Organ System Pathology USMLE Step 1 Practice Question
A 14-year-old boy with progressive hepatomegaly and jaundice presents with chronic hepatitis. Vital signs: BP 118/76, HR 88, RR 16, Temp 37°C, SpO2 98%. Liver biopsy shows PAS-positive, diastase-resistant globules within hepatocytes. Laboratory studies reveal elevated transaminases (AST 156 U/L). He denies alcohol use and has no history of viral hepatitis exposure. Notably, he also has early-onset emphysema. Which mechanism most likely accounts for his liver pathology?
Answer choices
- ADefective peroxisomal beta oxidation
- BFailure of ubiquitin conjugation
- CMisfolded protein retention in the endoplasmic reticulumCorrect answer
- DLysosomal glycogen accumulation
- EMicrotubule transport defect
- FCopper accumulation from ATP7B transporter dysfunction
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