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A. Falk, M.B. B.CH. B.A.O., M.B.B.Ch., Ph.D.

Assistant Professor, Johns Hopkins University School of Medicine

IgA nephropathy, or Berger disease, often presents in children as hematuria following infection. Lupus nephritis causes a nephrotic test Block 4 Full-length exams Answer C is incorrect. Ribavirin in combination with pegylated interferon-a2 is used in the treatment of hepatitis C. Lamivudine in com- Test Block 4 Answers 621 syndrome with proteinuria, hypoalbuminemia, edema, and hyperlipidemia; many patients also develop hypertension. Renal biopsy is important to determine treatment, and histologic findings are classified in five patterns, including mesangial and subendothelial deposits (called "wire-loop" lesions). Poststreptococcal glomerulonephritis is a common cause of nephritic syndrome that occurs about 10 days after pharyngitis. On light microscopy one would see diffuse proliferative glomerulonephritis without crescents. Renal amyloidosis is associated with chronic inflammatory diseases and causes nephrotic syndrome. Glomerular amyloid deposits can be seen on renal biopsy by staining with Congo red and examining the specimen under polarized light. The urogenital sinus in the male gives rise to the bladder, prostate, prostatic and membranous parts of the urethra and bulbourethral glands. The genital tubercle gives rise to the glans penis and corpus spongiosum in the male, or the glans clitoris in the female. In contrast, posterior urethral valves may be due to failure of regression of the urogenital sinus, which normally gives rise to the prostatic urethra. The mesonephric (wolffian) duct develops into the seminal vesicles, epididymis, ejaculatory duct, and vas deferens. The urogenital folds make up the ventral shaft of the penis and penile urethra in the male. This patient suffers from the autosomal recessive disorder alkaptonuria, a deficiency in homogentisic acid oxidase. As a result of the deficiency, there is an accumulation of alkapton bodies (homogentisic acid) in urine and cartilage. The lack of homogentisic oxidase blocks the metabolism of phenylalanine-tyrosine at the level of homogentisic acid. The homogentisic acid accumulates and a large amount is excreted, imparting a black color to the urine if allowed to stand and undergo oxidation. Affected patients are usually asymptomatic in childhood other than the change in urine color upon standing. In adulthood, the build-up of pigment in cartilage and its calcification can cause arthritic changes. By an unknown mechanism, the pigment causes the cartilage to lose its resiliency and become brittle and fibrillated. The arthropathy develops slowly and usually does not manifest until the patient is >30 years old. A deficiency of branched-chain a-ketoacid dehydrogenase would result in maple syrup urine disease, an inability to break down branched-chain amino acids. Refsum disease is a deficiency of phytanic acid oxidase and is characterized by an inability to break down branched-chain fatty acids. This deficiency results in albinism because patients are unable to make melanin, a derivative of tyrosine. This type of mutation is one of those found in the thalassemia picture, as described in this patient. In contrast, Mycoplasma pneumoniae infection typically yields a hemolytic anemia in which IgM antibodies are directed at the I (upper case) antigen. This disorder is a hypoproliferative anemia in which an appropriate reticulocytosis, as seen in this patient, is absent.

Most commonly, patients have alternating diarrhea and constipation, chronic abdominal pain that improves with stools, a change in stool frequency and consistency, and onset after emotional and/or stressful life events. Pseudomembranous colitis usually follows antibiotic therapy and is characterized by bloody diarrhea, fever, and leukocytosis. Primary sclerosing cholangitis is an extra-intestinal manifestation of ulcerative colitis. On colonoscopy, the mucosa demonstrates continuous superficial ulcerations with resultant inflammatory pseudopolyps. Crohn disease has a highly variable presentation; however, skip lesions, fissures, and strictures are generally evident on colonoscopy, endoscopy, and radiography. Celiac sprue is a disease of malabsorption characterized by bulky, fatty stools following meals. The patient has gallstones, as evidenced by her symptoms and ultrasound findings of multiple gallstones. Gemfibrozil, a fibrate, is contraindicated in the treatment of hypertriglyceridemia in the presence of gallstones. Fibrates can increase the development of gallstones, thus increasing the risk of cholecystitis. Ezetimibe, in combination with a statin, is an appropriate choice for treatment of this patient. Niacin would not be the first line of treatment for this patient, but it is not contraindicated. The most common tumor of the parotid gland is the pleomorphic adenoma or the mixed tumor, accounting for 50% of salivary tumors. The pleomorphic adenoma is a benign, well-differentiated, wellcircumscribed mass that grows slowly over the course of months to years. On histopathology, it is characterized by the presence of multiple cell types, classically epithelial cells in a chondromyxoid stroma. Mucoepidermoid carcinoma is an invasive, poorly differentiated cancer composed of mucosal and epidermal cell types. The laboratory values show marked leukocytosis, which is actually a common finding in acute diverticulitis. After the patient is stabilized and the acute diverticulitis has resolved, the patient should undergo colonoscopy to rule out malignancy. Colonoscopy is contraindicated during an acute episode of diverticulitis due to increased risk of bowel perforation. A sialic duct stone is an inorganic precipitate mechanically obstructing the opening of the sialic duct, resulting in an erythematous and inflamed oral mass. Sialic duct stones typically present with pain while eating due to the stone obstructing saliva from exiting the gland through the salivary duct. A wellcircumscribed mass of lymphoid cells in a salivary gland is virtually pathognomonic for a Warthin tumor, which make up 5-10% of salivary tumors. The upper third of the esophagus is made up of striated muscle (allows some voluntary control). The lower third of the esophagus is made up of smooth muscle (entirely involuntary). The wall of the esophagus becomes thin and atrophic and can have regions of fibrosis. It is helpful in the diagnosis of some genitourinary, gynecologic, and biliary pathologies as well as appendicitis. It may show the presence of diverticula and signs of adjacent inflammatory changes such as edema and fat stranding. However, it is not used to diagnose diverticulitis nor is it used to rule out colon cancer. The patient has parkinsonian symptoms due to the death of neurons in the basal ganglia (particularly in the putamen and globus pallidus). In addition, the liver biopsy shows evidence of cirrhosis (although this is a trichrome stain and not a copper stain, so the histopathology findings alone in this case are not specific). Copper accumulates in the liver, basal ganglia, bones, joints, kidneys, and Descemet membrane in the cornea (Kayser-Fleischer rings).

