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Old 09-08-2012
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A 32 yr Jewish woman presents to clinic complaining that her 'face has turned yelloe over the last 3 days' .She has no other concern at this time.She began taking oral comtraceptive two weeks ago. Her medical history is otherwise significant for acne vulgaris and dysmenorrhea. Lab findings are:

Liver studies:

Albumin 3.9g/dL
Direct bilirubin 4.5mg/dL
Alkaline phosphatase 3.1mg/dL
AST 32 U/L
ALT 30 U/L

Hepatitis panel:

HBsAg negative
Anti-HBsAg positive
Anti-HbcAg negative
Anti-HAV IgM and IgG negative
Anti-HCV negative

Liver biopsy is performed ,which shows dark granular pigment in the hepatocytes.Which of the following is the most likely diagnosis in this patient?

A.Rotor syndrome
B.Gilbert syndrome
C.Crigler-najjar syndorme
D.Dubin-Johnson syndrome
E.Liver steatosis secondary to oral contraceptive usage

Please answer with reasoning .Thank you
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Old 09-08-2012
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ans ..D........ the biopsy says it all ........

oc pills also slow down conjugated billirubin excretion so could have precipitated it ...
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Old 09-08-2012
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D.Dubin-Johnson syndrome as it is the only one which presents with conjugated hyperbilirubinemia in an adult.
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Dubin-Johnson syndrome because the liver its pigmented whereas in Rotor it isnt

I suffer from Gilbert syndrome btw (not that anyone cares just sharing)
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Old 09-11-2012
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Answer D

Dubin-Johnson and Rotor syndrome are two familial disorders of hepatic bile secretion that result in conjugated hyperbilirubinemia.A dark granular pigment is present in the hepatocytes of patient with Dubin-Johnson, but is not seen in association with Rotor Syndrome

Choice B. Glibert syndrome occurs in patient with no apparent liver disease who have mild unconjugated hyperbilirubinemia thought to be provoked by one of the classic triggers(eg fasting,stress,illness)

Choice E Crigler Najjar syndrome is divided ont type 1 nd 2,the former is more severe form that often results in permanent neurological impairment or death.Crigler-Najjar syndrome is very rare ,charchterized by significant unconjugated hyperbilirubinemia. Phototherapy or plasmapheresis are helpful in the short term, with liver transplant the only curative option.
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Old 09-11-2012
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D.Dubin-Johnson syndrome

black liver jaundice....because of accumulation of a melanin-like pigment in lysosomes

Last edited by cingulate.gyrus; 09-11-2012 at 04:48 AM. Reason: adding some thing
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