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  #1  
Old 10-08-2012
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Arrow Billy Step 1 Questions # 16

A 17-year-old woman, G2, P1, gives birth to a term infant after an uncomplicated pregnancy. The infant does well for 3
weeks, but then begins to have abdominal enlargement, light-colored stools, and dark urine. On physical examination, the
infant is icteric. There is hepatomegaly, but no splenomegaly or lymphadenopathy. Laboratory studies show serum AST of
101 U/L, ALT of 112 U/L, glucose of 81 mg/dL, and creatinine of 0.4 mg/dL. A liver biopsy is done, and the biopsy
specimen shows lobular disarray with focal hepatocyte necrosis, giant cell transformation, cholestasis, portal mononuclear
cell infiltrates, Kupffer cell hyperplasia, and extramedullary hematopoiesis. The infant recovers in 1 month, and by the age
of 1 year, all laboratory findings are normal. What is the most likely diagnosis?


□ (A) Erythroblastosis fetalis
□ (B) Extrahepatic biliary atresia
□ (C) Galactosemia
□ (D) Idiopathic neonatal hepatitis
□ (E) Primary biliary cirrhosis
□ (F) Von Gierke disease
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  #2  
Old 10-08-2012
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My best guess is idiopathic neonatal hepatitis !!
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Old 10-08-2012
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Default DD

......................??DD
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Old 10-08-2012
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Might be D as other condition need an intervention
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Old 10-08-2012
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My guess would be D)
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Old 10-08-2012
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waiting for answer.
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Old 10-08-2012
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□ (D) Idiopathic neonatal hepatitis
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Old 10-08-2012
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Default my answer :)

(D) Idiopathic neonatal hepatitis
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Old 10-09-2012
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Correct Answer Yes D is correct

Neonatal hepatitis is most often idiopathic, and most infants recover without specific therapy. Some cases are
caused by α1-antitrypsin deficiency, and some are due to extrahepatic biliary atresia. Patients with extrahepatic biliary
atresia require surgery to anastomose extrahepatic ducts and prevent progressive liver damage. Patients with
erythroblastosis fetalis have hydrops and icterus at birth because of maternal IgG antibody directed at fetal RBCs, leading
to hemolysis. Galactosemia is an inborn error of metabolism in which deficiency of galactose 1-phosphate
uridylyltransferase damages cells of the kidney, liver, and brain; there is hepatomegaly, splenomegaly, hypoglycemia, and
eventual cirrhosis. Primary biliary cirrhosis affects adults. Von Gierke disease results from deficiency of glucose-6-
phosphatase, and affected infants develop hypoglycemia, lactic acidosis, hyperuricemia, and hyperlipidemia
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Old 10-09-2012
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idiopathic neonatal hepatitis coz infant recovers by its own
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