Treatment Treatment may include fluoroscopic or ultrasound guided, drainage, and any necessary stabilization of the spine. Prognosis is related to the clinical stage at which spinal cord compression is relieved. Symptoms and Signs the characteristic picture is segmental atrophy with areflexia and segmental loss of pain and temperature sensation with intact proprioception. Syringobulbia causes dysphagia, nystagmus, pharyngeal and palatal weakness, asymmetric weakness and atrophy of the tongue, and sensory loss in the distribution of the trigeminal nerve. Conservative treatment includes avoiding high-force isometric contractions and Valsalva expiration, head elevation at night, and maintenance of the neck in a neutral position. Surgery, including decompression and shunt placement, is recommended for neurologic deterioration or intractable central pain. Pain and paraparesis show the best response; sensory loss, lower motor neuron signs, and brainstem findings are less likely to improve. Syringomyelia resulting from an intramedullary spinal cord tumor is treated with tumor resection and radiation if complete excision is not possible. The cavity is usually irregular and disrupts the anterior horns of the gray matter and the gray matter ventral to the central canal. Arteriovenous fistulas have direct communications between arteries and veins without interposition of any pathologic network. Lesions can be further categorized by location into four groups: paraspinal, epidural, dural, and intradural. Symptoms and Signs the most common initial symptoms are radicular pain, sensory disturbance, leg weakness, and bladder dysfunction. Foix-Alajouanine syndrome is a slowly progressive myelopathy that develops because of venous thrombosis caused by venous stasis, and causes progressive ascending paralysis. Arteries originating from the vertebral artery and the aorta supply the spinal cord. A single anterior spinal artery supplies the anterior two thirds of the cord, and the paired posterior arteries supply the dorsal one third. The anterior spinal artery is discontinuous and therefore requires multiple feeders. The region from T4 to T8, which contains few anastomoses, is considered to be at the greatest ischemic risk, especially in patients with systemic hypotension. An area at the boundary zones between the territories of the anterior and posterior spinal arteries is also at risk for ischemia. This may result in an acute progressive syndrome of weakness and spasticity, with little sensory change resembling amyotrophic lateral sclerosis. Other causes include dissecting aortic aneurysm, hypotension, and surgical clipping of aortic aneurysms. There is a low risk of spinal cord infarction associated with transforaminal Posterior radicular artery Posterior spinal arteries Spinal artery B. Treatment options must be individualized for the patient and include embolization (endovascular technique), surgery to ligate the feeding vessel and excise the abnormality, or a combination of these techniques. A complete cure by embolization can usually be achieved in patients with paraspinal lesions. If there are multiple feeders or tortuous vascular anatomy, surgery is usually the best option. Treatment may also consist of a combination of endovascular ablation followed by surgical excision. After treatment, most patients with spinal arteriovenous shunts show neurologic improvement. Spinal cord ischemia: Clinical and imaging patterns, pathogenesis, and outcomes in 27 patients.

