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Dr. Balderas Feb. 11, 2010 Pediatrics PATIENT HISTORY Francisco, R., Garcia, J., Garcia, M.M., Garcia, M.R., Garcia, R.K., Garcia, W.K., Garimbao, J. 2011-A Identifying Data EMA, an 8-year old, Filipino, Roman Catholic female, from Parang, Marikina was admitted for the 1st time at UERMMMCI Pediatrics Ward last Feb 3, 2010. Source and Reliability The source was the patient herself as well as her mom and aunt who were of fair reliability Chief Complaint Fever of 13 days and abdominal pain of 2 days History of Present Illness (HPI) 13 days pta, the patient suddenly developed a low grade fever (undocumented) which was intermittent. She was then medicated Paracetamol tablet 500mg 2x/day offering temporary relief of the fever. No other symptom was noted at this time. The fever continued intermittency until… 10 days pta, with her fever still unresolved, she developed cough described as productive with whitish phlegm, non-bloody with no noted aggravating or alleviating factors. She was also noted to have a swelling of the face which spontaneously subsided at the end of the day. There were no changes noted in the patient’s activity, eating and drinking habits as well as that of her bowel movements and urination. No associated headaches, joint or muscle pains were reported. No consultation was done in the hope that all her symptoms would resolve eventually but 6 days pta, with the persistence of the symptoms stated above, the patient now had blood in her urine described as red discoloration of the urine as reported by the patient. However, the mother ignored the patient’s complaint since she was unable to see the bloody urine for herself. Still, no consult was done. 2 days pta, facial edema was not apparent anymore but still with the fever and the cough and with the patient’s assertion of having a bloody urine, she was asked to urinate in a urinal pan. Amount and odor were not noted but urine was characterized by the mother as red in color. By this time the patient has begun feeling an intermittent peri-umbilical pain which was not associated with eating, defecation or urination. 1 day pta, fever and cough persisted with no noted worsening or improvement despite intake of Paracetamol as needed, although abdominal pain has increased which prompted them to seek consult at a clinic in the vicinity. IV medication unknown to the mother was given but no relief was noted until A few hours pta, still with cough and the increasing abdominal pain, the fever was now noted to have increased to moderate or high but without chills (undocumented). Because of this the patient was brought to our institution and was admitted thereafter Throughout the course of the patient’s illness prior to her admission there were no changes noted in her appetite, stool character, and weight and there was no complaints of sore throat, headache, vomiting, joint pains, myalgia and skin rash. Past History Birth History (Prenatal, Birth, and Neonatal) There was no fetomaternal complication during the course of pregnancy with regular check-ups, the details of which were unrecalled other than the mother being noted to be eclamptic/hypertensive. Medications/supplementations were not taken and the mother did not smoke, had no alcohol or illegal drug intake. The patient was born full term (unrecalled AOG) on January 11, 2001, to a then 34 y/o, G4P4(3013) mother via normal spontaneous delivery, physician assisted at Amang Rodriguez Hospital in Marikina. BW was 4lb while BL and APGAR were unknown but the patient gave a good cry and did not need any resuscitation. No complication/illness was noted during the first 28 days of the patient’s life except for pneumonia lasting for 4 days after 1 week from birth which warranted hospitalization at Amang and was then treated accordingly. The patient recovered with no complications and was then sent home Immunization History Vaccines were said to be complete, all of which were obtained from the health center Feeding History She was solely breastfed for a month and was then introduced to formula milk at 2 months (Bona) of 1:1 dilution ratio and was then shifted to bonakid from 6 months until her 1 st year wherein the formula was changed to Bear brand which the patient still consumes at present though not on a regular basis. Further details of her past feeding were unrecalled but was said to be introduced to solid foods at 6 months starting with rice. At present, the patient eats 3x a day consisting of a half cup of rice and a viand which would usually be hotdog, longganisa or sinigang na baboy. She is said to be picky and so she doesn’t