cause ARDS, including severe injuries (trauma), systemic infection (sepsis), pneumonia and shock. ARDS sometimes develops after extensive surgery. High altitudes. Mountain climbers and people who live in or travel to high-altitude locations run the risk of developing high-altitude pulmonary edema (HAPE). This condition — which typically occurs at elevations above 8,000 feet — can also affect hikers or skiers who start exercising at higher altitudes without first becoming acclimated. But even people who have hiked or skied at high altitudes in the past aren't immune. Although the exact mechanism isn't completely understood, HAPE seems to develop as a result of increased pressure from constriction of the pulmonary capillaries. Symptoms include headaches, insomnia, fluid retention, cough and shortness of breath. Without appropriate care, HAPE can be fatal. When to seek medical advice Acute pulmonary edema is life-threatening. Get emergency assistance if you have any of the following acute signs and symptoms: Trouble breathing or a feeling of Breathing difficulty along with profuse suffocating (dyspnea) sweating A bubbly, wheezing or gasping sound A blue or gray tone to your skin when you breathe A severe drop in blood pressure Pink, frothy sputum when you cough Acute pulmonary edema is likely to be incapacitating, so don't attempt to drive yourself to the hospital. Instead, dial 911 or emergency medical care and wait for help. Screening and diagnosis Because pulmonary edema requires prompt treatment, you'll initially be diagnosed on the basis of your symptoms and a physical exam. You may also have blood drawn — usually from an artery in your wrist — so that it can be checked for the amount of oxygen and carbon dioxide it contains (arterial blood gas concentrations). Once your condition is more stable, your doctor will ask about your medical history, especially whether you have ever had cardiovascular or lung disease. You'll also likely have a chest X-ray, which can help Pathology Review Page 105 support a diagnosis of pulmonary edema. And you may have further tests to determine why you developed fluid in your lungs. These tests may include: Electrocardiography (ECG). This noninvasive test can reveal a wide range of information about your heart. During an ECG, patches attached to your skin receive electrical impulses from your heart. These are recorded in the form of waves on graph paper or a monitor. The wave patterns show your heart rate and rhythm, and whether areas of your heart show diminished blood flow. Echocardiography (diagnostic cardiac ultrasound exam). Another noninvasive test, echocardiography uses a wand-shaped device called a transducer to generate high-frequency sound waves that are reflected from the tissues of your heart. The sound waves are then sent to a machine that uses them to compose images of your heart on a monitor. The test can help diagnose a number of heart problems, including valve problems, abnormal motions of the ventricular walls, fluid around the heart (pericardial effusion) and congenital heart defects. It also accurately measures the amount of blood your left ventricle ejects with each heartbeat (ejection fraction, or EF). Although a low EF often indicates a cardiac cause for pulmonary edema, it's possible to have cardiac pulmonary edema with a normal EF. Transesophageal echocardiography (TEE). In a traditional cardiac ultrasound exam, the transducer remains outside your body on the chest wall. But in TEE, a soft, flexible tube with a special transducer tip is inserted through your mouth and into your esophagus — the passage leading to your stomach. The esophagus lies immediately behind your heart, which allows for a closer and more accurate picture of your heart and central pulmonary arteries. You'll be given a sedative to make you more comfortable and prevent gagging. You may have a sore throat for a few days after the procedure, and there's a slight risk of perforation or bleeding from the esophagus. Pulmonary artery catheterization. If other tests don't reveal the reason for your pulmonary edema, your doctor may suggest a procedure to measure the pressure in your lung capillaries (wedge pressure). During this test, a small, balloon-tipped catheter is inserted through a vein in your leg or arm into a pulmonary artery. The catheter has two openings connected to pressure transducers. The balloon is inflated and then deflated, giving pressure readings. Complications If pulmonary edema persists, it can raise pressure in the pulmonary artery and eventually the right ventricle begins to fail. The right ventricle has a much thinner wall of muscle than does the left side. The increased pressure backs up into the right atrium and then into various parts of your body, where it can cause: Leg swelling (edema) Abdominal swelling (ascites) Buildup of fluid in the membranes that surround your lungs (pleural effusion) When not treated, acute pulmonary edema can be fatal. In some instances it may be fatal even if you receive treatment. The outcome depends in part on the condition of your heart and lungs before you developed edema and on the amount of fluid in your lungs. Drug-induced pulmonary edema can be a cause of death in people who abuse narcotics. Treatment Administering oxygen is the first step in the treatment for pulmonary edema. If bad enough the use of BIPAP may be indicated with generalized settings of 15/5, rate 16, 100% o2. Depending on your condition and the reason for your pulmonary edema, you may also receive one or more of the following medications: Preload reducers. Doctors commonly use nitroglycerin and diuretics, such as furosemide (Lasix), to treat pulmonary edema. These medications dilate the veins in your lungs and elsewhere in your body, which decreases fluid pressure going into your heart and lungs. Morphine (Astramorph, Roxanol). This narcotic, for years a mainstay in treating cardiac pulmonary edema, may be used to relieve shortness of breath and associated anxiety. But some doctors believe that the risks of morphine may outweigh the benefits and are more apt to use other, more effective, drugs. Pathology Review Page 106 Afterload reducers. These drugs dilate the peripheral vessels and take a pressure load off the left ventricle. Some examples of afterload reducer medications include nitroprusside (Nitropress), enalapril (Vasotec) and captopril (Capoten). Aspirin. Your doctor may recommend starting aspirin therapy if you're not already taking it. Aspirin helps thin your blood so that it moves through your small blood vessels more easily. Blood pressure medications. If you have high blood pressure when you develop pulmonary edema, you'll be given medications to control it. On the other hand, if your blood pressure is too low, you're likely to be given drugs to raise it. Pulmonary Embolism A pulmonary embolism or PE, is a sudden blockage in a lung artery, usually due to a blood clot that traveled to the lung from a vein in the leg. A clot that forms in one part of the body and travels in the bloodstream to another part of the body is called an embolus. PE can cause: Permanent damage to part of your lung from lack of blood flow to lung tissue Low oxygen levels in your blood leading to tissue necrosis and brain damage Death (if there are multiple clots they may travel to the heart and brain as well) In most cases, PE is a complication of a condition called deep vein thrombosis (DVT). In DVT, blood clots form in the deep veins of the body—most often in the legs. These clots can break free, travel through the bloodstream to the lungs, and block an artery. DVTs occur as a result of inactivity for extended periods of time (such as after surgery, truck drivers, long plan trips…).This is unlike clots in the veins close the skin's surface, which remain in place and do not cause PE. Major Risk Factors People at high risk for a blood clot that travels to the lungs are those who: Have deep vein thrombosis (DVT, a blood clot in the leg) or a history of DVT Have had PE before People who recently have been treated for cancer or who have a central venous catheter are more likely to develop DVT. The same is true for people who have been bedridden or have had surgery or suffered a broken bone in the past few weeks. Other risk factors for DVT, which can lead to PE, include sitting for long periods of time; pregnancy and the 6-week period after pregnancy, and being overweight or obese. Women who take hormone therapy or birth control pills also are at increased risk for DVT. What Are the Signs and Symptoms of Pulmonary Embolism? Pathology Review Page 107 Major Signs and Symptoms Signs and symptoms of pulmonary embolism (PE) include unexplained shortness of breath, difficulty breathing, chest pain, coughing, or coughing up blood and arrhythmias (PVCs). In some cases, the only signs and symptoms are related to deep vein thrombosis (DVT). These include swelling of the leg or along the vein in the leg, pain or tenderness in the leg, a feeling of increased warmth in the area of the leg that's swollen or tender, and red or discolored skin on the affected leg. How Is Pulmonary Embolism Diagnosed? Medical History and Physical Exam Identify your risk factors for deep vein thrombosis (DVT) and PE See how likely it is that you could have PE Rule out other possible causes for your symptoms Diagnostic Tests Ultrasound. Doctors use this test to look for blood clots in your legs. Ultrasound uses highfrequency sound waves to check the flow of blood in your veins. A gel is put on the skin of your leg. A hand-held device called a transducer is placed on the leg and moved back and forth over the affected area. The transducer gives off ultrasound waves and detects their echoes after they bounce off the vein walls and blood cells. A computer then turns the echoes of the ultrasound waves into a picture on a computer screen, where your doctor can see the blood flow in your leg. If blood clots are found in the deep veins of your legs, you will begin treatment. DVT and PE are both treated with the same medicines. Spiral CT scan or CT angiogram. Doctors use this test to look for blood clots in your lungs and in your legs. Dye is injected into a vein in your arm to make the blood vessels in your lungs and legs more visible on the x-ray image. While you lie on a table, an x-ray tube rotates around you, taking pictures from different angles. This test allows doctors to detect PE in most patients. The test only takes a few minutes. Results are available shortly after the scan is completed. Ventilation-perfusion lung scan (VQ scan). Doctors use this test to detect PE. The VQ scan uses a radioactive material to show how well oxygen and blood are flowing to all areas of the lungs. Pulmonary angiography is another test used to diagnose PE. It's not available at all hospitals, and a trained specialist must perform the test. A flexible tube called a catheter is threaded through the groin (upper thigh) or arm to the blood vessels in the lungs. Dye is injected into the blood vessels through the catheter. X-ray pictures are taken to show the blood flow through the blood vessels in the lungs. If a clot is discovered, the doctor may use the catheter to extract it or deliver medicine to dissolve it. Certain blood tests may help the doctor find out whether you're likely to have PE. A D-dimer test measures a substance in the blood that's released when a clot breaks up. High levels of the substance mean there may be a clot. If your test is normal and you have few risk factors, PE isn't likely. Other blood tests check for inherited disorders that cause clots and measure the amount of oxygen and carbon dioxide in your blood (arterial blood gas). A clot in a blood vessel in your lung may lower the level of oxygen in your blood. To rule out other possible causes of your symptoms, the doctor may use one or more of the following tests. Echocardiogram uses sound waves to check heart function and to detect blood clots inside the heart. EKG (electrocardiogram) measures the rate and regularity of your heartbeat. Chest x ray provides a picture of the lungs, heart, large arteries, ribs, and diaphragm. Magnetic resonance imaging (MRI) uses radio waves and magnetic fields to make pictures of organs and structures inside the body. In many cases, an MRI can provide information that can't be seen on an x ray. How Is Pulmonary Embolism Treated? Goals of Treatment The main goals of treating pulmonary embolism (PE) are to: Pathology Review Page 108 Stop the blood clot from getting bigger Keep new clots from forming Treatment may include medicines to thin the blood and slow its ability to clot. If your symptoms are life threatening, the doctor may give you medicine to dissolve the clot more quickly. Rarely, the doctor may use surgery or another procedure to remove the clot. Specific Types of Treatment Medicines Anticoagulants which are blood-thinning medicines, decrease your blood's ability to clot. They're used to stop blood clots from getting bigger and to prevent clots from forming. They don't break up blood clots that have already formed. (The body dissolves most clots with time.)Anticoagulants can be taken as either a pill, an injection, or through a needle or tube inserted into a vein (called intravenous, or IV, injection). Warfarin is given in a pill form. (Coumadin® is a common brand name for warfarin.) Heparin is given as an injection or through an IV tube. Your doctor may treat you with both heparin and warfarin at the same time. Heparin acts quickly. Warfarin takes 2 to 3 days before it starts to work. Once warfarin starts to work, usually the heparin will be stopped. Pregnant women usually are treated with heparin only, because warfarin is dangerous for the pregnancy. If you have deep vein thrombosis, treatment with anticoagulants usually lasts for 3 to 6 months. If you have had blood clots before, you may need a longer period of treatment. If you're being treated for another illness, such as cancer, you may need to take anticoagulants as long as risk factors for PE are present. The most common side effect of anticoagulants is bleeding. This happens if the medicine thins your blood too much. This side effect can be life threatening. Sometimes, the bleeding can be internal. This is why people treated with anticoagulants usually receive regular blood tests. These tests are called PT and PTT tests, and they measure the blood's ability to clot. These tests also help the doctor make sure you're taking the right amount of medicine. Call your doctor right away if you have easy bruising or bleeding. Thrombin inhibitors are a newer type of anticoagulant medicine. They're used to treat some types of blood clots for patients who can't take heparin. Emergency Treatment When PE is life threatening, doctors may use treatments that remove or break up clots in the blood vessels of the lungs. These treatments are given in the emergency room or in the hospital. Thrombolytics are medicines given to quickly dissolve a blood clot. They're used to treat large clots that cause severe symptoms. Because thrombolytics can cause sudden bleeding, they're used only in life-threatening situations. In some cases, the doctor may use a catheter to reach the blood clot. A