256. The answer is b. (Tierney, 39/e, pp 1130-1133.) Cushing syndrome occurs secondary to corticosteroid use, nonpituitary neoplasms (i.e., small cell carcinoma of the lung), adrenal adenomas, adrenal carcinomas, and bilateral adrenal nodular hyperplasia. Cushing's disease is hypercortisolism due to ACTH hypersecretion by the pituitary gland, usually because of a small (<1 cm), benign pituitary microadenoma. Symptoms include central obesity, striae, hirsutism, easy bruisability, proximal myopathy, osteoporosis, amenorrhea, hypertension, glucose intolerance, and hypokalemia. Urinary cortisol is a good screening test for Cushing syndrome. Alcoholic patients and depressed patients may have hypercortisolism (pseudo-Cushing state). Polycystic ovary disease (SteinLeventhal syndrome) is a disorder that causes increased levels of testosterone, hirsutism, infertility, and menstrual irregularity.
257. The answer is a. (Tierney, 39/e, pp 1088-1090.) Acromegaly (hypersecretion of growth hormone after closure of the epiphyses) is almost always caused by a pituitary adenoma (benign 99% of the time). Patients present with tall stature, large hands, large feet, prominent mandible, prog-nathism, coarse facial features, wide tooth spacing, deep voice, macroglos-sia, and carpal tunnel syndrome. Patients may have headache, visual field defects, hypertrophy of the laryngeal tissue causing obstructive sleep apnea, hypertension, cardiomegaly, multiple skin tags, premalignant colonic polyps, and diabetes mellitus. Gigantism occurs before the closure of the epiphyses. Amyloidosis is a group of disorders characterized by infiltration of various organs (kidney, heart, intestine, endocrine) by protein fibrils. Patients with amyloidosis may have macroglossia and carpal tunnel syndrome. Macroglossia is also seen in hypothyroidism. Coarse features may run in families (familial prognathism).
258. The answer is e. (Seidel, 4/e, pp 255-257.) Examination of the thyroid may be done from the front or the back of the patient. Either way, the patient should be positioned with the neck flexed slightly forward and laterally to the side being examined. The thyroid gland should rise freely with swallowing. The consistency of the thyroid should be smooth and pliable. Coarse or gritty tissue implies an inflammatory process. The right lobe of the thyroid is 25% larger than the left lobe.
259. The answer is a. (Tierney, 39/e, pp 1106-1115.) The patient most likely has Graves' disease (Basedow's disease), which is the most common cause of thyrotoxicosis or hyperthyroidism. Other signs of Graves' disease are heat intolerance, menstrual irregularity, weight loss, and pretib-ial myxedema. Patients present between the ages of 20 and 40, and women are affected more than men. The disorder is due to antibodies that bind to the thyroid stimulating hormone (TSH) receptor, causing it to stimulate the thyroid gland to hyperfunction. Plummer's disease is an autonomous toxic adenomatous disease of the thyroid; patients do not present with ophthalmopathy or dermapathy. Hashimoto thyroiditis or chronic lym-phocytic thyroiditis is a common autoimmune disease (positive antimi-crosomal antibodies and antithyroid peroxidase antibodies) that causes hypothyroidism. Patients present with a diffuse, firm, nontender goiter. Patients with Hashimoto thyroiditis are susceptible to postpartum thy-roiditis. Struma ovarii is thyroid tissue contained in a dermoid ovarian tumor. Granulomatous or subacute (de Quervain's) thyroiditis is due to release of preformed thyroglobulin and follows a viral infection; the thyroid gland is painful and tender to palpation. The treatment for subacute thy-roiditis is aspirin or nonsteroidal anti-inflammatory agents.
260. The answer is d. (Tierney, 39/e, pp 1104-1106.) Symptoms of hypothyroidism include constipation, depression, edema, tongue thickening, cold intolerance, Queen Anne sign (missing lateral one-third of eyebrows), muscle cramps, weight gain, goiter, amenorrhea, galactorrhea, pleural effusion, pericardial effusion, cardiomegaly, bradycardia, hypothermia, hyponatremia, anemia, and hypertension. Patients are said to have "hung-up" reflexes (a prolonged relaxation phase). Amiodarone has high iodine content and causes hypothyroidism in 8% of patients. Myxedema is a rare complication of hypothyroidism; patients present with coma, severe hypotension, hypothermia, hypoventilation, and hypoxemia. Cretinism is congenital (infantile) hypothyroidism.
261. The answer is a. (Fauci, 14/e, p 1990.) A pituitary tumor may impinge on the optic chiasm. The temporal field fibers are damaged as they decussate at the optic chiasm and the result is a bitemporal hemianopsia.
262. The answer is d. (Sapira, p 490.) Tapping on cranial nerve VII as it exits the parotid gland will cause spasm or contraction of the facial muscles on the same side of the face that is being tapped in states of hypocalcemia (tetany). This is called the Chvostek sign. Clinical signs of hypocalcemia include paresthesias, neuromuscular irritability, and a prolonged QT interval on electrocardiogram. Hypocalcemia causes rickets in children and osteomalacia in adults. The clinical presentation of hypercalcemia consists of "bones" (fractures, osteitis fibrosa), "stones" (renal calculi), "abdominal groans" (anorexia, constipation, vomiting, peptic ulcers, pancreatitis), and "psychic overtones" (anxiety, depression, and insomnia). Patients with hypokalemia present with muscle weakness, muscle cramps, and flaccid paralysis. Hyperkalemia may lead to areflexia, flaccid paralysis, and electro-cardiographic abnormalities such as peaked T waves, prolongation of the PR interval, widening of the QRS complex, and ventricular tachycardia.
