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Systemic Disorders Chapter 11 Medical Considerations Hyperpituitarism  Excess hormone production by the anterior pituitary gland  Caused most often by a benign tumor (pituitary adenoma) that produces growth hormone  Giantism results if it occurs before the closure of long bones.  Acromegaly results when hypersecretion occurs during adult life. Clinical Features and Oral Manifestations of Hyperpituitarism  Affects both men and women, most commonly during the fourth decade of life  Patients experience poor vision, light sensitivity, enlargement of hands and feet, and an increase in rib size.  Facial changes  Enlargement of maxilla and mandible may cause separation of teeth and malocclusion.  Frontal bossing and an enlargement of nasal bones may lead to deepening of voice.  Mucosal changes  May have thickened lips and macroglossia Diagnosis and Treatment of Hyperpituitarism  Diagnosis involves measurement of growth hormone.  Treatment often includes pituitary gland surgery. Hyperthyroidism (Thyrotoxicosis)  Excess production of thyroid hormone  More common in women than men  The most common cause is Graves disease Graves disease Appears to be due to an autoimmune disorder in which a substance is produced that abnormally stimulates the thyroid gland  Other causes include hyperplasia of the gland, benign and malignant tumors of the thyroid, pituitary gland disease, and metastatic tumors. Clinical Features of Hyperthyroidism  Rosy complexion, erythema of the palms, excessive sweating, fine hair, softened nails  The patient may have exophthalmos.  Anxiety, weakness, restlessness, and cardiac problems may also be associated. Treatment of Hyperthyroidism  May include surgery, medications to suppress thyroid activity, or administration of radioactive iodine Hypothyroidism  A decreased output of thyroid hormone  Causes include developmental disturbances, autoimmune disease, iodine deficiency, drugs, and pituitary disease  Cretinism When it occurs in infancy and childhood  Myxedema When it occurs in older children and adults Hypothyroidism (cont.)  Oral manifestations  In infants Thickened lips, enlarged tongue, and delayed eruption of teeth  In adults Enlarged tongue Addison Disease  Primary adrenal cortical insufficiency  In most cases, the cause of destruction of the adrenal gland is unknown – it may be an autoimmune disease.  It may be due to a tumor or tuberculosis.  To compensate, the pituitary gland increases production of ACTH. Addison Disease (cont.)  Clinical features  This hormone causes stimulation of melanocytes.  Bronzing of the skin may occur, as well as melanotic macules on oral mucosa.  Treatment  Steroid replacement therapy Blood Disorders  Disorders of Red Blood Cells and Hemoglobin  Disorders of White Blood Cells  Bleeding Disorders Blood Disorders (cont.)  The complete blood count examines red blood cells, white blood cells, and platelets.  It provides information about the number of each type of cell, the ratio of types, and the appearance of the cells. Anemia  A reduction in the oxygen-carrying capacity of blood  Most often related to a decrease in the number of circulating red blood cells Nutritional anemias A deficiency in a substance required for the normal development of red blood cells, commonly vitamins Suppression of bone marrow stem cells Anemia (cont.)  Clinical features  Pallor of skin and oral mucosa  Angular cheilitis  Erythema and atrophy of oral mucosa  Loss of filiform and fungiform papillae on the dorsum of the tongue Iron Deficiency Anemia  An insufficient amount of iron is supplied to bone marrow for red blood cell development.  May occur as a result of deficient iron intake, blood loss from heavy menstrual bleeding or chronic gastrointestinal bleeding, poor iron absorption, or an increased requirement for iron in situations such as pregnancy or infancy  Plummer-Vinson syndrome may result from long standing iron deficiency anemia.  Includes dysphagia, atrophy of the upper alimentary tract, and a predisposition to developing oral cancer Clinical Features and Oral Manifestations of Iron Deficiency Anemia  Often asymptomatic, may have nonspecific symptoms such as weakness and fatigue  In severe cases may see angular cheilitis, pallor of oral tissue, and an erythematous, smooth, painful tongue Diagnosis and Treatment of Iron Deficiency Anemia  Laboratory tests show a low hemoglobin content and reduced hematocrit.  Red blood cells appear smaller than normal (microcytic) and light in color (hypochromic)  Treatment  Dietary supplements Sickle Cell Anemia  An inherited blood disorder  When someone is heterozygous, it is called sickle cell trait.  When someone is homozygous, they are much more severely affected.  Occurs before age 30 and is more common in women than in men  The red blood cells develop a sickle shape when there is decreased oxygen.  This can be triggered by exercise, exertion, administration of a general anesthetic, pregnancy, or even sleep. Clinical Features and Oral Manifestations of Sickle Cell Anemia  The person has weakness, shortness of breath, fatigue, joint pain, and nausea.  Radiographic  There is a loss of trabeculation, and large, irregular marrow spaces appear.  A “hair-on-end” pattern may be seen in the skull. Diagnosis and Treatment of Sickle Cell Anemia  The sickle-shaped cells may be seen on a blood smear.  The number of red blood cells is usually low, as is the hemoglobin content.  Treatment is largely supportive, involves administration of oxygen and IV and oral fluid. Leukemia  Malignant neoplasms of hematopoietic stem cells  Characterized by an excessive number of abnormal white blood cells in circulating blood  Unknown cause; some are investigating oncogenic viruses  There are many different types categorized as to whether they are acute or chronic. Acute Leukemias  Characterized by very immature cells and a rapidly fatal course if not treated  Acute lymphoblastic leukemia – involves immature lymphocytes Primarily affects children and young adults Good prognosis  Acute myeloblastic leukemia – involves immature granulocytes Primarily affects adolescents and young adults. Prognosis is not as good. Hemophilia  A disorder of blood coagulation  Results in severely prolonged clotting time  Due to a deficiency in plasma proteins involved in coagulation Types of Hemophilia  The two most common types are type A and type B.  Transmitted as X-linked diseases through an unaffected carrier daughter to a son Type A Caused by a deficiency of plasma thromboplastinogen or factor VIII Type B Christmas disease Less common, the clotting defect is plasma thromboplastin or factor IX