catheter is a flexible tube placed in a vein to allow easy access to the bloodstream for medical treatment. The catheter is inserted into the groin (upper thigh) or arm and threaded through a vein to the clot in the lung. The catheter may be used to extract the clot or deliver medicine to dissolve it. Rarely, surgery may be needed to remove the blood clot. Other Types of Treatment When you can't take medicines to thin your blood, or when you're taking blood thinners but continue to develop clots anyway, the doctor may use a device called a vena cava filter to keep clots from traveling to your lungs. The filter is inserted inside a large vein called the inferior vena cava (the vein that carries blood from the body back to the heart). The filter catches clots before they travel to the lungs. This prevents PE, but it doesn't stop other blood clots from forming. Graduated compression stockings can reduce the chronic (ongoing) swelling that may occur after a blood clot has developed in a leg. The leg swelling is due to damage to the valves in the leg veins. Graduated compression stockings are worn on the legs from the arch of the foot to just above or below the knee. These stockings are tight at the ankle and become looser as they go up the leg. This causes a gentle compression (or pressure) up the leg. The pressure keeps blood from pooling and clotting. Pathology Review Page 109 Pneumonia Types of Infectious Pneumonia General considerations Pneumonias can be classified by the etiologic agent, by the type of host reaction to injury or, most commonly in pathology, by the morphologic pattern of infection: bronchopneumonia (bacterial); lobar pneumonia (bacterial); interstitial pneumonia (viral); granulomatous pneumonia (mycobacterial and fungal). The most important classification clinically is by etiologic agent since the traditional anatomic distinctions have little clinical relevance. Community-acquired infections are usually contrasted with nosocomial (hospital-acquired) infections of the lungs in terms of predicting etiologic agents and thus clinical course. Bronchopneumonia Bronchopneumonia corresponds to patchy consolidation of the lung since infection is centered around a bronchus or bronchiole and involves the immediately adjacent alveoli. Histologically, the response is suppurative inflammation. Bronchopneumonia can be so extensive and confluent as to overlap with lobar pneumonia. Spread of infection is through the airways. The pleura is usually not involved. Patients are usually younger or older, are febrile, and have a cough productive of sputum. A true sputum sample is necessary to aid in the diagnosis, since an inadequate sample may yield only normal mouth flora. Finding intracellular bacteria within macrophages or neutrophils from an adequate sputum sample is highly indicative of pneumonia. Culture is necessary for identification of the organism and determination of antibiotic sensitivities. In many cases, the microbiologic agent is never identified. Certain organisms tend to be associated with distinctive histopathological changes as well as clinical courses. Some examples are: a. Pseudomonas aeruginosa - tends to infect the lung hematogenously, causing infection and necrosis of vascular walls which results characteristically in a hemorrhagic pneumonia . Pseudomonas infection is common in burn and immunocompromised patients, and often has a fulminant course. Pseudomonas also occurs in patients with cystic fibrosis, where spread is airborne and the result again is usually fatal. b. Legionella pneumonia has characteristic morphology described as an acute fibrinopurulent exudative pneumonia by which there is a mixture of neutrophils and macrophages, within a background fibrinous exudate. The inflammatory response tends to spare the alveolar walls, so necrosis and hemorrhage do not generally occur. Legionella is a short gram-negative coccobacillus which is difficult to visualize in tissue. Legionella was first recognized in 1976 after an outbreak of cases in Philadelphia. Since that time at least 23 species and 49 serogroups have been identified and some have been attributed to other outbreaks such as "Pontiac fever." All environmental sources of legionella relate to water and include cooling towers, evaporative condensers, and potable water, especially hot water. Legionella often has an acute onset, but is usually responsive to appropriate antibiotics. Serology, culture, and immunofluorescence of sputum are now available for identification of the organism. Histopathologic Variants of Bronchopneumonia are: c. neutrophilic (exudative) : which is the common appearance of pneumonia in which neutrophils fill alveoli. If the causative organism is recognized & treated with the appropriate antibiotic, resolution with preservation of the lung architecture generally occurs. d. histiocytic: characteristic of legionella and mycoplasma; macrophages predominate. e. With Hyaline Membranes: usually seen in interstitial viral pneumonias, with the exception of streptococcal and E. coli. f. with coagulative necrosis: g. with abscess formation: h. with hemorrhage: particularly seen with Pseudomonas (septic vasculitis), and sometimes with Klebsiella. Pathology Review Page 110 i. with leukopenia: particularly in leukemic patients or other patients on chemotherapy in which there is profound iatrogenic neutropenia. These patients will get a pneumonia in which edema and many organisms are found in the absence of cellular infiltrates. j. with granulomas: (will be discussed under infectious granulomas of the lung). Lobar pneumonia Lobar pneumonia is so named because the inflammatory process, also suppurative, tends to involve an entire lobe uniformly. In some cases however, the inflammation may extend to other lobes or be incomplete within a single lobe and be difficult to distinguish from bronchopneumonia. The causative organism in about 95% of the cases of lobar pneumonia is Streptococcus pneumoniae and hence the process is synonymous with pneumoccocal pneumonia, although pneumococcus can also result in a bronchopneumonia. Classically, the inflammatory consolidation uniformly involves the entire lobe and is often accompanied by a reactive fibrinous pleuritis. Pathogenetically, this infection spreads through the interalveolar pores; microorganisms enter the lung by inhalation and initially cause a profuse exudation of fluid which spreads rapidly and uniformly throughout the lobe, providing a good medium for bacterial growth. Pneumococcus, a gram-positive diplococcus has a thick polysaccharide coat which resists phagocytosis. This spread can involve the entire lobe within a matter of hours. Four stages of the disease are recognized and are based on the gross appearance of the lung. k. Stage of congestion: at12-24 hours; edema . l. Stage of red hepatization: 2-3 days; redness is due to intense congestion, and a bit of hemorrhage. Because of the fibrinous-neutophilic exudate , the lung solidifies and is firm, having the consistency of liver, whence the term, hepatization. m. Stage of gray hepatization: 3-4 days: gray is due to increased numbers of white cells and fibrin and decreased intensity of blood in the lungs with compression of capillaries. n. Stage of resolution: 2-5 days; follows macrophage phagocytosis and clearance, and usually restitution of the normal pulmonary architecture. o. Organization : may be more prevalent since the advent of antibiotics, which are thought to alter normal inflammatory-repair mechanisms, resulting in organization and fibrosis rather than pure resolution`. Alternatively, organization may be due to superinfection by other organisms. With organization, tufts of fibrous tissue fill distal airways and alveoli. Later this fibrous tissue is incorporated into the interstitium, resulting in interstitial fibrosis and remodeling of the normal architecture. Complications of lobar pneumonia: 20-30% get bacteremias, which can lead to meningitis, endocarditis, arthritis, and a variety of infections elsewhere in the body. A significant proportion of those that get bacteremia die despite antibiotic therapy. As an infectious process, lobar pneumonia often presents with fever, chills, and a productive cough. Sputum may vary from watery to rusty as the disease advances. With loss of functional parenchyma, patients become short of breath and may be cyanotic. Pleural involvement may cause pain or a friction rub. Antibiotics alter or interrupt the nature progression of the disease and may be responsible for scarring of the lung when its occurs. In most patients who survive without antibiotic intervention, the involved lung will return to normal. Complications common to lobar and bronchopneumonia Abcesses may be independent of pneumonia; two patterns: Multiple abscesses usually occur with hematogenous infection or with bronchopneumonia by a virulent (often aerobic) organism that causes tissue necrosis. Solitary abscess: usually due to an anaerobic organism, e.g. in alcoholics with depressed reflexes who aspirate. Empyema is an infection of the pleura, in which there is a purulent exudative process. Since this occurs in a closed space, healing usually results in pleural fibrosis. Pathology Review Page 111 Mycobacterial-Tuberculosis Tuberculosis is a chronic communicable disease due to a bacteria which has a worldwide distribution and which most commonly infects the lungs but can involve any organ. In the U.S. in 1985, 22,201 new cases of tuberculosis were reported to the CDC, a slight decrease from the previous year. Of these, 2,481 were from New York. Males outnumbered females nearly 2:1. About 5 - 10 % died of their disease despite the fact that tuberculosis is considered preventable and curable. In 1986, a rise in the number of new cases of tuberculosis was reported in the US. The accompanying schematic is a simplification of tuberculous infection in man taking into consideration mycobacterium in terms of infectivity, type of host response, and varied pathologic responses with respect to primary versus secondary infection. Infectivity and disease depend on virulence, induced hypersensitivity, and immunity (or host resistance). Virulence and development of type IV immune hypersensitivity probably are related to the complex lipids and carbohydrates which form the mycobacterial wall. Induced hypersensitivity and immunity are probably both influenced by activated macrophages in the host. The typical cellular response 2-4 weeks after infection is formation of a granuloma. Typically these undergo central necrosis (caseation ). The causative agent is detected by culture and/or by tissue staining for acid-fast organisms. Mycobacteria are acid-fast bacilli which are slightly curved and beaded. Primary infection refers to the initial exposure and this usually results in self-limited disease with formation of a solitary granuloma of the lung (Ghon focus), often with granulomas along the routes of lymphatic drainage from that segment of lung and in hilar lymph nodes (Ghon complex). The Ghon focus is usually in the upper portion of the lower lobe, or the lower portion of the upper lobe near the fissure. Rarely there is progressive primary infection which may result in fulminant bronchopneumonia following airway spread of mycobacteria, or miliary spread either through the veins, arteries, or lymphatics. Essentially, the primary lesion during the process of caseation can erode into airways or vascular structures with dissemination to other areas appropriate for the distribution of that structure. Routes of Dissemination: Lymphatic spread: multiple granulomas may result in a tuberculoma; obliterative bronchitis; obliterative pleuritis; bronchial compression by tuberculous lymphadenitis. Airway spread: acinar-nodose pneumonia may lead to tuberculoma; caseous pneumonia leads to cavitary abcess. Arterial spread: pulmonary miliary tuberculosis. Venous spread: pulmonary or systemic miliary tuberculosis. A small percentage of people will later develop secondary infection, 9201 probably due to reactivation of latent mycobacteria, although reinfection is another possible mechanism. Secondary infection usually occurs in the lung apices, possibly due to the higher oxygen tensions in these regions, keeping in mind that mycobacteria are strict aerobes. The apical infection usually resolves by fibrosis and calcification, but less commonly can develop into progressive secondary infection which again can result in pneumonia, miliary spread , empyema, or tracheobronchial infection. Isolated organ involvement also occurs (intestine; adrenal leading to acute Addison's dise ; bone, such Pott's disease of the spine; and meninges). Chronic infection is occasionally associated with amyloidosis. Treatment is with antituberculous drugs. Fungal Pneumonias General Considerations Fungi are the second most common cause of infectious granulomas in the lungs. They have a tendency to form necrotizing granulomas similar or identical to tuberculous granulomas, and often cavitate centrally. Sometimes they result in suppurative granulomas in which neutrophils dominate the center. The most common responsible fungi, 9213 are dimorphic molds, especially histoplasma, of which the tissue form is yeasts; other yeast include cryptococcus, coccidioidomycoses, and blastomycosis. The frequency of these infections varies with geographic location. The most common yeast infection around New York is histoplasma. Pathology Review Page 112 Histoplasma Histoplasma capsulatum is a small (2-5 micron) oval budding yeast which is common in the U.S., especially in Ohio and the Mississippi Valley. This dimorphic fungus is found in the soil and bird droppings such as bat droppings in caves, and infection is usually the result of inhalation. The incidence of infection in the US is high although the most common manifestation is none, that is infection is latent and the patient is asymptomatic. Other expressions of disease are primary pulmonary which is self-limited; chronic pulmonary occasionally in the setting of COPD; and disseminated. The form that infection takes usually depends on host resistance and immunocompetence. Pathologically lesions due to histoplasma vary, although the most common are incidental fibrocalcific nodules (old healed granulomas) which are densely hyalinized and contain only occasional histoplasma yeast. These lesions may calcify and erode into a bronchus causing broncholiths, or stones. An immunocompetent host can response with classical necrotizing or non-necrotizing granulomas. In fulminant disseminated infection, as seen in AIDS, yeast pack macrophages which fill alveoli and infiltrate the interstitium. Cryptococcus Cryptococcus neoformans is a somewhat pleomorphic round-to-oval 4-10 micron yeast which may have a characteristic thick mucinous capsule which stains bright red with mucicarmine stain; some cryptococci may be unencapsulated. This yeast has a worldwide distribution and is found in bird (pigeon) droppings. Infection again in by inhalation. The most common infection is meningitis (cerebral). The other basic form of infection is pulmonary. Encapsulated: more virulent and often results in fulminant infection Non-encapsulated: frequently granulomatous inflammation Coccidioidomycosis Coccidioides immitis has large spherules filled with multiple endospores which excyst, enlarge and mature into a new spherule.