eat vegetables. She also loves softdrinks and junk food. Throughout her development, no allergic reaction to any food introduced to her was noted. Developmental History Growing up, her development was not deviant from that of her siblings. She is currently in grade 3 at a public school near their home garnering average scores. She can socialize well and has lots of friends. Playing outside with neighbors is her usual past time is said to like dancing much. She would throw tantrums at times mainly due to rivalry with siblings about toys. She has no mannerisms or tics, nightmares or other sleeping problems. Past Illnesses/Hospitalizations No major illness/hospitalization/surgery/ history of allergic reactions prior to the current admission apart from pneumonia during 1st week of life as stated above. Family History (-) Hypertension (-) Diabetes Mellitus (-) CVA (-) Kidney Problems (-) Cancer (-) TB (-) Asthma (+) Aplastic anemia (grandmother paternal side) Patient Profile (Social and Environmental) The patient lives in a 1-story house with 1 toilet along with 5 other occupants in Parang, Marikina. The place is said to be clean and far from dumpsite, factory, or river with garbage disposal 3x a day. Water for both drinking and household purposes are from the faucet. Her father working as a construction worker and brother, stockman in a department store, are the main source of income aside from their own retail store which is manned by her mother most of the time. Finances are said to be adequate to meet daily needs. No one else in the family is known to suffer from the same illness as that of the patient. PHYSICAL EXAMINATION General Survey: Patient is awake, alert, and ambulatory but uncooperative and is not in any cardio-respiratory distress Vital Signs PR: BP: RR: Temperature: Anthropometrics: Length: 64in (162.56cm) Weight: 49kg (108.03lbs) BMI: 18.5 Skin: coarse due to presence of pink-pinkish red maculopapular rash distributed on all his extremities, and red scar-like lesions on the back, warm to touch with normal turgor, nails pink with no signs of infection, pallor, cyanosis or clubbing HEENT: Head: The skull was symmetric, atraumatic with well distributed black hair, no hair loss/infestations. Scalp without any masses, lesions, signs of trauma and pigmentations. No facial asymmetry was noted. Face have scars on the nasolabial line with red non pruritic patches on the cheeks (~0.5-3cm in diameter) Eyes: Eyes are symmetrical, with no exopthalmos/enophthalmos or edema, anicteric sclera and pink conjunctivae with both pupils constricting on direct and indirect pupillary reflex test. VA: OS= J2, OD=J1. Fundoscopy: (+) ROR Ears: Auricles are aligned with no gross deformities, lesions, swelling or masses or discharge. Nose: no gross deformities, swelling, bleeding, lesions, erosions, masses, infections. Nasal septum at the midline Mouth: Pink, moist lips with no deformities but with pustules (~1-2mm) on the buccal mucosa, uvula and tongue at the midline, with no tonsillar redness/enlargement Neck: Trachea midline, thyroid is non palpable as well as lymph nodes Thorax and Lungs: Chest symmetrical with equal expansion and excursion and without gross abnormalities or respiratory lag. No intercostal retractions, no use of accessory respiratory muscles. No lesions, masses on anterior chest but with scars on the back with no tenderness noted, equal tactile fremitus. Vesicular breath sounds with no adventitious sounds on auscultation Cardiovascular: Adynamic precordium with no deformities/palpable thrills or bruits. Distinct S1 and S2 heard loudest at the 4th-5th ICS left parasternal border with regular rate and rhythm. No murmurs or adventitious heart sounds noted Abdomen: It was flat and symmetrical with no lesions/deformities/discolorations. Normoactive bowel sounds with no bruits over all quadrants. Quadrants were non-tender and tympanitic. Musculoskeletal: There were no gross deformities of joints and extremities, with normal tone and a muscle grade of 5/5 on all muscle groups with full range of motion and no crepitations. No tenderness on joints and extremities. Genitourinary: Tanner stage I, uncircumcised penis, foreskin fully covering the glans with no lesion/gross deformity on the shaft. Testes are descended, scrotal rugae present MSE: the patient is awake, alert, and cooperative Cranial Nerves: I- intact for both nostrils IIDirect Pupillary Reflex: OU=intact Indirect Pupillary Reflex: OU=intact Fundoscopy: OU=(+) ROR III, IV and VI- full range of motion with no nystagmus V-sensory: intact VII-face is symmetric with no weakness VIII- no gross hearing impairment IX-X: uvula