1077)(C) Hirschsprung disease is a genetic disordercaused by the absence of enteric nerve cells in the wall of the sigmoid colon and/or rectum.The portion of the bowel wall without nerve ganglia (aganglionic) cannot relax in responseto bowel content so that the stool builds up behind the obstruction. In some children the problems begin shortly after birth, other infants are not acutely ill, but develop chronicsymptoms such as constipation or anemia.
Cholecystitis (choice A), caused by inflammation of the gallbladder, gastroesophageal refluxdisease GERD (choice B), and polymyositis(choice D), a disorder affecting esophageal skeletal muscle, do not affect the neuronal regulation of the large intestine. Hirschsprung disease is almost always treated by surgical removal of the affected bowel segment and thenjoining the healthy bowel segments (choice E).A GI motility disorder might improve on its own due to the ability of the enteric nervoussystem in healthy GI tract portions to learn new motility patterns. However, it takes a very longtime and the success is not certain.

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1078 ) An 89-year-old man develops a persistent cough with increasing dyspnea. He worked in the shipyards during his 20s, is a lifelong smoker, and has a positive purified protein derivative (PPD) skin test. During the physical examination, a pleural effusion is detected and subsequently drained. Laboratory analysis reveals a specific gravity of 1.013, protein of 1.4 g/dL, and a small number of macrophages. Based upon these laboratory values, which of the following is the most likely diagnosis for this patient?

1078 ). (A) Pleural effusion that has a specific gravity less than 1.012 and a protein concentration of less than 1.5 g/dL is classified as a transudateand is associated with circulatory problems (increased hydrostatic pressure, decreased oncotic pressure, lymphatic obstruction).Exudates usually have a specific gravity >1.020 and protein concentration higher than 3 g/dL and are associated with inflammatory or neoplastic processes. Based upon the laboratory results, the patient has a transudate which rules out infection (choices C and D) and neoplasms (choices E and F). The persistent cough and dyspnea indicate that the fluid is accumulating in his lungs, which occurs in left-sided heartfailure. A pure right-side heart failure would beexpected to produce a systemic rather than pulmonary edema.


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1079 ) An 18-year-old woman presented with 1 week of history of fever and malaise. She had mild
jaundice and elevated temperature. Hemoglobin was 13.8 g/dL, leukocyte count 13 × 109 per liter.Serum bilirubin was elevated (42 mmol/L) and
contained 95% unconjugated bilirubin. Liver enzyme tests were normal. Which of the following is the most likely cause of these signs and symptoms?

# dr.Usmle

1079 ) . (B) Glucuronyl transferase is the enzyme that conjugates bilirubin in the liver, after which it is excreted in bile or urine. A hereditary defect. in glucuronyl transferase concentration, or activity, is called Gilbert syndrome. It may lead to mild jaundice and general discomfort with typical onset in childhood or early adulthood.Alcohol poisoning (choice A) leads to liver damage, and an elevation of conjugated bilirubin. Abnormalities of liver enzyme tests would be expected. Lactate dehydrogenase (choice C) catalyzes the conversion of lactate to pyruvate as part of cellular energy production. Since many cells including red blood cells are rich in LDH, increased serum LDH levels could point toward excessive hemolysis, but would not be a cause for it. Although hemolysis that exceeds the capacity of the liver to clear bilirubin from serum (choice D) would lead to increased unconjugated bilirubin, it is not the best choice due to the woman’s normal hemoglobin (12–16g/dL for females). Obstruction of bile flow (choice E) leads to backup of largely conjugated bilirubin in the blood stream.


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1080 ) During the process of protein synthesis, the factor eEF-2 induces the hydrolysis of GTP. The energy of this hydrolysis is coupled to which of
the following?

# dr.Usmle

1080 ) (E) The translation factor, eEF-2, is involved in the process of peptide elongation. Specifically, eEF-2 catalyzes the GTP-dependent translocation of the ribosomes along the mRNA to the next codon. Attachment of an amino acid to a tRNA (choice A) is catalyzed by the family ofaminoacyl-tRNA synthetases. Alignment of the mRNA on the 40S ribosome (choice B) is facilitated by the initiation factor eIF-1. Formation of the 80S initiation complex (choice C) occurs after complete assembly of the 40S preinitiation complex and requires the activity of eIF-5. Formation of the peptide bond (choice D) occurs through the action of peptidyl transferase.


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