ÊIn vitro, it also may have hyphae which form arthrospores, a particularly infectious agent which needs careful handling in the lab. Coccidioides is found in the Southwest US, Mexico, Central America and San Joaquin Valley in soil. Clinical Expressions of infection are: (1) primary pulmonary coccidioidomycosis, which tends to be asymptomatic; (2) primary extrapulmonary occurring in skin, and uncommon; (3) residual pulmonary Infection occurs in 2-8% of patients with primary disease and is represented as a solitary nodule or cavitary lesion; and (4) disseminated form: <1% with a predilection for immunosuppressed, American Indians and Blacks. Mycoplasma Pneumonia A common cause of URI, with pneumonia in approximately 10%, but accounting for about 15-20% of all pneumonias in the general population. Mycoplasma is a benign, self-limited disease with few complications; so very few cases have been examined histologically. The peak incidence is 5-15 years of age. Cold agglutinins are frequently found in the blood (RBCs clump at 4 degrees). Infection causes a bronchiolar lesion with a nonspecific neutrophil-rich exudate in the lumina, bronchiolar metaplasia, and bronchiolar wall lymphoplasmacytic infiltrates. Mycoplasma is the smallest free living organism (approx. 200 nanometers) and at one time was called Eaton's Agent. Pneumocystis (carinii) Pneumonia Pneumocystis was first descibed in 1905 (Brazil) in rats. During WW II pneumocystis pneumonia (PCP) was recognized in malnourished infants in Europe. In l957 was the first reported in case in the U.S. Pneumocystis is an extracellular protozoan parasite (or possibly a fungus) which in humans almost exclusively infects the lung. Infection typically leads to an interstitial infiltrate of lymphocytes and plasma cells, and to foamy, intra-alveolar exudates within which the organism are found. Pneumocystis attaches to pneumocytes without fusion, development of a glycocalyx, or invasion. The organisms occur as cysts, excysted forms, and trophozoites. The cysts are round and about 4 microns. The excysted forms are helmet-shaped and other irregular shapes. Pneumocystis organisms will stain with silver (GMS) and gram stains (gram posititve) while the trophozoites will stain with giemsa. The cysts contain multiple sporozoites which excyst and develop as trophozoites. Pneumocystis organisms tend to cause injury by their selective attachment to type I pneumocytes. Diagnosis can be made by Pathology Review Page 113 identifying the organism cytologically by looking for cysts or trophozoites in sputum, washings from the lung or bronchoalveolar lavage (BAL). Diagnosis can also be made by identifying the organisms on biopsy of the lung. PCP is generally treated with either bactrim or pentamidine. PCP is most frequently seen today in AIDS and less in other immunocompromised patients. PCP is rare in immunocompetent hosts, although there is an apparent increase in the incidence of PCP infections in patients who are minimally compromised. The route of spread is unknown. Pneumocystis cannot be cultured to date. AIDS patients tend to get recurrent infection and progressive scarring with diffuse interstitial fibrosis has been considered a complication of treatment. Association of Asbestos with Certain Tumors -- Bronchogenic Carcinoma With asbestos exposure is usually located in the lower lobes In smokers it is usually in the upper lobes In asbestos-exposed people who don't smoke there is a 5-10x increase in bronchogenic CA In people who smoke and have been exposed there is a 50-100x increase incidence of bronchogenic CA, (therefore additive effects) -- Mesothelioma A tumor that arises from the pleural lining Tends to spread over the pleural surface and can involve one or both lungs (NOTE: This is the same mesothelium that is found in the peritoneum and some of these patients will develop a peritoneal mesothelioma.) Two Types of Cell Growth Involved with These Tumors Epithelial Fibrous / Fibrosarcomatous Some say that you can't diagnose mesothelioma until the patient has died because these cells have characteristics which overlap with other, more common adenocarcinomas and fibrosarcomas. Staining Mesotheliomas Stain with PAS but after digestion they will be negative (adenocarcinomas tend to stain positively after digestion) Immunoperoxidase stains Mesotheliomas tend to be positive with cytokeratin and, Negative with CEA (carcinoembryonic antigen). EM shows what looks like an epithelial cell with cell junctions and villi, but adenocarcinomas will look similar It is the combination of all information that allows you to determine if asbetos exposure has occurred and if the condition is a mesothelioma, and then to make an antemortem diagnosis and properly treat the patient. Pneumococcal pneumonia can infect the upper respiratory tract and can spread to the blood, lungs, middle ear, or nervous system. Pneumococcal pneumonia mainly causes illness in children younger than 2 years old and adults 65 years of age or older. The elderly are especially at risk of getting seriously ill and dying from this disease. In addition, people with certain medical conditions such as chronic heart, lung, or liver diseases or sickle cell anemia are also at increased risk for getting pneumococcal pneumonia. People with HIV infection, AIDS, or people who have had organ transplants and are taking medicines that lower their resistance to infection are also at high risk of getting this Cause Pneumonia can be caused by a variety of viruses, bacteria, and sometimes fungi. Pneumococcal pneumonia is caused by bacteria called Streptococcus pneumoniae. S. pneumoniae is also called pneumococcus. Pneumococcal meningitis is an inflammation or infection of the membranes covering the brain and spinal cord caused by Streptococcus pneumoniae (also called pneumococcus). Pathology Review Page 114 Transmission Pneumococcus is spread through contact between people who are ill or who carry the bacteria in their throat. You can get pneumococcal pneumonia from respiratory droplets from the nose or mouth of an infected person. It is common for people, especially children, to carry the bacteria in their throats without being sick. Symptoms Pneumococcal pneumonia may begin suddenly. You may first have a severe shaking chill which is usually followed by High fever Nausea Cough Vomiting Shortness of breath Headache Rapid breathing Tiredness Chest pains Muscle aches Treatment Antibiotics are used to treat this disease. The symptoms of pneumococcal pneumonia usually go away within 12 to 36 hours after you start taking medicine. Some bacteria such as S. pneumoniae, however, are now capable of resisting and fighting off antibiotics. Such antibiotic resistance is increasing worldwide because these medicines have been overused or misused. RT involvement: Obviously pneumonia affects the lungs meaning sputum will be produced and in mass quantities. If the patient has a limited gag or impaired cough, this means you will be doing bronchial hygiene to clear the airway. RT’s can practically diagnose pneumonia based on physical assessment; for example: Most patients with pneumonia fall into these categories; old, very you, HIV, immune suppressed, homeless, convalescent home, impaired neurologically and alcoholics. After this note the sputum color, consistency, amount, and smell. Then note pain on inspiration, fever, tachypnea, and increased WBC on CBC. Then finish it off with noting consolidation on CXR and analysis of sputum with C/S. Left lung white out due to mucus plugs from pneumonia. Mycoplasma pneumonia is an infection of the lungs caused by Mycoplasma pneumoniae (M. pneumoniae). Causes M. pneumoniae is a common cause of mild pneumonia that usually affects people younger than 40. Various studies suggest that it causes 15-50% of all pneumonia in adults and an even higher percentage of pneumonia in school-aged children.People at highest risk for mycoplasma pneumonia include those living or working in crowded areas such as schools and homeless shelters, although many people who contract mycoplasma pneumonia have no identifiable risk factor. Symptoms The symptoms are generally mild and appear over a period of 1 to 3 weeks. They may become more severe in some people. Common symptoms include the following: Pathology Review Page 115 o Usually dry Headache o Usually without phlegm or Fever (may be high) blood Chills Chest pain Excessive sweating Sore throat Cough Less frequently seen symptoms include: Skin lesions or rash Neck lump Eye pain or soreness Rapid breathing Muscle aches and joint stiffness Ear pain Exams and Tests A physical examination may reveal enlarged lymph nodes and inflammation of the eardrum. An examination of the chest with a stethoscope (auscultation) reveals crackles. These tests help confirm the diagnosis: Blood tests for antibodies to mycoplasma Sputum culture Chest x-ray Treatment Antibiotics may be prescribed for more serious symptoms related to mycoplasma pneumonia. Home care includes rest and a high-protein diet with adequate fluids. CMV pneumonia Cytomegalovirus is a large herpes-type virus commonly found in humans that can cause serious infections in people with impaired immunity. CMV pneumonia is treated with antiviral medications, which may stop the replication of the virus but will not destroy it. Chickenpox, acute pneumonia - chest X-ray This chest x-ray shows cloudiness throughout the lungs, caused by acute pneumonia following chickenpox. Pneumonia, as a complication of chickenpox, rarely occurs in children, but occurs in about one-fifth of adults. Pathology Review Page 116 Pulmonary Hypertension Also called: Pulmonary arterial hypertension Pulmonary arterial hypertension (PAH) is continuous high blood pressure in the pulmonary artery. The average blood pressure in a normal pulmonary artery is about 14 mmHg when the person is resting. In PAH, the average is usually greater than 25 mmHg. The pulmonary arteries are the blood vessels that carry oxygen-poor blood from the right ventricle in the heart to the small arteries in the lungs. In PAH, three types of changes may occur in the pulmonary arteries: The muscles within the walls of the arteries may tighten up. This makes the inside of the arteries narrower. The walls of the pulmonary arteries may thicken as the amount of muscle increases in some arteries. Scar tissue may form in the walls of arteries. As the walls thicken and scar, the arteries become increasingly narrow. Tiny blood clots may form within the smaller arteries, causing blockages. There is less room for the blood to flow through these narrower arteries. The arteries may also stiffen. Over time, some of the arteries may become completely blocked. The narrowing of the pulmonary arteries causes the right side of heart to work harder to pump blood through the lungs. Over time, the heart muscle weakens and loses its ability to pump enough blood for the body's needs. This is called right heart failure. Heart failure is the most common cause of death in people with PAH. There are two types of PAH: Primary pulmonary arterial hypertension (PPAH) is inherited or occurs for no known reason. Secondary pulmonary arterial hypertension (SPAH) either is caused by or occurs because of another condition. The conditions include chronic heart or lung disease, blood clots in the lungs, or a disease like scleroderma. What Causes Pulmonary Arterial Hypertension? Use of appetite suppressants, especially fenfluramine (fen-FLOO-ra-men) and dexfenfluramine (deks-fen-FLOO-ra-men) Chronic use of cocaine or amphetamines HIV infection Liver disease Connective tissue diseases, such as scleroderma or lupus erythematosus Secondary pulmonary arterial hypertension (SPAH) is caused by a variety of conditions. Chronic obstructive pulmonary disease is the most common cause in adults. And RDS most common in neonates. Other Conditions That Can Lead to SPAH Obstructive sleep apnea Congenital heart defects Chronic blood clots in the pulmonary artery What Are the Signs and Symptoms of Pulmonary Arterial Hypertension? Dyspnea/increased WOB Bluish lips and skin (cyanosis) Fatigue Chest pain Dizziness Racing pulse Fainting spells (syncope) Palpitations (a strong feeling of a fast heartbeat) Swelling in the ankles or legs (edema) As the disease advances: The pumping action of your heart grows weaker. Your energy decreases. In the more advanced stages, you: Are able to perform very little activity Have symptoms even when resting May become completely bedridden Pathology Review Page 117 Limitations on Physical Activity Doctors may classify your symptoms based on how much activity you can comfortably undertake. The classes are the same as those for heart failure. They are: Class 1: No limits—Ordinary physical activity does not cause undue tiredness or shortness of breath. Class 2: Slight or mild limits—Person is comfortable at rest, but ordinary physical activity results in tiredness or shortness of breath. Class 3: Marked or noticeable limits—Person is comfortable at rest, but less than ordinary physical activity causes tiredness or shortness of breath. Class 4: Severe limits—Person is unable to carry on any physical activity without discomfort. Symptoms may also be present at rest. If any physical activity is undertaken, discomfort increases. How Is Pulmonary Arterial Hypertension Diagnosed? There is no one specific test that will show why you have pulmonary arterial hypertension (PAH). Even in its later stages, the signs of the disease are similar to those of other heart and lung conditions. Your doctor will determine if you have PAH by conducting a series of tests to: Determine the pressure in your pulmonary artery Find out how well your heart and lungs are working Rule out any other conditions that may be causing the hypertension These tests include: Chest x ray. A chest x ray takes a picture of your heart and lungs. It can show if the pulmonary arteries or the right side of the heart are enlarged. It will also help your doctor rule out a number of lung diseases, including chronic obstructive pulmonary disease (COPD), as the cause of your PAH. EKG (electrocardiogram). This test is used to measure the rate and regularity of your heartbeat, as well as the size and position of the right ventricle in your heart. It can help the doctor rule out a number of diseases of the heart. Echocardiogram. This test uses sound waves to create a moving picture of your heart. Echocardiogram provides information about the size and shape of your heart and how well your heart chambers and valves are functioning. The test also can identify areas of poor blood flow to the heart, areas of heart muscle that are not contracting normally, and previous injury to the heart muscle caused by poor blood flow. There are several different types of echocardiograms, including a stress echocardiogram. During this test, an echocardiogram is done both before and after your heart is stressed either by having you exercise or by injecting a medicine into your bloodstream that makes your heart beat faster and work harder. A stress echocardiogram is usually done