at the midline with synchronous swallowing and intact gag reflex XI- good shoulder shrug XII- tongue is at midline Reflexes: Deep tendon reflexes on biceps, triceps, knee and ankle have a grade of +2, (-) Babinski reflex Sensory: pain perception is intact for all extremities Cerebellar: intact Primary Impression: Acute Poststreptococcal Glomerulonephritis Differential Diagnoses: DIFFERENTIAL DX: Henoch-Schonlein Purpura RULE IN: -Renal involvement -Gross Hematuria -Asian prevalence -Occurs mainly in young children. RULE OUT: -Typical anaphylactoid purpura ex: Henoch-Schonlein Purpura. -Arthritis and/or arthralgia -No fever -Boys Urinary Tract Infection (UTI) -Hematuria -Fever -Female gender -Increased blood pressure -Edema -Dark urine -Fever -Female gender -Asian -Blood pressure and GFR affected. -(Some) Acute nephritis and hematuria. -Edema -No edema -No increased blood pressure. Systemic Lupus Erythematosus (SLE) Membranoproliferative Glomerulonephritis (MPGN) -Butterfly skin rash -Arthritis -Most commonly 20-45 years of age (onset.) -Young adults. -Urinary abnormalities persist past time of expected resolution for acute poststrep. Glomerulonephritis. Primary Diagnosis: Acute Poststreptococcal Glomerulonephritis ETIOLOGY AND EPIDEMIOLOGY. Acute poststreptococcal glomerulonephritis, aside from IgA nephropathy, is one of the most common glomerular causes of gross hematuria in children. Acute poststreptococcal glomerulonephritis develops when there is infection of the throat or skin by certain nephritogenic strains of group A β-hemolytic streptococci. Types 1, 2, 4, 3, 25, 49, and 12 are more associated with pharyngitis. It typically affects children between the ages of 2 and 14 years. This certain patient being 8 ½ years old makes her part of the common age group for the disease. However it is more common in males and the familial or cohabitant incidence is as high as 40%. As seen in the history, this patient is a female and none of the other family members show the same signs and symptom concurrently with the patient. Poststreptococcal glomerulonephritis is considered a sequelae of streptococcal pharyngitis especially rampant during cold weather months. Its counterpart infection during the warmer summer months is a streptococcal skin infections or pyoderma. PATHOPHYSIOLOGY Most forms of acute poststreptococcal glomerulonephritis are mediated by an immunologic process. Both cellular and humoral immunity is important in the pathogenesis of acute poststreptococcal glomerulonephritis. Humoral immunity in acute poststreptococcal glomerulonephritis is presumed to be mediated by the in situ formation of nephritogenic streptococcal antigen-antibody complexes and circulating immune complexes . The most widely proposed mechanism for the development of acute poststreptococcal glomerulonephritis is that nephritogenic streptococci produce proteins with unique antigenic determinants. These antigenic determinants have a particular affinity for sites within the normal glomerulus. Following release into the circulation, these antigens bind to these sites within the glomerulus. Once bound to the glomerulus, they activate complement directly by interaction with properdin. Glomerular-bound streptococcal antibodies also serve as fixed antigens and bind to circulating antistreptococcal antibodies forming immune complexes. Complement fixation via the classical pathway leads to generation of additional inflammatory mediators and recruitment of inflammatory cells. The 2 major nephritogenic antigens that have been identified are zymogen, a precursor of exotoxin B called SPEB (also described as nephritis strain-associated protein [NSAP]), and nephritis plasmin binding protein (NAPlr). NSAP was detected in renal biopsies of patients with acute poststreptococcal glomerulonephritis but not in other forms of AGN or rheumatic fever . NSAP has antigenic, biochemical, and structural similarities to streptokinase from group C streptococcal organisms, binds to plasmin, and is a plasminogen activator. Bound plasmin can cause tissue destruction by direct action on the glomerular basement membrane or by indirect activation of procollagenases and other matrix metalo-proteinases. NAPlr can also activate the alternate complement pathway, leading to accumulation of polymorphonuclear cells and macrophages and local inflammation. Also, the in situ–formed and circulating immune complexes can readily pass through the altered glomerular basement membrane and accumulate on the subepithelial space as humps. Other nonimmmune complex mediated mechanisms have been proposed for the development of acute poststreptococcal