USMLE题库Hepatic1-10-4-6.pptxVIP

Hepatic题库Q 1 Autopsy of a 21-yaer-old Caucasian shows extensive atrophy of basal ganglia and increased brain copper content. The main mechanism of excess copper removal in the healthy human body is:A.Glomerular filtration B.Renal tubular secretion C.Hepatic excretion into bile D.Intestinal epithelial accumulation E.Colonic active secretionA 1: Correct answer: CWilsons disease is an autosomal recessive disorder characterized by toxic accumulation of copper within organ tissues (especially the liver, brain, and eye).Normally, approximately 60% of ingested copper (0.6-3.0 mg/day) is absorbed in the stomach and duodenum, loosely bound to circulating albumin, and then transported to the liver. Within the liver, copper is incorporated into an a2-globulin to form ceruloplasmin. The ceruloplasmin is then resecreted into plasma, where it accounts for 90-95% of circulating copper. Normal total body copper is estimated at 50-150 mg. Senescent ceruloplasmin and the remainder of ingested, unabsorbed copper are secreted into bile and excreted in stool, which is the primary route for copper elimination. Renal losses represent 5-15% of daily copper excretion.(Choices A, D, and E) Glomerular filtration, intestinal epithelial accumulation, and colonic active secretion are not the main mechanisms of excess copper removal.(Choice B) Renal tubular secretion is the mechanism responsible for 5-15% of daily copper excretion. Educational Objective:Absorbed copper is used to form ceruloplasmin, which accounts for 90-95% of circulating copper. Senescent ceruloplasmin and the unabsorbed copper are secreted into bile and excreted in stool, which is the primary route for copper elimination.Q 2A 29-year-old Caucasian male experiences fever, fatigue, joint pain and urticaria-type skin rash. Once the symptoms start to abate a surge of serum alanine aminotransferase (ALT) and aspartate aminotransferase (AST) is noted. This patient most likely suffers from:Gilbert syndrome Acute viral hepatitis Acute