to find out if you have decreased blood flow to your heart (coronary artery disease). Stress Test. Some heart problems are easier to diagnose when your heart is working harder and beating faster than when it’s at rest. During stress testing, you exercise (or are given medicine if you are unable to exercise) to make your heart work harder and beat faster while heart tests are performed. During exercise stress testing, your blood pressure and EKG readings are monitored while you walk or run on a treadmill or pedal a bicycle. Other heart tests, such as nuclear heart scanning or echocardiography, also can be done at the same time. These would be ordered if your doctor needs more information than the exercise stress test can provide about how well your heart is working. If you are unable to exercise, a medicine can be injected through an intravenous line (IV) into your bloodstream to make your heart work harder and beat faster, as if you are exercising on a treadmill or bicycle. Nuclear heart scanning or echocardiography is then usually done. During nuclear heart scanning, radioactive tracer is injected into your bloodstream, and a special camera shows the flow of blood through your heart and arteries. Echocardiography uses sound Pathology Review Page 118 waves to show blood flow through the chambers and valves of your heart and to show the strength of your heart muscle. Your doctor also may order two newer tests along with stress testing if more information is needed about how well your heart works. These new tests are magnetic resonance imaging (MRI) and positron emission tomography (PET) scanning of the heart. MRI shows detailed images of the structures and beating of your heart, which may help your doctor better assess if parts of your heart are weak or damaged. PET scanning shows the level of chemical activity in different areas of your heart. This can help your doctor determine if enough blood is flowing to the areas of your heart. A PET scan can show decreased blood flow caused by disease or damaged muscles that may not be detected by other scanning methods. Spirometry: It is most useful for ruling out obstructive lung diseases like COPD. Cardiac catheterization. This test provides a precise measure of the blood pressure in the right side of your heart and the pulmonary artery. Cardiac catheterization is the only way to get this measure. It also shows the amount of blood the right ventricle pumps with each heartbeat. This helps your doctor evaluate the pumping ability of the right ventricle. This procedure must be performed in the hospital by a specialist. Usually, these tests are sufficient to confirm that you have PAH. Sometimes these tests do not rule out all possible causes for the PAH. In that case, your doctor may call for these additional tests: Perfusion lung scan. This test shows how the blood is moving in your lungs and whether there are large blood clots that may be causing the PAH. Pulmonary arteriography. When the results of a perfusion lung scan do not rule out blood clots in the pulmonary arteries, your doctor may order a pulmonary arteriogram. This test also shows blood clots and other blockages in the blood vessels in the lung. Blood tests. Blood tests will rule out HIV, auto-immune diseases like scleroderma, and liver disease. Polysomnography (POL-e-som-NOG-ra-fe). This test will help your doctor rule out sleepdisordered breathing as a cause of your PAH. If these tests do not show an underlying cause for the PAH, the diagnosis is primary pulmonary arterial hypertension. How Is Pulmonary Arterial Hypertension Treated? The goals of treatment for patients with pulmonary arterial hypertension (PAH) are to: Treat the underlying cause. This is the first priority in patients with secondary pulmonary arterial hypertension (SPAH). Sometimes this treatment can correct the PAH. Reduce symptoms and improve quality of life. Slow the growth of the smooth muscle cells and the development of blood clots. Increase the supply of blood and oxygen to the heart, while reducing its workload. These treatments include: Medicines Oxygen Lung transplantation Medicines Anticoagulants (AN-te-ko-AG-u-lants) reduce the formation of blood clots. Calcium channel blockers relax blood vessels and increase the supply of blood and oxygen to the heart, while reducing its workload. These drugs can be very helpful, but only for a small amount of patients. All patients that take them should be monitored carefully. Epoprostenol (e-poe-PROST-en-ole) is a type of medicine called a prostacyclin (pros-ta-SI-klin). It is currently considered the most effective therapy for primary pulmonary arterial hypertension (PPAH). It may also be helpful in treating some forms of severe SPAH. This medication widens the lung arteries and prevents blood clot formation. Until recently, it was given intravenously through a permanent tube, or catheter, placed in a vein in the neck and connected to a batterypowered pump. But in December 2004, a new form of the drug, iloprost, was approved for the treatment of PAH. This medicine can be inhaled through a nebulizer. This makes it more Pathology Review Page 119 convenient and less painful to take. Plus the medicine goes directly to the lungs, where it is needed. Treprostinil, another prostacyclin, also relaxes blood vessels and increases the supply of blood to the lungs, reducing the workload of the heart. It can be given under the skin. Bosentan, a relatively new treatment, widens the lung arteries and reduces blood pressure. It also is available in pill form. Until doctors learn more about the long-term effects, all patients taking this drug should be monitored for complications, especially those involving the liver. Nitric oxide inhalation, which causes the pulmonary arteries to widen or open, is also being used by some doctors. Sildenafil (Viagra®), another drug that causes the pulmonary arteries to open, has recently been shown to improve the condition of PAH patients and is expected to be approved soon. This drug is available in pill form. Diuretics (water or fluid pills) may help ease symptoms and improve the heart's performance in some patients with PAH. Oxygen You may need oxygen therapy if the level of oxygen in your blood is low. Oxygen is usually given through nasal prongs or a mask. Over time, you may need oxygen around the clock. Lung Transplantation Surgery to replace one or both diseased lungs with healthy lungs from a human donor may help some patients. This procedure is usually recommended for patients for whom medical therapy is no longer effective. Complications include rejection by the body of the transplanted lung and infection. Transplant patients must take medicines for life to reduce the chances that their body will reject the transplanted lung. Other Possible Treatments Researchers also are studying whether stem cell transplantation combined with gene therapy may provide a cure for PAH in the not too distant future. Persistent Pulmonary Hypertension Of The Newborn (PPHN) Persistent pulmonary hypertension of the newborn is a cardiopulmonary disorder characterized by systemic arterial hypoxemia secondary to elevated pulmonary vascular resistance with resultant shunting of pulmonary blood flow to the systemic circulation. This pathophysiologic syndrome has been variously described as: Persistent pulmonary vascular Neonatal pulmonary ischemia obstruction Persistent transitional circulation Persistent fetal circulation Pulmonary vasospasm Persistent pulmonary hypertension of the newborn (PPHN) is the result of elevated pulmonary vascular resistance to the point that venous blood is diverted to some degree through fetal channels (i. e. the ductus arteriosus and foramen ovale) into the systemic circulation and bypassing the lungs, resulting in systemic arterial hypoxemia.This disorder can be classified into three forms dependent on the likely etiology of the pulmonary hypertension: 1. PPHN associated with pulmonary parenchymal disease, such as hyaline membrane disease, meconium aspiration, or transient tachypnea of the newborn as the cause of alveolar hypoxia o known as secondary PPHN or appropriate PPHN o alveolar oxygen tension appears to be the major determinant of pulmonary artery vasoconstriction. 2. PPHN with radiographically normal lungs and no evidence of parenchymal disease o frequently called Persistent Fetal Circulation (PFC), or primary or inappropriate PPHN 3. PPHN associated with hypoplasia of the lungs o most often in the form of diaphragmatic hernia Pathology Review Page 120 o associated with an anatomic reduction in capillary number in addition to the pathophysiology listed below This syndrome is usually noted in term or post-term infants. The baby presents clinically with cyanosis and respiratory distress, with tachypnea, but with minimal retractions during the first day of life. The infants chest radiograph may be normal (as noted in infants with primary PPHN, i.e. PFC) or demonstrate various abnormalities compatible with aspiration, pneumonia, diaphragmatic hernia, or hyaline membrane disease. Supplemental oxygen is needed in an attempt to correct arterial hypoxemia. With a large rightto-left shunt through a patent ductus arteriosus, an oxygen tension gradient may be noted between the preductal arterial circulation (i.e. right upper extremity) and the postductal arterial circulation (i.e. lower extremities). However, this gradient may not be present if substantial shunting is present at the level of the foramen ovale. Systemic hypotension is a late finding usually resulting from both heart failure and persistent hypoxemia. The diagnosis is confirmed echocardiographically. The most appropriate treatment of Persistent Pulmonary Hypertension of the Newborn remains unclear. In order of increasing aggressiveness and invasiveness: 1. Improve alveolar oxygenation 2. Minimize pulmonary vasoconstriction 3. Maintain systemic blood pressure and perfusion 4. Consider induction of an alkalotic state 5. Consider a trial of vasodilatation 6. Consider extracorporeal membrane oxygenation support 1. Improving alveolar oxygenation with supplemental oxygen (FiO2). o This is especially important when pulmonary parenchymal disease exists, as improvement in alveolar oxygenation will often result in a normal relaxation of the pulmonary arteries and improved pulmonary blood flow. 2. Minimize "inappropriate" pulmonary vasoconstriction o By over ventilating an infant and producing hypocapnia, hypoxic vasoconstriction can be blunted allowing pulmonary blood flow to increase. This may result in improvement in oxygenation. However, use of mechanical ventilatory support to achieve hypocapnic alkalosis can result in pulmonary trauma and may be related to long term hearing deficits noted in follow-up. Since vaosconstriction appears related to intracellular pH rather than pCO2 levels use of alkalinizing agents such as sodium bicarbonate has become commonplace. Controlled trials of alkalosis do suggest a positive benefit in some infants, but not all. o Maintenance of systemic arterial blood pressure and, by inference, pulmonary perfusion, appears to have some benefit. Theoretically, increasing system arterial pressure may result in decreased right-to-left shunt flow across a patent ductus arteriosus, increased pulmonary blood flow, and hopefully, improved oxygenation. Dopamine and dobutamine are frequently utilized to improve cardiac output and systemic blood pressure. o Vasodilators have been reported to be effective in a certain proportion of infants. However, these agents are nonspecific and frequently result in vasodilatation of both the pulmonary and systemic vascular beds. Most commonly used is tolazoline (Priscoline) The complete mechanism of action of tolazoline is not clear although it appears to be an alpha-sympathetic blocker. Such substances as norepinephrine which are a-sympathetic agents are known vasoconstrictors. By blocking this effect, tolazoline might produce vasodilation. Other work suggests that tolazoline's effect is mediated by histamine. When histamine receptors in the lung are blocked chemically (experimentally), tolazoline becomes a vasoconstrictor. Endotracheally administered tolazoline has been reported to be helpful. o Nitric Oxide Inhaled nitric oxide has been studied intensively as therapy for Persistent Pulmonary Hypertension of the Newborn. This gaseous free-radical compound was previously known as endothelial-derived-relaxation-factor. Inhaled nitric oxide directly Pathology Review Page 121 3. 4. activates soluble guanylate cyclase resulting in increased levels of cyclic-GMP in vascular smooth muscle cells. This results in vascular relaxation by prohibiting myosin protein cross-bridge formation in smooth muscle. Multiple controlled trials have demonstrated that inhaled nitric oxide improves oxygenation in many infants with PPHN. A controlled trial of inhaled nitric oxide in infants with congenital diaphragmatic hernia demonstrated no efficacy. Nitric oxide was FDA approved 12/23/99 for use in term and near-term infants with hypoxic respiratory failure requiring ventilatory support who have clinical and/or echocardiographic evidence of pulmonary hypertension. o Prostaglandin I2 (prostacyclin) This is a complex molecule made from arachadonic acid and is one of the major endogenous vasodilators in the lung. It is normally produced by the lung when lung vessels are in a constricted state, thereby relaxing them. Whether PPHN is the result of faulty PGI2 production is not known. Administration of pharmacologic doses of PGI2 to babies with persistent pulmonary hypertension has proven successful even when tolazoline failed. Other therapies proposed as beneficial in Persistent Pulmonary Hypertension of the Newborn include magnesium sulphate and arginine infusion, and the use of perflourocarbon liquid ventilation. At this point, these therapies are experimental and cannot be recommended. o When circulatory collapse has occurred, the use of an inotropic agent such as dopamine can increase systemic resistance and improved perfusion. Use of both dopamine and tolazoline together have been tried in desperation with occasional beneficial results When other therapies have failed to result in patient improvement, Extracorporeal Membrane Oxygenation (ECMO) support has been used with good success. Pierre Robin’s Syndrome Pierre Robin syndrome is a condition present at birth in which an infant has a very small lower jaw, a tongue that tends to fall back and downward, and a soft cleft palate.The syndrome is also called Pierre Robin complex or sequence. The specific causes of Pierre Robin syndrome are unknown. It may be part of many genetic syndromes. The lower jaw develops slowly over the first few months of life before birth, but speeds up during the first year after birth. The falling back of the tongue may cause choking episodes and feeding and breathing difficulties, especially when the child sleeps. Symptoms Cleft soft palate Large-appearing tongue in relation to jaw High-arched palate Natal teeth (teeth appearing when the Jaw that is very small jaw with baby is born) significant receding chin Recurrent ear infections Jaw placed unusually far back in the throat Small opening in the roof