glomerulonephritis. Firstly, a role for delayed-type hypersensitivity has been implicated in the pathogenesis of this disease. Early in the course, resident endothelial and mesangial cells are predominantly proliferated, and this is accompanied by infiltration with polymorphonuclear leukocytes and monocytes. Macrophages are effector cells that cause resident cellular proliferation. The infiltration of macrophages in the glomeruli is mediated by complement-induced chemotaxis and, most likely, by an antigen-specific event related to delayed-type hypersensitivity mediated by helper/inducer T cells. Secondly, streptococcal M proteins and pyrogenic exotoxins can act as superantigens. These cause a marked expansion of T cells expressing specific T-cell receptor B-chain variable gene segments. Thirdly, autologous immunoglobulin G (IgG) in acute poststreptococcal glomerulonephritis becomes antigenic and elicits an anti-IgG rheumatoid factor response, leading to formation of cryoglobulins. Cryoglobulins, rheumatoid factors, and other autoimmune phenomena occur in acute poststreptococcal glomerulonephritis and are thought to play a role in the pathogenesis of the disease together with streptococcal superantigens. As observed by light microscopy, glomerular changes are generalized and diffuse. The glomerular tufts usually appear enlarged and swollen, and a moderate-to-marked increase in proliferation of mesangial and epithelial cells is present. Polymorphonuclear leukocytes are also often observed as part of the inflammatory process. In persons with the most severe disease, the glomeruli appear bloodless because of the associated edema of the capillary walls, which impedes glomerular perfusion. A direct correlation exists between the severity of the histologic process and the clinical manifestations of the disease during the acute phase and possibly the prognosis. Granular deposits of IgG and C3 are typically found when the specimen is studied by immunofluorescent microscopy; other immunoglobulins (Igs) and fibrinogen are often observed. Electron microscopy of renal tissue from patients with poststreptococcal acute glomerulonephritis usually reveals subepithelial electron-dense deposits (humps). In most patients with moderate-to-severe AGN, a measurable reduction in volume of glomerular filtrate (GF) is present, and the capacity to excrete salt and water is usually diminished, leading to expansion of the extracellular fluid (ECF) volume. The expanded ECF volume is responsible for edema and, in part, for hypertension, anemia, circulatory congestion, and encephalopathy. CLINICAL MANIFESTATIONS Poststreptococcal glomerulonephritis is most common in children aged 5–12 years old, this patiend being part of this age group. The typical patient develops an acute nephritic syndrome 1–2 wk after an antecedent streptococcal pharyngitis. In this patient, she developed cough 4 days prior to the claims of hematuria. Depending on the severity of renal involvement, patients may develop various degrees of edema, hypertension, and oliguria. This patient particularly developed facial edema 6 days prior to admission. Edema typically results from salt and water retention; nephrotic syndrome may develop in 10–20% of cases. Patients may develop encephalopathy and/or heart failure owing to hypertension or hypervolemia. Encephalopathy may also possibly result from the direct toxic effects of the streptococcal bacteria on the central nervous system via bacteremia. Nonspecific symptoms such as malaise, lethargy, abdominal or flank pain, and fever are common, in which this patient’s chief complaint was abdominal pain and fever. The acute phase generally resolves within 6–8 wk. Although urinary protein excretion and hypertension usually normalize by 4–6 wk after onset, persistent microscopic hematuria may persist for 1–2 yr after the initial presentation. Diagnostics Diagnosis of this disease makes use of the ff: Antibody titers (ASO), BUN and creatinine levels and other serological findings, and urinalysis. But basically, high index of suspicion is all that is necessary since ASO titers are mainly done to detect previous streptococcal infection as further discussed below and are mainly used for statistical purposes and this disease may be diagnosed clinically. Antibody titers to extracellular products of streptococci show evidence of preceding streptococcal infection and are positive in more than 95% of patients with pharyngitis and 80% of patients with skin infections. The antistreptolysin (ASO), antinicotinamide adenine dinucleotidase (anti-NAD), antihyaluronidase (AHase), and anti–DNAse B are commonly positive after