Syndromes

• Small: < 20 mg/dL
• Fecal occult blood test (FOBT) every year (colonoscopy is needed if results are positive)
• Rapid heart rate
• Bladder outlet obstruction
• Eat more fruits and vegetables.
• Another class of hormone therapy medicines is called aromatase inhibitors. Medicines such as exemestane (Aromasin) work as well or even better than tamoxifen in postmenopausal women with breast cancer. Aromatase inhibitors block estrogen from being made in the body.
• Inability to speak or move parts of his or her body (see stroke symptoms)
• To get to the lungs, blood must cross an atrial septal defect (ASD), ventricular septal defect (VSD), or a patent ductus artery (PDA). (These conditions are described above.) This condition severely restricts blood flow to the lungs.
• Convulsions
• Bleeding problems

The hypertension may occur in isolated bursts or chronically and may be associated with palpitations, Answer E is incorrect. The three classic findings associated with Graves disease are hyperthyroidism, ophthalmopathy, and dermopathy/pretibial myxedema. Although some cases of Hashimoto thyroiditis may present as a transient hyperthyroidism (with symptoms including palpitations and increased metabolic rate) from an initial disruption of thyroid follicles, the majority of cases present with signs and symptoms of hypothyroidism, such as intolerance to cold weather, weight gain, and mental and physical slowness. Plummer disease is characterized by a nodular goiter that has a hyperfunctioning nodule, causing hyperthyroidism. As opposed to Graves disease, Plummer disease is not accompanied by ophthalmopathy or dermopathy/pretibial myxedema. Iodine deficiency causes hypothyroidism, manifested with signs and symptoms that include intolerance to cold weather, weight gain, and mental and physical slowness. Papillary carcinoma of the thyroid, the most common form of thyroid cancer, usually presents as an asymptomatic thyroid nodule with signs of obstruction from the tumor such as hoarseness, cough, dysphagia, or dyspnea or a cervical lymph node mass (as opposed to symptoms of hyper- or hypothyroidism). This is coupled with increased mineralocorticoid (aldosterone) production due to the shunting of precursors, such as pregnenolone and progesterone, through mineralocorticoid pathways. The low sex steroid levels manifest clinically as a female phenotype with no sexual maturation. Remember the mnemonic "Salt, Sugar, and Sex" for the layers of the adrenal cortex and their respective products, with "salt" corresponding to the outer zona glomerulosa, "sugar" corresponding to the middle zona fasciculata, and "sex" corresponding to the inner zona reticularis. The symptoms are similar to those of 17a-hydroxylase deficiency, manifesting as hypertension and hypokalemia; however, virilization would also be present. The medulla produces catecholamines (epinephrine and norepinephrine); neither 17a-hydroxylase nor 21b-hydroxylase is required for the synthesis of catecholamines. Cortisol synthesis requires 21b-hydroxylase and 17a-hydroxylase and is therefore deficient in this patient. Synthesis of the sex hormones requires 17a-hydroxylase, but not 21b-hydroxylase, and is therefore deficient in this patient, leading to her primary amenorrhea. The symptoms are similar to those of 17ahydroxylase deficiency, manifesting as hypertension and hypokalemia; however, virilization would also be present. The stress caused by the viral upper respiratory infection likely increased her insulin requirements, precipitating fat breakdown and ketogenesis. Labs demonstrate hyperglycemia, increased ketone levels, leukocytosis, and a metabolic acidosis. On binding with its ligand, the receptor translocates from the cytoplasm into the cell nucleus, and the ligand-receptor complex acts as a transcription factor. Other hormones that act through nuclear steroid hormone receptors include cortisol, aldosterone, vitamin D, testosterone, estrogen, and progesterone. Histamine and vasopressin activate phospholipase C, resulting in the cleavage of phosphatidylinositol diphosphate into inositol triphosphate and diacylglycerol. Intracellular insulin acts via a tyrosine kinase cascade and not via nuclear hormone receptors. Portal systems consist of two capillary beds directly connected by veins; these keep hormones from being diluted before reaching the pituitary. The cells of the pituitary gland do not carry out any processing of hypothalamic hormones. This is a clear indication that the disorder is most likely autosomal dominant in inheritance pattern. Fabry disease is an Xlinked recessive disorder characterized by peripheral neuropathy, cardiovascular disease, and angiokeratomas. Familial dysautonomia is an autosomal recessive disorder in which individuals have a poorly developed autonomic nervous system, resulting in such manifestations as labile blood pressure, reduced production of tears, and an inappropriate response to stress. Mitochondrial inheritance is characterized by transmission of a trait to the offspring of an affected mother but never to the offspring of an affected father, because mitochondria are inherited from only the egg and not the sperm. This patient has symptoms of a glucagonoma, a rare glucagonsecreting tumor that can cause hyperglycemia, diarrhea, and weight loss. The hyperglycemia seen in these patients will not respond to oral hypoglycemic agents because of the uncontrolled excess glucagon production that continues despite increased insulin levels. Glucagonomas also are associated with necrolytic migratory erythema, a skin rash consisting of painful, pruritic erythematous papules that blister, erode, and crust over. Hormones are delivered from the anterior pituitary to the hypothalamus via the systemic circulation, just as they are delivered to other tissues.