of the mouth, which causes choking Infants with this condition should NOT be put on their back, so that the tongue does not fall back into the airway. Problems associated with this syndrome tend to get better over the first few years as the lower jaw grows to a more normal size. In moderate cases, the patient requires placement of a tube through the nose and into the airway to avoid airway blockage. In severe cases, surgery is needed to prevent upper airway obstruction. A tracheostomy (surgery to make a hole in the windpipe) is sometimes required. Feeding must be done very carefully to avoid choking and breathing liquids into the airway. Pathology Review Page 122 Paroxysmal supraventricular tachycardia (PSVT) Paroxysmal supraventricular tachycardia (PSVT) is a rapid heart rate, which occurs from time to time (paroxysmal). PSVT starts with events taking place above the ventricles. Normally, the chambers of the heart (atria and ventricles) contract in a coordinated manner. The contractions are caused by an electrical signal that begins in the sinoatrial node (also called the sinus node or SA node). The signal is conducted through the atria (the upper heart chambers) and stimulates the atria to contract. Paroxysmal supraventricular tachycardia (PSVT) can be initiated in the SA node; in the atria or the atrial conduction pathways; or in the AV node. It occurs most often in young people and infants. Risks include smoking, caffeine, alcohol use, and illicit drug use. PSVT can occur with digitalis toxicity. It can be a form of a re-entry tachycardia (an electrical current is caught in a reentrant loop, excessively stimulating the heart), as in the case of Wolff-Parkinson-White syndrome. Symptoms Palpitations (a sensation of feeling the Chest tightness heart beat) Fainting Rapid pulse Dizziness Anxiety, feeling of impending doom Shortness of breath Note: Symptoms may start and stop suddenly, and can last for a few minutes or several hours. A PSVT lasting more than 50% of the day is considered an incessant PSVT. Exams and Tests An examination during a PSVT episode detects a rapid heart rate. The heart rate may be 150 to 250 beats per minute (bpm) (in children the heart rate tends to be very high). There may be signs of poor perfusion (blood circulation) such as light-headedness. Between episodes of PSVT, the heart rate is normal (60 to 100 bpm). An ECG during symptoms shows PSVT. Because of the sporadic nature of the PSVT, its diagnosis may require continuous ambulatory monitoring. The most common is the 24-hour Holter monitoring. For longer recording periods, a "loop recorder" (with computer memory) is used. An electrophysiology study (EPS) is often necessary for an accurate diagnosis, and to recommend the best treatment. PSVT can occur with few or no symptoms, and may not require treatment. If symptoms occur or there are underlying cardiac disorders, treatment may be necessary. People having an episode of PSVT can try to interrupt the episode with a Valsalva maneuver. This consists of holding the breath and straining (pushing with the abdomen as if to provoke a bowel movement) or coughing while sitting with the upper body bent forward. Splashing ice water on the face has been reported by some people as helpful. Persons having an episode of PSVT may be offered therapy to interrupt the arrhythmia and convert it to a normal sinus rhythm. In the emergency room, a health care provider may massage the carotid arteries in the neck in an attempt to interrupt the arrhythmia. Caution -- do not try this at home! This technique can cause Pathology Review Page 123 severe slowing of the heart rate. Electrical cardioversion (shock) is successful in conversion of PSVT to a normal sinus rhythm in many cases. Another way to rapidly convert a PSVT is to administer intravenous medications, including adenosine and verapamil. Other medications may be used, such as procainamide, beta-blockers, and propafenone. Aside from treating isolated episodes of PSVT, some patients may require a long-term or definitive treatment of the PVST which is directed toward the prevention or complete eradication of further episodes or arrhythmia. Such long-term treatment includes: Daily medications -- such as propafenone, flecainide, moricizine, sotalol, and amiodarone. Radiofrequency catheter ablation -- currently the treatment of choice for most PSVT's. Surgical modification of the electrical conduction pathway (the pathways in the heart that conduct the impulse to contract) -- this may be recommended in some cases when other heart surgery for other reasons is also indicated. Pacemakers -- very occasionally used in children with PSVT who have not responded to any other treatment. The pacemaker is designed to interrupt (override) the tachycardia. Patent ductus arteriosus (PDA) Patent ductus arteriosus (PDA) is a condition in which a blood vessel called the ductus arteriosus fails to close normally in an infant soon after birth. (The word "patent" means open.) The condition leads to abnormal blood flow between the aorta and pulmonary artery, two major blood vessels surrounding the heart. Before birth, the ductus arteriosus allows blood to bypass the baby's lungs by connecting the pulmonary arteries (which supply blood to the lungs) with the aorta (which supplies blood to the body). Soon after the infant is born and the lungs fill with air, this blood vessel is no longer needed. It will usually close within a couple of days. If the ductus arteriosus does not close, there will be abnormal blood circulation between the heart and lungs. The condition is more common in premature infants and those with neonatal respiratory distress syndrome. Infants with genetic disorders, such as Down syndrome, and whose mothers had German measles (rubella) during pregnancy are at higher risk for PDA. PDA is common in babies with congenital heart problems, such as hypoplastic left heart syndrome, transposition of the great vessels, and pulmonary stenosis. Symptoms A small PDA may not cause any symptoms. However, most infants do not tolerate a PDA and may have symptoms such as: Bounding pulse Sweating while feeding Fast breathing Tiring very easily Poor feeding habits Poor growth Shortness of breath Exams and Tests Babies with PDA often have a characteristic heart murmur that can be heard with a stethoscope. However, in premature infants, a heart murmur may not be heard. Doctor's may suspect the condition if the infant has breathing or feeding problems soon after birth. Changes may be seen on chest x-rays. The diagnosis is confirmed with an echocardiogram. Sometimes, a small PDA may not be diagnosed until later in childhood. Treatment The goal of treatment, if the rest of circulation is normal or close to normal, is to close the PDA. In the presence of certain other heart problems, such as hypoplastic left heart syndrome, the PDA may actually be lifesaving. Sometimes, a PDA may close on its own. Premature babies have a high rate of closure within Pathology Review Page 124 the first 2 years of life. In full-term infants, a PDA rarely closes on its own after the first few weeks. When treatment is appropriate, medications such as indomethacin or a special form of ibuprofen are generally the first choice. If these measures do not work or can't be used, a medical procedure may be needed. A transcatheter device closure is a minimally invasive procedure that uses a thin, hollow tube. The doctor passes a small metal coil or other blocking device through the catheter to the site of the PDA. This blocks blood flow through the vessel. Such endovascular coils have been used successfully as an alternative to surgery.There is no agreement about which young babies are most likely to benefit from surgery if medications are not going to help, and which babies would be better off untreated. Surgery may be needed if the catheter procedure does not work or cannot be used. Surgery involves making a small cut between the ribs to repair the PDA. Surgical treatment of PDAs may be performed on older children even if they have no symptoms because the PDA will not close by itself. Pectus carinatum (Pigeon Chest) Pectus carinatum describes a protrusion of the chest over the sternum, often described as giving the person a bird-like appearance. Pectus carinatum may occur as a solitary abnormality or in association with other genetic disorders or syndromes. The condition causes the sternum to protrude, with a narrow depression along the sides of the chest. This gives the chest a bowed-out appearance similar to that of a pigeon. People with pectus carinatum generally develop normal hearts and lungs, but the deformity may prevent these from functioning optimally. There is some evidence that pectus carinatum may prevent complete expiration of air from the lungs in children. These young people may have a decrease in stamina, even if they do not recognize it. Apart from the possible physiologic consequences, pectus deformities can have a significant psychologic impact. Some children live happily with pectus carinatum. For others, though, the shape of the chest can damage their self-image and self-confidence, possibly disrupting connections with others. Causes Congenital pectus carinatum (present Marfan's syndrome at birth) Morquio syndrome Trisomy 18 Multiple lentigines syndrome Trisomy 21 Osteogenesis imperfecta Homocystinuria Pathology Review Page 125 Poliomyelitis Poliomyelitis is a disorder caused by a viral infection. The virus, known as poliovirus, infects nerves. This infection can lead to temporary paralysis or, in more severe cases, permanent paralysis or death. Poliomyelitis is a communicable disease caused by infection with the poliovirus. Transmission of the virus occurs by direct person-to-person contact, by contact with infected secretions from the nose or mouth, or by contact with infected feces. The virus enters through the mouth and nose, multiplies in the throat and intestinal tract, and then is absorbed and spread through the blood and lymph system. Incubation (the time from being infected with the virus to developing symptoms of disease) ranges from 5 to 35 days (average 7 to 14 days). Risks include: Travel to an area that has experienced a polio outbreak Lack of immunization against polio and subsequent exposure to a case of polio In areas that had an outbreak, the more susceptible populations include children, pregnant women, and the elderly. Polio has been eradicated in a number of countries, and now occurs only in a handful of regions worldwide. Until recently, the last case of non-vaccine related polio in the United States was in 1979. However, in November 2005, four children in an Amish community in Minnesota were diagnosed with polio. None of these children had been vaccinated for polio, which is now a routine measure in the United States. Besides this small outbreak, there have been very few cases of polio in the Western hemisphere since the late 1970s. This is due to a massive eradication program, which included mass vaccination in these regions. However, there are still areas of the world where polio is widespread. The disease is more common in the summer and fall. Symptoms There are three basic patterns of polio infection: subclinical infections, nonparalytic, and paralytic. Approximately 95% of infections are subclinical infections, which may go unnoticed. Clinical poliomyelitis affects the central nervous system (brain and spinal cord), and is divided into nonparalytic and paralytic forms. It may occur after recovery from a subclinical infection. PARALYTIC POLIOMYELITIS Fever, occurring 5 to 7 days before Stiff neck and back other symptoms Muscle weakness, asymmetrical (only on one side or worse on one side) Headache o Rapid onset Pathology Review Page 126 Progresses to paralysis Swallowing difficulty Location depends on where Muscle pain the spinal cord is affected Muscle contractions or muscle spasms, Abnormal sensations (but not loss of particularly in the calf, neck, or back sensation) of an area Drooling Sensitivity to touch, mild touch may be Breathing difficulty painful Irritability or poor temper control Difficulty beginning to urinate Positive Babinski's reflex Constipation Bloated feeling of abdomen Exams and Tests Examination may shows signs of meningeal irritation (similar to meningitis), such as stiff neck or back stiffness with difficulty bending the neck. When sitting, the person may need to support the body with their arms. The person may have difficulty lifting the head or lifting the legs when lying flat on the back. Reflexes may be abnormal. The disorder may resemble encephalitis, and it may affect the cranial nerves and cause difficulty with facial expression, swallowing, chewing, and so on. It may also cause choking or difficulty breathing.Viral cultures of throat washings, stools, or cerebrospinal fluid (CSF) confirm the diagnosis (see CSF collection). Routine CSF examination may be normal or show slight increase in pressure, protein, and white blood cells. Another way to make the diagnosis is to test for a rise in levels of the antibodies to the polio virus. Treatment The goal of treatment is to control symptoms while the infection runs its course.Lifesaving measures, particularly assistance with breathing, may be necessary in severe cases. Symptoms are treated according to their presence and severity. Antibiotics may be used to treat urinary tract infections. Medications, such as bethanechol, may reduce urinary retention. Pain killers are used to reduce headache, muscle pain, and spasms. Narcotics are not usually given because they increase the risk of breathing difficulty. Moist heat (heating pads, warm towels, etc.) may reduce muscle pain and spasm. Activity is limited only by the extent of discomfort and the extent of muscle weakness. Physical therapy, braces or corrective shoes, orthopedic surgery, or similar interventions may eventually be necessary to maximize recovery of muscle strength and function. o o Respiratory Syncytial Virus (RSV) Respiratory syncytial virus (RSV) is a very common virus. This virus causes mild, cold-like symptoms in adults and older healthy children. It can cause serious respiratory infections in young babies, especially those in certain high-risk groups. RSV is the most common respiratory pathogen in infants and young children. It has infected nearly all infants by the age of two years. Seasonal outbreaks of acute respiratory illness occur each year, on a schedule that is somewhat predictable in each region. The season typically begins in the fall and runs into the spring. RSV is spread easily by physical contact. Touching, kissing, and shaking hands with an infected person can spread RSV. Transmission is usually by contact with contaminated secretions, which may involve tiny droplets, or objects that droplets have touched. RSV can live for half an hour or more on hands. The virus can also live up to five hours on countertops and for several hours on used tissues. RSV often spreads very rapidly in crowded households and day care centers. In infants and young children, RSV can cause pneumonia, bronchiolitis (inflammation of the small airways of the lungs), and croup. In healthy adults and older children, RSV is usually a mild