pharyngitis, and anti–DNAse B and AHase titers are more often positive following skin infections. ASO titers are frequently used to document streptococcal infection, but a more sensitive test is the streptozyme test, which tests antibodies to ASO, anti–DNAse B, AHase, and anti-NAD. Studies suggest that the relatively unavailable antizymogen titer test is superior to both anti–DNAse B and ASO titers. Antizymogen titers that are 2 dilutions higher than the mean in healthy controls are reported to have a sensitivity of 88% and a specificity of 85% in the diagnosis of streptococcal infection in patients with glomerulonephritis. In addition, high antibody titers to glyceraldehydes phosphate dehydrogenase are also found in persons with acute poststreptococcal glomerulonephritis (APSGN). In general, the antibody titers are elevated at 1 week, peak at 1 month, and fall toward preinfection levels after several months. Patients with APSGN also have elevated BUN and creatinine values. This reflects the decrease in the glomerular filtration rate that occurs in the acute phase. The elevations are usually transient. Their failure to normalize within several weeks or months indicates that the patient may not have a true APSGN and suggests seeking an alternative diagnosis. Low serum complement levels indicative of an antigen-antibody interaction are a universal finding in the acute phase of APSGN. In most uncomplicated cases, the complement levels return to normal in 6-8 weeks. Prolonged hypocomplementemia suggests an alternative diagnosis. Occasionally, low complement levels persist for 3 months. The level of reduction of serum complement levels does not have any prognostic significance. Urinalysis results are always abnormal showing hematuria and proteinuria which are present in all cases. Urine sediment has red blood cells, red blood cell casts, white blood cells, granular casts, and, rarely, white blood cell casts. Dysmorphic red blood cells indicative of glomerular hematuria can usually be detected by performing phase-contrast microscopy. Red blood cell casts are best detected in first, early-morning urine specimens examined by the physician immediately after the patient voids. Hematuria usually resolves within 3-6 months but may persist as long as 18 months. Microscopic hematuria may be present in patients in whom the disease has otherwise clinically resolved. Proteinuria may be mild or so severe that it causes nephrotic syndrome. Approximately 5-10% of patients with APSGN have nephrotic-range proteinuria. Proteinuria usually disappears in 6 months. A mild increase in urinary protein excretion is present in 15% at 3 years and 2% at 10 years. Patients with nephrotic-range proteinuria in the acute phase or persistent heavy proteinuria have a worse prognosis. This is often associated with an evolution to a garlandlike pattern of immune deposits as the disease progresses. Chest radiographs may show findings of congestive heart failure. Renal ultrasound images usually reveal normalsized kidneys bilaterally. Imaging studies may be necessary to exclude or check for other complications like heart enlargement due to congestive heart failure. If presentation is atypical, a biopsy of the kidney may be required. PSGN would then reveal hypercellularity of the glomeruli which is the most striking characteristic of this disease with diffuse proliferation and leukocytic and mononuclear infiltration on microscopy. Basement membrane and blood vessels however are normal. Treatment Treatment is supportive and focuses on control of hypertension and edema if present. A loop diuretic (furosemide) should be given in order to remove excess fluid which reduced edema and also helps to correct hypertension. Other things that help to control blood pressure include avoiding foods that contain high amounts of salt. An antibiotic for streptococcal infection such as penicillin is used to control local symptoms and to prevent spread of infection to close contacts. However, antimicrobial therapy does not appear to prevent the development of GN, except if given within the first 36 hours. PSGN has a good prognosis with permanent renal failure being very uncommon (1-3%). Within a week or so of onset, most patients with PSGN begin to experience spontaneous resolution of fluid retention and hypertension. Complete resolution of hematuria and proteinuria occurs within 3-6 weeks of the onset of nephritis but proteinuria may persist for 6 months and microscopic hematuria for up to 1 year after onset. Eventually all urinary abnormalities should disappear, hypertension should subside, and renal function should return to normal.