11. An 18-year-old man is noted on physical examination to have marked bounding
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Question
11. An 18-year-old man is noted on physical examination to have marked bounding pulses in the upper extremities, but only weak pulses palpable in the lower extremities. Pulmonary function is normal. He does not have cyanosis. The most probable condition leading to these findings isa. Pulmonic stenosis
b. Tetralogy of Fallot
c. Coarctation of the aorta
d. Transposition of the great vessels
e. Patent ductus arteriosus
12. An 8-year-old boy was born with a large ventricular septal defect (VSD) that was never surgically corrected. Now, years later, he has increasing dyspnea along with hypoxia and cyanosis. The mechanism for the production of these symptoms is most likely to be
a. Natural closure of the VSD
b. Pulmonary hypertension
c. Acute myocardial infarction
d. Endocardial fibroelastosis
e. Left atrial thrombosis
13. A 45-year-old woman has had worsening shortness of breath for several years. She has to sleep sitting up on two pillows. She has difficulty swallowing. There is no history of chest pain. She is afebrile. Recently, she had a "stroke" with resultant inability to move her left leg and difficulty moving her left arm. A chest radiograph reveals a near-normal left ventricular size with a prominent left atrial border. Which of the following conditions is most likely to account for these findings
a. Essential hypertension
b. Cardiomyopathy
c. Mitral valve stenosis
d. Aortic coarctation
e. Left renal artery stenosis
14. A newborn presents to the pediatrician with kernicterus, jaundice, and elevation of serum unconjugated bilirubin; the patient dies soon after her first birthday. This patient most likely had a deficiency in which of the following enzymes?
a. Aldolase B
b. Galactose-1-phosphate uridyltransferase
c. Glucose 6-phosphatase
d. Uridine diphosphate-glucuronosyltransferase
e. Uroporphyrinogen decarboxylase
15. A 59-year-old man who was active all his life develops sudden severe anterior chest pain that radiates to his back. Within minutes, he is unconscious. He has a history of hypertension, but a recent treadmill test had revealed no evidence for cardiac disease. Which of the following do you suspect
a. Tear in the aortic intima
b. Group A streptococcal infection
c. Right middle cerebral artery embolus
d. Acute viral myocarditis
e. Pulmonary embolus
Explanation / Answer
11. (C) "A post-ductal (adult type) coarctation of the aorta results in increased flow to upper extremities supplied from the great vessels prior to the coarctation." 12. (B) "He has an Eisenmenger complex in which a longstanding left-to-right shunt leads to pulmonary hypertension and a reversal to a right-to-left shunt." 13. (C) "Mitral valve stenosis leads to left atrial enlargement, but the left ventricle is usually small. THere is typically a 'fishmouth' shaped mitral valve that has stenosis as well as insufficiency, since it does not close completely. Most mitral valvular disease in adults results from rheumatic valvulitis. The episode(s) of rheumatic fever occurred years before." 14. (D) . "This constellation of symptoms is suggestive of Crigler-Najjar syndrome type I, a hereditary hyperbilirubinemia that is fatal within 18 months of life secondary to kernicterus (bilirubin deposition in brain tissue). Multiple genetic defects in the allele for the bilirubin uridine diphosphate-glucuronosyltransferase (UGT1) may give rise to this disorder. This enzyme is responsible for conjugating bilirubin with one or two molecules of glucuronic acid. The liver is morphologically normal by light and electron microscopy" 15. (A) "The history is typical for an aortic dissection, which starts as an intimal tear. Many patients are hypertensive." Helpful Links/Sources: http://www.docstoc.com/docs/23409972/first-aid-Q-and-A-step-1_09_-_-part-2_ http://library.tcmedc.org/webpath/exam/multorg/examidx.htm Hope this helps :)
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