respiratory illness. Although studies have shown that people produce antibodies against the virus, infections continue to occur in people of all ages. Each year up to 125,000 infants are hospitalized due to severe RSV disease, and about 1-2% of these infants die. Infants born prematurely, those with chronic lung disease, those who are immunocompromised, and those with certain forms of heart disease are at increased risk for severe RSV Pathology Review Page 127 disease. Those who are exposed to tobacco smoke, who attend daycare, who live in crowded conditions, or who have school-age siblings are also at higher risk. Symptoms Breathing difficulty or labored Fever breathing Nasal flaring Cough Rapid breathing (tachypnea) Croupy cough (often described as a Shortness of breath "seal bark" cough) Stuffy nose Cyanosis (bluish discoloration of skin Wheezing caused by lack of oxygen) Note: Symptoms vary and differ with age. Infants under age 1 are most severely affected and often have the most trouble breathing. Older children usually have only mild, cold-like symptoms. Symptoms usually appear 4-6 days after exposure. Exams and Tests Rapid tests for this virus can be performed at many hospitals on fluid obtained from the nose. RT’s may obtain this sample. Treatment Antibiotics do not help in the treatment of RSV. Mild infections go away without treatment. Infants and children with a severe RSV infection may be admitted to the hospital so they can receive oxygen, humidified air, and fluids by IV. Bronchodilators are mostly ineffective! Ribavirin may be given in extreme cases using a SPAG device. Some evidence suggests that children who have had RSV bronchiolitis have an increased risk for asthma. Prevention A simple way to help prevent RSV infection is to wash your hands often, especially before touching your baby. It's important to make certain that other people, especially care givers, take precautions to avoid giving RSV to your baby. The following simple steps can help protect your baby: Insist that others wash their hands with warm water and soap before touching your baby. Have others avoid contact with the baby if they have a cold or fever. If necessary, it may be helpful to wear a mask. Be aware that kissing the baby can spread RSV infection. Try to keep young children away from your baby. RSV is very common among young children and easily spreads from child to child. Do not smoke inside your house, car or anywhere near your baby. Exposure to tobacco smoke increases the risk of RSV illness. Parents with high-risk young infants should avoid crowds during outbreaks of RSV. Moderate-to-large outbreaks are often reported in the local news and newspapers to provide parents with an opportunity to avoid exposure. The drug Synagis (palivizumab) is approved for prevention of RSV disease in children younger than 24 months of age who are at high risk for serious RSV disease. Ask your doctor if your child is at high risk for RSV and whether this medicine should be given. Retrolental fibroplasia; ROP Retinopathy of prematurity (ROP) is abnormal blood vessel development in the retina of the eye in a premature infant. The blood vessels of the retina begin to develop 3 months after conception and complete their development at the time of normal birth. If an infant is born very prematurely, eye development can be disrupted. The vessels may stop growing or grow abnormally from the retina into the normally clear gel that fills the back of the eye. The vessels are fragile and can leak, causing bleeding in the eye. Scar tissue may develop and pull the retina loose from the inner surface of the eye. In severe cases, this can result in vision loss. In the past, routine use of excess oxygen to treat premature babies stimulated abnormal vessel growth. Currently, oxygen can be easily and accurately monitored, so this problem is rare. Today, the risk of developing ROP depends on the degree of prematurity. Generally, the smallest and sickest premature babies have the highest risk. Typically all babies younger than 30 weeks Pathology Review Page 128 gestation or weighing fewer than 3 pounds at birth are screened for the condition. Certain high-risk babies who weigh 3 - 4.5 pounds or who are born after 30 weeks should also be screened. In addition to prematurity, other risks factors may include: Brief stop in breathing (apnea) Low blood oxygen Heart disease Respiratory distress High carbon dioxide (CO2) in the blood Slow heart rate (bradycardia) Infection Transfusions Low blood acidity (pH) The rate of ROP in moderately premature infants has decreased dramatically with better care in the neonatal intensive care unit. Ironically, however, this has led to high rates of survival of very premature infants who would have had little chance of survival in the past. Since these very premature infants are at the highest risk of developing ROP, the condition may actually be becoming more common again. Symptoms There are 5 stages of ROP. Stage I: There is mildly abnormal blood vessel growth. Stage II: Blood vessel growth is moderately abnormal. Stage III: Blood vessel growth is severely abnormal. Stage IV: Blood vessel growth is severely abnormal and there is a partially detached retina. Stage V: There is a total retinal detachment. The blood vessel changes cannot be seen with the naked eye. An eye exam is needed to reveal such problems. An infant with ROP may be classified as having “plus disease” if the dilation and twisting of the blood vessels matches or exceeds a standard photograph. Symptoms of severe ROP include: Abnormal eye movements Crossed eyes Severe nearsightedness White-looking pupils (leukocoria) Exams and Tests High-risk infants and those younger than 30 weeks gestation or born weighing fewer than 3 lbs should have retinal exams. The first exam usually should be 4 - 9 weeks after birth, depending on the baby’s gestational age. Babies born at 27 weeks or later usually have their exam at 4 weeks of age. Those born earlier usually have exams later. Follow-up examinations are determined based on the results of the first exam. Babies do not need another examination if the blood vessels in both retinas have completed normal development. Treatment Early treatment has been shown to improve a baby’s chances for normal vision. Treatment should start within 72 hours of the eye exam. Some babies with “plus disease” need immediate treatment. Treatment may include cryotherapy (freezing) to prevent the spread of abnormal blood vessels. Laser therapy (photocoagulation) may be used to prevent complications of advanced ROP. The laser therapy stops the abnormal blood vessels from growing. It can be performed in the nursery using portable equipment. To be effective, it must be done before scarring and detachment occurs Surgery is needed if the retina detaches. Surgical procedures continue to improve, but may not result in good vision. RT’s need to be aware of high FIO2’s with premature babies! Keep O2 as low as possible <40% Sarcoidosis Sarcoidosis involves inflammation that produces tiny lumps of cells in various organs in your body. The lumps are called granulomas because they look like grains of sugar or sand. They are very small and can be seen only with a microscope. These tiny granulomas can grow and clump together, making many large and small groups of lumps. If many granulomas form in an organ, they can affect how the organ works. Pathology Review Page 129 This can cause symptoms of sarcoidosis. Sarcoidosis can occur in almost any part of your body, although it usually affects some organs more than others. It usually starts in one of two places: Lungs Lymph nodes, especially the lymph nodes in your chest cavity. Sarcoidosis also often affects your: Skin Liver. Eyes Less often, sarcoidosis affects your: Spleen Bones and joints. Brain Thyroid gland Nerves Breasts Heart Kidneys Tear glands Reproductive organs. Salivary glands Sarcoidosis almost always occurs in more than one organ at a time. Sarcoidosis has an active and a nonactive phase: In the active phase, the granulomas form and grow. In this phase, symptoms can develop, and scar tissue can form in the organs where the granulomas occur. In the nonactive phase, the inflammation goes down, and the granulomas stay the same size or shrink. But the scars may remain and cause symptoms. The course of the disease varies greatly among people. In many people, sarcoidosis is mild. The inflammation that causes the granulomas may get better on its own. The granulomas may stop growing or shrink. Symptoms may go away within a few years. In some people, the inflammation remains but doesn't get worse. You may also have symptoms or flare-ups and need treatment every now and then. In other people, sarcoidosis slowly gets worse over the years and can cause permanent organ damage. Although treatment can help, sarcoidosis may leave scar tissue in the lungs, skin, eyes, or other organs. The scar tissue can affect how the organs work. Treatment usually does not affect scar tissue. Changes in sarcoidosis usually occur slowly (e.g., over months). Sarcoidosis does not usually cause sudden illness. However, some symptoms may occur suddenly. They include: Disturbed heart rhythms Arthritis in the ankles Eye symptoms. In some serious cases in which vital organs are affected, sarcoidosis can result in death. Sarcoidosis is not a form of cancer. There is no known way to prevent sarcoidosis. Sarcoidosis was once thought to be an uncommon condition. It's now known to affect tens of thousands of people throughout the United States. Because many people who have sarcoidosis have no symptoms, it's hard to know how many people have the condition. Normally, your immune system defends your body against things that it sees as foreign and harmful. It does this by sending special cells to the organs that are being affected by these things. These cells release chemicals that produce inflammation around the foreign substance or substances to isolate and destroy them. In sarcoidosis, this inflammation remains and leads to the development of granulomas or lumps. You can't catch sarcoidosis from someone who has it. What Are the Signs and Symptoms of Sarcoidosis? Many people who have sarcoidosis have no symptoms. Often, the condition is discovered by accident only because a person has a chest x ray for another reason, such as a pre-employment x ray. Some people have very few symptoms, but others have many. Symptoms usually depend on which organs the disease affects. Lung Symptoms Shortness of breath Pathology Review Page 130 A dry cough that doesn't bring up phlegm, or mucus Wheezing Pain in the middle of your chest that gets worse when you breathe deeply or cough (rare). Lymph Node Symptoms Enlarged and sometimes tender lymph nodes—most often those in your neck and chest but sometimes those under your chin, in your arm pits, or in your groin. Skin Symptoms Various types of bumps, ulcers, or, rarely, flat areas of discolored skin, that appear mostly near your nose, eyes, back, arms, legs, and scalp. They usually itch but aren't painful. They usually last a long time. Painful bumps that usually appear on your ankles and shins and can be warm, tender, red or purple-to-red in color, and slightly raised. This is called erythema nodosum (er"i-the'mah nodo'sum). You may have fever and swollen ankles and joint pain along with the bumps. The bumps often are an early sign of sarcoidosis, but they occur in other diseases too. The bumps usually go away in weeks to months, even without treatment. Disfiguring skin sores that may affect your nose, nasal passages, cheeks, ears, eyelids, and fingers. This is called lupus pernio (loo'pus per'nio). The sores tend to be ongoing and can return after treatment is over. Heart Symptoms Shortness of breath Irregular heartbeat, including palpitations (a fluttering feeling of Swelling in your legs rapid heartbeats) and skipped beats Wheezing Sudden loss of consciousness Coughing Sudden death. Liver Symptoms Fever Pain in the upper right part of your abdomen, under the right ribs Fatigue Enlarged liver. Itching Parotid (pah-rot'id) and Other Salivary Gland Symptoms Swelling, which makes your cheeks look puffy Excessive dryness in your mouth and throat. Blood, Urinary Tract, and Kidney Symptoms Increased calcium in your blood or urine, which can lead to painful kidney stones Confusion Increased urination. Nervous System Symptoms Bell's palsy, a disorder that may be Headaches. caused by a virus. Vision problems. Paralysis of your arms or legs that Weakness or numbness of an arm or results from sarcoidosis affecting your leg. spinal cord. Coma (rare). Weakness, pain, or a "stinging needles" Drooping of one side of your face that sensation in areas where many nerves results from sarcoidosis affecting a are affected by sarcoidosis. facial nerve. This can be confused with Pituitary (pi-tu'i-tar"e) Gland Symptoms (Rare) Headaches Weakness or numbness of an arm or leg Vision problems Coma (rare). Other Symptoms Nasal obstruction or frequent bouts of sinusitis. Pathology Review Page 131 Enlarged spleen, which leads to a decrease in platelets in your blood and pain in your upper left abdomen. Platelets are needed to help your blood clot. Sarcoidosis may also cause more general symptoms, including: Uneasiness, feeling sick (malaise), an Fever overall feeling of ill health Night sweats Tiredness, fatigue, weakness Sleep problems Loss of appetite or weight Sepsis Systemic inflammatory response syndrome (SIRS) Sepsis is a severe illness caused by overwhelming infection of the bloodstream by toxin-producing bacteria. Sepsis is caused by bacterial infection that can originate anywhere in the body. Common sites include the following: The kidneys (upper urinary tract infection) The liver or the gall bladder The bowel (usually seen with peritonitis) The skin (cellulitis) The lungs (bacterial pneumonia) Meningitis may also be accompanied by sepsis. In children, sepsis may accompany infection of the bone (osteomyelitis). In hospitalized patients, common sites of infection include intravenous lines, surgical wounds, surgical drains, and sites of skin breakdown known as decubitus ulcers or bedsores. The infection is often confirmed by a positive blood culture, though blood cultures may be negative in individuals who have been receiving antibiotics. In sepsis, blood pressure drops, resulting in shock. Major organs and systems, including the kidneys, liver, lungs, and central nervous system, stop functioning normally. A change in mental status and hyperventilation may be the earliest signs of impending sepsis. Sepsis is often life-threatening, especially in people with a weakened immune system or other medical illnesses. Symptoms Fever or hypothermia (low body Skin rash temperature) Rapid heart beat Hyperventilation Confusion or delirium Chills Decreased urine output Shaking Warm skin Exams and Tests White blood cell count that is low or high Platelet count that is low Blood culture that is positive for bacteria Blood gases that reveal acidosis Kidney function tests that are abnormal (early in the course of disease) This disease may also alter the results of the following tests: Peripheral smear may demonstrate a low platelet count and destruction of