Hepatobiliary Disease USMLE Step 1 Practice Question
A 14-year-old boy presents with chronic hepatitis and progressive dyspnea. Vital signs show HR 92 bpm, RR 22/min, BP 118/76 mmHg, and SpO2 92% on room air. Laboratory studies reveal AST 156 U/L and ALT 142 U/L. Chest imaging demonstrates early emphysematous changes. Liver biopsy shows periodic acid-Schiff positive, diastase-resistant globules within hepatocytes. He denies recent infections or alcohol use. Which of the following is the most likely diagnosis?
Answer choices
- ACrigler-Najjar syndrome
- BWilson disease
- CPrimary sclerosing cholangitis
- DAlpha-1 antitrypsin deficiencyCorrect answer
- EHemochromatosis
- FCystic fibrosis
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