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A 3 yr-old white girl was referred to a pediatric gastroenterologist because of elevated levels of liver enzymes and total and direct bilirubin. The patient has a history of renal tubular acidosis and peripheral pulmonic stenosis. On examination, the patient was noted to have a broad forehead and deep-set, widely spaced eyes. Scratch marks were seen on the skin secondary to pruritus. A liver biopsy will most likely show:

a)   Bile duct proliferation

 b)   Giant cell transformation

 c)   Bile duct paucity

 d)   PAS-positive diastase-resistant globules

 e)   No changes
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c)   Bile duct paucity