red blood cells. Fibrin degradation products are often elevated, a condition that may be associated with a tendency to bleed. Blood differential -- with immature white blood cells seen Treatment Septic patients usually require monitoring in an intensive care unit (ICU). "Broad spectrum" intravenous antibiotic therapy should be initiated as soon as sepsis is suspected. The number of antibiotics administered may be decreased when the results of blood cultures become available and the causative organism is identified. The source of the infection should be discovered, if possible, which may mean further diagnostic testing. Sources such as infected intravenous lines or surgical drains should be Pathology Review Page 132 removed, and sources such as abscesses should be surgically drained. Supportive therapy with oxygen, intravenous fluids, and medications that increase blood pressure may be required for a good outcome. Dialysis may be necessary in the event of kidney failure, and mechanical ventilation is often required if respiratory failure occurs. RT involvement: Sepsis often leads to ARDS! People with sepsis will have an increased O2 consumption and need supplemental O2. Silicosis Silicosis is a disabling and often fatal lung disease caused by breathing dust that has very small pieces of crystalline silica in it. Crystalline silica is found in concrete, masonry, sandstone, rock, paint, and other abrasives. The cutting, breaking, crushing, drilling, grinding, or abrasive blasting of these materials may produce fine silica dust. It can also be in soil, mortar, plaster, and shingles. The very small pieces of silica dust get in the air that you breathe and become trapped in your lungs. Even the very small pieces of dust that you cannot see will harm you. As the dust builds up in your lungs, the lungs are damaged and it becomes harder to breathe. Symptoms Symptoms may not appear in the early stages of chronic silicosis. In fact, chronic silicosis may go undetected for 15 to 20 years after exposure. As silicosis progresses, symptoms may include: Shortness of breath Severe cough Weakness Because the body's ability to fight infections may be weakened by silica in the lungs, other illnesses (such as tuberculosis) may result and can cause: Fever Chest pains Weight loss Respiratory failure Night sweats These symptoms can become worse over time, leading to death. Subcutaneous emphysema Crepitus; Subcutaneous air; Tissue emphysema Occurs when air gets into tissues under the skin covering the chest wall or neck. This can happen due to stabbing, gun shot wounds, other penetrations, CVP line placement or blunt trauma. Air can also be found in between skin layers on the arms and legs during certain infections, including gas gangrene. Subcutaneous emphysema can often be seen as a smooth bulging of the skin. When a health care provider feels (palpates) the skin, it produces an unusual crackling sensation as the gas is pushed through the tissue. This is a rare condition. When it does occur, possible causes include: Pneumothorax (collapsed lung) Ruptured esophagus Ruptured bronchial tube May require intubation in severe cases No treatment usually required. Systemic lupus erythematosus (SLE) SLE is a chronic, inflammatory autoimmune disorder. It may affect the skin, joints, kidneys, and other organs. SLE (lupus) is an autoimmune disease. This means there is a problem with the body's normal immune system response. Normally, the immune system helps protect the body from harmful substances. But in patients with an autoimmune disease, the immune system can't tell the difference between harmful substances and healthy ones. The result is an overactive immune response that attacks otherwise healthy cells and tissue. This leads to chronic (long-term) inflammation.The underlying cause of autoimmune diseases is not fully known. Some researchers think autoimmune diseases occur after Pathology Review Page 133 infection with an organism that looks like certain proteins in the body. The proteins are later mistaken for the organism and wrongly targeted for attack by the body's immune system. SLE may be mild or severe enough to cause death. SLE affects nine times as many women as men. It may occur at any age, but appears most often in people between the ages of 10 and 50 years. African Americans and Asians are affected more often than people from other races. Symptoms Symptoms vary from person to person, and may come and go. The condition may affect one organ or body system at first. Others may become involved later. Almost all people with SLE have joint pain and most develop arthritis. Frequently affected joints are the fingers, hands, wrists, and knees. Inflammation of various parts of the heart may occur as pericarditis, endocarditis, or myocarditis. Chest pain and arrhythmias may result from these conditions. Fever Sensitivity to Nosebleed sunlight Fatigue Swallowing difficulty Joint pain and General discomfort, Skin color is patchy swelling uneasiness or ill Red spots on skin feeling (malaise) Arthritis Fingers that change Skin rash -- a Swollen glands color upon pressure "butterfly" rash over Muscle aches or in the cold the cheeks and Nausea and Numbness and bridge of the nose vomiting tingling affects about half of Pleurisy (causes Mouth sores those with SLE. The chest pain) Hair loss rash gets worse Pleural effusions Abdominal pain when in sunlight. Seizures Visual disturbance The rash may also Psychosis Blood disorders, be widespread. Blood in the urine including blood clots Coughing up blood Exams and Tests The diagnosis of SLE is based upon the presence of at least four out of eleven typical characteristics of the disease. The doctor will listen to your chest with a stethoscope. A sound called a heart friction rub or pleural friction rub may be heard. A neurological exam will also be performed. Tests used to diagnose SLE may include: Antinuclear antibody (ANA) panel including anti-DNA and anti-Smith antibodies Chest x-ray showing pleuritis or pericarditis Urinalysis to show blood, casts, or protein in the urine CBC Kidney biopsy This disease may also alter the results of the following tests: WBC count ESR Serum globulin electrophoresis Cryoglobulins Rheumatoid factor Coombs' test - direct Urine protein Complement component 3 (C3) Serum protein electrophoresis Complement Mononucleosis spot test Anti-thyroid microsomal antibody Anti-thyroglobulin antibody Anti-mitochondrial antibody Anti-smooth muscle antibody Treatment There is no cure for SLE. Treatment is aimed at controlling symptoms. Your individual symptoms determine your treatment. Mild disease that involves a rash, headaches, fever, arthritis, pleurisy, and pericarditis requires little therapy. Nonsteroidal anti-inflammatory medications (NSAIDs) are used to treat arthritis and pleurisy. Corticosteroid creams are used to treat skin rashes. An anti-malaria drug called Pathology Review Page 134 hydroxychloroquine) and low dose corticosteroids are sometimes used for skin and arthritis symptoms.Severe or life-threatening symptoms (such as hemolytic anemia, extensive heart or lung involvement, kidney disease, or central nervous system involvement) often require treatment by a rheumatologist and other specialists in the specific area. Corticosteroids or medications to decrease the immune system response may be prescribed to control the various symptoms. Some health care professionals use cytotoxic drugs (drugs that block cell growth) to treat people who do not respond well to corticosteroids or who must use high doses of corticosteroids. Tetanus It is a potentially deadly nervous system disease due to the bacteria Clostridium tetani (C. tetani). Spores of the bacteria C. tetani live in the soil and are found around the world. In the spore form, C. tetani may remain inactive in the soil, but it can remain infectious for more than 40 years. Tetanus causes approximately five deaths per year in the United States. Infection begins when the spores enter the body through an injury or wound. The spores release active bacteria that spread and make a poison called tetanospasmin. Tetanospasmin blocks nerve signals from the spinal cord to the muscles, causing severe muscle spasm. Spasmodic contractions can be so powerful that they tear the muscles or cause compression fractures of the vertebrae. Tetanus often begins with mild spasms in the jaw muscles (trismus), neck muscles, and facial muscles. Stiffness rapidly develops in the chest, back, abdominal muscles, and sometimes the laryngeal muscles (which then interferes with breathing). Muscular seizures (tetany) cause sudden, powerful, and painful contraction of muscle groups. These episodes can cause fractures and muscle tears. The time between infection and the first sign of symptoms is 5 days to 15 weeks, with 7 days as the average. Most cases of tetanus in the United States occur in those who have not been properly vaccinated against the disease. Symptoms Spasms and tightening of the jaw Tetanic seizures (painful, powerful muscle ("lockjaw") muscle contractions) Stiffness and spasms of various muscle Irritability groups Fever o Neck muscles Excessive sweating o Chest muscles Swallowing difficulty o Abdominal muscles Hand or foot spasms o Back muscles, often causing Drooling arching (opisthotonos) Uncontrolled urination or defecation Treatment Treatment may include: Medicine to reverse the poison (tetanus immune globulin) Antibiotics including penicillin, clindamycin, erythromycin, or metronidazole Surgery to clean the wound and remove the source of the poison (debridement) Muscle relaxers such as diazepam Bedrest with a nonstimulating environment Tetralogy of Fallot TOF refers to four types of heart defects present at birth (congenital). Tetralogy of Fallot is classified as a cyanotic heart defect because the condition causes too little oxygen levels in the blood, which leads to cyanosis (a bluish-purple coloration to the skin). The classic form of Tetralogy includes 4 defects within the heart structures: Ventricular septal defect (hole between the right and left ventricles) Narrowing of the pulmonary outflow tract (tube that connects the heart with the lungs) An aorta (tube that carries oxygenated blood to the body) that grows from both ventricles, rather than exclusively from the left ventricle A thickened muscular wall of the right ventricle (right ventricular hypertrophy) At birth, infants may not show the signs of the cyanosis, but later may develop sudden frightening episodes (called "Tet spells") of bluish skin from crying or feeding. Tetralogy of Fallot occurs in Pathology Review Page 135 approximately 5 out of 10,000 infants.The cause of most congenital heart defects is unknown. Multiple factors seem to be involved. Prenatal factors associated with higher than normal risk for this condition include maternal rubella or other viral illnesses during pregnancy, poor prenatal nutrition, maternal alcoholism, mother over 40 years old, and diabetes. There is a high incidence of chromosomal disorders in children with tetralogy of Fallot, such as Down syndrome and Di George's syndrome (a partial gene deletion that results in heart defects, low calcium levels, and immune deficiency.) Symptoms Difficult feeding (poor feeding habits) Passing out Failure to gain weight Sudden death Poor development Clubbing of fingers (skin or bone enlargement around the finger nails) Cyanosis which becomes more pronounced during periods of agitation Squatting during episodes of cyanosis Exams and Tests A physical examination with a stethoscope almost always reveals a heart murmur. Tests may include: EKG (electrocardiogram) may show the thickening of the right ventricle muscle CBC may show an increase in red blood cells Chest x-ray may show a "boot shaped" heart and dark lungs Cardiac catheterization helps show blood vessels in the lungs and heart Echocardiogram provides a definite diagnosis Treatment Surgery to repair heart defects is always done when the infant is very young. Sometimes more than one surgery is needed. The first surgery may be done to help increase blood flow to the lungs, and a surgery to correct the problem is done at a later time. Corrective surgery is done to widen part of the narrowed pulmonary tract and close the ventricular septal defect. Tips for parents of children with tetralogy of Fallot: If a child does become blue, immediately place the child on his or her side and put the knees up to the chest. Calm the baby and seek medical attention Feed the child slowly Give smaller, more frequent meals Decrease the child's anxiety by remaining calm Minimize crying by trying to anticipate the child's needs Recruit others to care for the child to prevent yourself from becoming exhausted \ Pathology Review Page 136 Tracheomalacia Acquired tracheomalacia is a weakness and floppiness of the walls of the windpipe (trachea) that develops after birth. Acquired tracheomalacia occurs when previously normal cartilage in the wall of the windpipe starts to break down. There are two types of acquired tracheomalacia. Type 2 develops when there is pressure on the airway. This may be due to an abnormality of the blood vessels surrounding the trachea or a tumor in the neck or throat. Type 3 tracheomalacia results from long term use of a breathing tube (intubation) or repeated infections involving the trachea. Acquired tracheomalacia is very uncommon. Symptoms Breathing problems that get worse with coughing, crying, or upper respiratory infections Breathing noises that may change with position and improve during sleep High-pitched breathing Rattling, noisy breaths Exams and Tests A physical examination confirms the symptoms. A chest x-ray may show narrowing of the trachea when exhaling. Even if the x-ray is normal, it is needed to rule out other problems. A procedure called a laryngoscopy provides the definitive diagnosis. This procedure allows the otolaryngologist (ear, nose, and throat doctor, or ENT) to see the structure of the airway and determine how severe the problem is. Other tests that may be performed include: Airway fluoroscopy CT scan Barium swallow Lung function tests Bronchoscopy Magnetic resonance imaging (MRI ) Treatment Persons with tracheomalacia must be monitored closely when they have respiratory infections. Continuous positive airway pressure (CPAP) may be necessary for adults with respiratory distress. Rarely, surgery is needed. A stent may be needed to hold the airway. Transient Tacypnea of the Newborn TTN; Wet lungs - newborns; Retained fetal lung fluid; Transient RDS Transient tachypnea is a respiratory disorder usually seen shortly after delivery in full- or near-term babies. Transient means it is short-lived (usually less than 24 hours). Tachypnea means rapid breathing (most normal newborns take 40-60 breaths per minute). As the baby grows in the womb, the lungs make a special fluid. This fluid fills the developing baby's lungs and helps them grow. When the baby is born at term, chemicals released during labor tell the lungs to stop making this special fluid. The baby's lungs start removing or reabsorbing it. The first few breaths your baby takes after delivery fill the lungs with air and help to clear most of the remaining lung fluid. Some of