263. The answer is b. (Seidel, 4/e, pp 267-268.) A thyroglossal ductal cyst is a midline neck structure and is a remnant of the passage of the thyroid gland from the base of the tongue into the neck. A carotid body tumor arises from the carotid body at the bifurcation of the common carotid artery. A lipoma is a fatty tumor that can be found anywhere in the subcutaneous tissue. A thyroid bruit, usually seen with Graves' disease (hyperthyroidism), is turbulent blood flow heard with a stethoscope. A goiter is an enlarged thyroid gland. A brachial cleft cyst is a lateral neck structure, usually located near the upper third of the sternocleidomastoid muscle, and is a remnant of embryologic development. Laryngoceles are lateral neck swellings that increase in size with Valsalva maneuver.
264. The answer is c. (Fauci, 14/e, pp 1988, 1997, 2051-2054.) Ninety percent of the adrenal gland must be destroyed for Addison's disease (hypo-adrenalism) to develop; glucocorticoids, mineralocorticoids, and androgens are affected. Etiologies include tuberculosis, malignancy, sarcoidosis, trauma, histoplasmosis, hemochromatosis, amyloidosis, sepsis, cytomegalovirus infection, and medications (ketoconazole, rifampin, anticoagulants, and anticonvulsants). Patients without a clear etiology have idiopathic hypoadrenal-
ism. Symptoms include weakness, hypotension, anorexia, weight loss, and hyperpigmentation of the skin. Patients may have hyponatremia, hyperkalemia, and eosinophilia. Adults with craniopharyngioma present with headache, visual problems, papilledema, personality changes, and hypopitu-itarism. Sheehan syndrome is postpartum hemorrhage and necrosis of the pituitary gland. Empty sella syndrome occurs when CSF fills the sella space and flattens the pituitary gland, which continues to function normally. The disorder is seen in obese, hypertensive, multiparous women. Insulinoma causes hypoglycemia, which is often a feature of hypoadrenalism. Schmidt syndrome is the combination of Hashimoto thyroiditis with Addison's disease. Pituitary apoplexy occurs in <5% of patients with a pituitary macroadenoma (>1 cm); patients complain of headache, neck stiffness, fever, and visual disturbance and may present with acute adrenal insufficiency.
265. The answer is b. (Fauci, 14/e, pp 1883-1884, 2250.) Premature osteoporosis is not caused by menopause-induced estrogen deficiency and may be secondary to medication use (diphenylhydantoin, corticosteroids, heparin), hyperthyroidism, anorexia nervosa, malabsorptive disease, hyper-parathyroidism, multiple myeloma, immobilization, tobacco use, and alcoholism. Patients have a reduced bone mass with a normal mineral matrix. Osteomalacia is a disorder with reduced mineralization of the matrix; patients need to be evaluated for vitamin D deficiency. Scleromalacia is an inflammatory disorder often seen in patients with rheumatoid arthritis associated with chemosis and scleral-conjunctival inflammation. Patients with Paget's disease (increased bone turnover with the formation of disorganized bone) present with pain, enlarging skull bones (increasing hat size and hearing loss), skeletal deformities (bowing of the lower extremities), and increased warmth of the skin overlying the tibias.
266. The answer is b. (Seidel, 4/e, pp 789-790.) Over 50% of patients with diabetes mellitus develop neuropathy; loss of pressure, pain, and temperature sensation compounded by reduced blood flow may lead to amputations. Patients require frequent foot examinations, good foot hygiene, supportive footwear, and a screening test for loss of pressure sensation using a standard 10-g nylon filament. While the patient's eyes are closed, the monofilament is applied in a random pattern in several areas of the plantar surface for 1.5 s. Loss of sensation means the patient has no protective pain sensation to alert him or her to injury or skin breakdown.
267. The answer is b. (Fauci, 14/e, pp 292-294.) Hirsutism is growth of coarse, male-pattern hair in a woman. It is a sign of androgen excess and patients must be evaluated for ovarian or adrenal tumors. The typical workup for hirsutism includes a testosterone level, LH, follicle-stimulating hormone (FSH), 17-OH progesterone, and prolactin. The patient described most likely has 21-hydroxylase deficiency, which is the most common form of congenital adrenal hyperplasia. The highly elevated 17-OH progesterone concentration (which will be even higher after stimulation with synthetic ACTH) supports the diagnosis. Cushing syndrome seems unlikely in a patient without cushingoid features (central obesity). Idiopathic hirsutism applies to patients who have normal adrenal glands and ovaries. Seventy percent of patients with polycystic ovary disease (PCOD) present with hirsutism. They have elevated serum testosterone levels and elevated LH values. Patients with PCOD have slightly elevated levels of 17-OH progesterone after ACTH stimulation. Medications such as bodybuilding steroids, minoxidil, cyclosporine, oral contraceptives, and phenytoin can cause hirsutism.
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