the fluid may also clear when the baby's chest is squeezed while passing through the birth canal. Babies born before 38 weeks gestation may not respond as well to the chemical signals released during labor, and there may be more fluid in the lung at birth. If you had a cesarean section without being in labor, the chemical signals telling the lung to stop making and start removing lung fluid are not as strong, again leaving more fluid in the lung at the time of birth. If you have diabetes or received large amounts of pain medications during labor your baby is also at risk of developing TTN. Symptoms Newborns with TTN have respiratory problems soon after birth, usually within 1 - 2 hours. Symptoms include: Rapid, noisy breathing, such as grunting Flaring nostrils or movements between the ribs or breastbone known as retractions Exams and Tests Pathology Review Page 137 The mother’s pregnancy and labor history are important to make the diagnosis. Tests performed on the baby may include: Chest x-ray to rule out other causes of breathing problems Blood count and blood culture to try to rule out infection TTN is usually diagnosed after monitoring your baby for 1-2 days. Treatment Nasal CPAP, Oxygen via hood or high flow oxygen usually needed. Fluid will diminish in a day or two. Tuberculosis, TB TB is a common and deadly infectious disease caused by mycobacteria, mainly Mycobacterium tuberculosis. Tuberculosis most commonly attacks the lungs (as pulmonary TB) but can also affect the central nervous system, the lymphatic system, the circulatory system, the genitourinary system, bones, joints and even the skin. Other mycobacteria such as Mycobacterium bovis, Mycobacterium africanum, Mycobacterium canetti, and Mycobacterium microti can also cause tuberculosis, but these species do not usually infect healthy adults. Over one-third of the world's population has been exposed to the TB bacterium, and new infections occur at a rate of one per second. Not everyone infected develops the fullblown disease; asymptomatic, latent TB infection is most common. However, one in ten latent infections will progress to active TB disease, which, if left untreated, kills more than half of its victims. Symptoms When the disease becomes active, 75% of the cases are pulmonary TB. Symptoms include chest pain, coughing up blood, and a productive, prolonged cough for more than three weeks. Systemic symptoms include fever, chills, night sweats, appetite loss, weight loss, pallor, and often a tendency to fatigue very easily. In the other 25% of active cases, the infection moves from the lungs, causing other kinds of TB more common in immunosuppressed persons and young children. Extrapulmonary infection sites include the pleura, the central nervous system in meningitis, the lymphatic system in scrofula of the neck, the genitourinary system in urogenital tuberculosis, and bones and joints in Pott's disease of the spine. Bacterial species Scanning electron micrograph of Mycobacterium tuberculosis The primary cause of TB , Mycobacterium tuberculosis (M. TB), is an aerobic bacterium Since MTB has a cell wall but lacks a phospholipid outer membrane, it is classified as a Gram-positive bacterium. However, if a Gram stain is performed, MTB either stains very weakly Gram-positive or does not retain dye due to the high lipid & mycolic acid content of its cell wall. Since MTB retains certain stains after being treated with acidic solution, it is classified as an acid-fast bacillus (AFB). The most common staining technique, the Ziehl-Neelsen stain, dyes AFBs a bright red that stands out clearly against a blue background. Pathology Review Page 138 Transmission When people suffering from active pulmonary TB cough, sneeze, speak, kiss, or spit, they expel infectious aerosol droplets 0.5 to 5 µm in diameter. A single sneeze, for instance, can release up to 40,000 droplets. Each one of these droplets may transmit the disease, since the infectious dose of tuberculosis is very low and the inhalation of just a single bacterium can cause a new infection. Tuberculosis is spread by aerosols created by coughing or sneezing. People with prolonged, frequent, or intense contact are at particularly high risk of becoming infected, with an estimated 22% infection rate. A person with active but untreated tuberculosis can infect 10–15 other people per year. Others at risk include people in areas where TB is common, people who inject illicit drugs (especially when sharing needles), residents and employees of high-risk congregate settings, medically under-served and low-income populations, high-risk racial or ethnic minority populations, children exposed to adults in high-risk categories, patients immunocompromised by conditions such as HIV/AIDS, people who take immunosuppressant drugs, and health care workers serving these high-risk clients. Diagnosis Mantoux tuberculin skin test Tuberculosis can be a difficult disease to diagnose, due mainly to the difficulty in culturing this slowgrowing organism in the laboratory. A complete medical evaluation for TB must include a medical history, a chest X-ray, and a physical examination. Tuberculosis radiology is used in the diagnosis of TB. It may also include a tuberculin skin test, a serological test, microbiological smears and cultures. The interpretation of the tuberculin skin test depends upon the person's risk factors for infection and progression to TB disease, such as exposure to other cases of TB or immunosuppression. Progression Progression from TB infection to TB disease occurs when the TB bacilli overcome the immune system defenses and begin to multiply. In primary TB disease—1 to 5% of cases—this occurs soon after infection. However, in the majority of cases, a latent infection occurs that has no obvious symptoms. These dormant bacilli can produce tuberculosis in 2 to 23% of these latent cases, often many years after infection.The risk of reactivation increases with immunosuppression, such as that caused by infection with HIV. In patients co-infected with M. tuberculosis and HIV, the risk of reactivation increases to 10% per year. Other conditions that increase risk include drug injection, mainly due to the lifestyle of IV drug users; recent TB infection or a history of inadequately treated TB; chest X-ray suggestive of previous TB, showing fibrotic lesions and nodules; diabetes mellitus; silicosis; prolonged corticosteroid therapy and other Pathology Review Page 139 immunosuppressive therapy; head and neck cancers; hematologic and reticuloendothelial diseases, such as leukemia and Hodgkin's disease; end-stage kidney disease; intestinal bypass or gastrectomy; chronic malabsorption syndromes; or low body weight. Treatment Treatment for TB uses antibiotics to kill the bacteria. The two antibiotics most commonly used are rifampicin and isoniazid. However, instead of the short course of antibiotics typically used to cure other bacterial infections, TB requires much longer periods of treatment (around 6 to 12 months) to entirely eliminate mycobacteria from the body. Latent TB treatment usually uses a single antibiotic, while active TB disease is best treated with combinations of several antibiotics, to reduce the risk of the bacteria developing antibiotic resistance. People with these latent infections are treated to prevent them from progressing to active TB disease later in life. Ventricular Fibrillation; VF Ventricular fibrillation (VF) is a severely abnormal heart rhythm (arrhythmia) that causes death unless immediately treated. VF is responsible for 75 - 85% of sudden deaths in persons with heart problems. To pump blood out to the body, all areas of the heart normally squeeze (contract) at the same time. The heart’s upper chambers (the atria) contract before the heart’s bottom two chambers (ventricles). During VF, however, the contractions become disorganized. The most common cause of VF is a heart attack, but VF can occur whenever the heart does not get enough oxygen or if a person has other heart disorders. Conditions that can lead to VF include: Congenital heart disease Heart surgery Electrocution accidents or injury to the Ischemia (lack of oxygen to the heart heart muscle because of narrowed coronary arteries or shock) Heart attack Heart muscle disease, including cardiomyopathies While most people suffering from VF have no previous history of heart disease, many have risk factors for cardiovascular disease, such as smoking, hypertension, and diabetes. Symptoms A person who has a VF episode will suddenly collapse or become unconscious, because the brain and muscles have stopped receiving blood from the heart. The following symptoms may occur within 1 hour before the collapse: Chest pain Rapid heart beat Dizziness Shortness of breath Nausea Exams and Tests VF is an emergency condition. Seek immediate medical attention. The pulse in the neck and groin area may be hard or impossible to feel. The person will be unresponsive. The health care provider will listen to the heart with device called a stethoscope. The heartbeats may not be heard, or they may be very irregular. A cardiac monitor will show a disorganized heart rhythm. Treatment Immediate Defibrillation with either 200 (biphasic) or 360 (monophasic) defibrillator. Followed by CPR and ACLS drugs. Pathology Review Page 140 Ventricular Septal Defect; Interventricular septal defect Ventricular septal defect describes one or more holes in the wall that separates the right and left ventricles of the heart. Ventricular septal defect is one of the most common congenital (present from birth) heart defects. Before a baby is born, the right and left ventricles of its heart are not separate. As the fetus grows, a wall forms to separate these two ventricles. If the wall does not completely form, a hole remains. This hole is known as a ventricular septal defect, or a VSD. It is estimated that up to 1% of babies are born with this condition. The baby may have no symptoms, and the hole can eventually close as the wall continues to grow after birth. If the hole is large, then too much blood will be pumped to the lungs, leading to congestive heart failure. These babies are often have symptoms related to heart failure and may need medicine to control the symptoms and surgery to close the hole. The cause of VSD is not yet known. This defect often occurs along with other congenital heart defects. In adults, interventricular septal defects are a rare, but serious complication of heart attacks. These holes are related to the heart attack and do not result from a birth defect. Symptoms Shortness of breath Fast heart rate Fast breathing Pounding heart Hard breathing Sweating while feeding Paleness Frequent respiratory infections Failure to gain weight Exams and Tests Listening with a stethoscope usually reveals a heart murmur (the sound of the blood crossing the hole). The loudness of the murmur is related to the size of the defect and amount of blood crossing the defect. Tests may include: Chest x-ray -- looks to see if there is a large heart with fluid in the lungs ECG -- shows signs of an enlarged left ventricle Echocardiogram -- used to make a definite diagnosis Cardiac catheterization (rarely needed, unless there are concerns of high blood pressure in the lungs, in which case surgery to close the defect is generally not recommended) Treatment If the defect is small, no treatment is usually needed. However, the baby should be closely monitored by a health care provider to make sure that the hole eventually closes properly and signs of heart failure do not occur. With congestive heart failure, medications such as digitalis (digoxin) and diuretics may be prescribed. Regardless of the size of the defect, all children with a VSD need to take antibiotics before undergoing dental work and certain other invasive procedures. If symptoms continue despite medication, surgery to close the defect with a Gore-tex patch is needed. Some VSDs can be closed with a special device during a catheterization. Treating a VSD that does not have symptoms is controversial, and should be carefully discussed with your health care provider Pathology Review Page 141 Ventricular tachycardia VT is a rapid heart beat initiated within the ventricles, characterized by 3 or more consecutive premature ventricular beats. Ventricular tachycardia is a potentially lethal disruption of normal heartbeat (arrhythmia) that may cause the heart to become unable to pump adequate blood through the body. The heart rate may be 160 to 240 (normal is 60 to 100 beats per minute). Ventricular tachycardia can occur in the absence of apparent heart disease. It can also develop as an early or a late complication of a heart attack, or during the course of cardiomyopathy, valvular heart disease, myocarditis, and following heart surgery. Healed heart attacks form scar tissue which can lead to ventricular tachycardia. This can occur days, months, or years after the heart attack. Ventricular tachycardia can also result from anti-arrhythmic medications (an undesired effect) or from altered blood chemistries (such as a low potassium level), pH (acid-base) changes, or insufficient oxygenation. A common mechanism for ventricular tachycardia is reentry (re-stimulation of the electrical conductive pathway from a single initial stimulus). Ventricular tachycardia is classified as nonsustained (often defined as lasting less than 30 seconds) or sustained. "Torsade de pointes" is a form of ventricular tachycardia with a specific variation in the conduction of the ventricular stimulus. Symptoms Sensation of feeling the heart beat Shortness of breath (palpitations) Chest discomfort (angina) Light-headedness or dizziness Fainting Note: Symptoms may start and stop suddenly. In some cases, there are no symptoms. Ventricular tachycardia can occur in episodes during which the person will have a rapid pulse or the symptoms described above. The blood pressure may be normal or low. Loss of consciousness may occur. Ventricular tachycardia is a potentially lethal arrhythmia and may result in an absent pulse. Ventricular tachycardia may be seen on: An ECG A continuous ambulatory electrocardiogram (Holter monitor) A loop recorder, for ambulatory ECG recordings exceeding 24 hrs An intracardiac electrophysiology study (EPS) Blood chemistries and other tests may be performed. Treatment Treatment varies with the symptoms, the situation, and the underlying cardiac disorder. No treatment may be required in some cases. Ventricular tachycardia may become an emergency situation and may be require CPR, electrical defibrillation or cardioversion (electric shock), or intravenous anti-arrhythmic Pathology Review Page 142 medications (such as lidocaine, procainamide, bretylium, or sotalol). Long-term treatment of ventricular tachycardia may require the use of oral anti-arrhythmic medications (such as procainamide, amiodarone, or sotalol). Anti-arrhythmic medications, however, may have severe side effects, and their use is currently decreasing in favor of other treatments. Some ventricular tachycardias may show in the electrophysiologic study to be suitable for an ablation procedure. Radiofrequency catheter ablation is a curative treatment for selected tachycardias. Pathology Review Page 143