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Dubin-Johnson syndrome

Summary of Dubin-Johnson syndrome
Dubin–Johnson syndrome is a rare, autosomal recessive, benign disorder that causes an isolated increase of conjugated bilirubin in the serum. Classically, the condition causes a black liver due to the deposition of a pigment similar to melanin. This condition is associated with a defect in the ability of hepatocytes to secrete conjugated bilirubin into the bile, and is similar to Rotor syndrome. It is usually asymptomatic, but may be diagnosed in early infancy based on laboratory tests. No treatment is usually needed.

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Gastrointestinal system

Pathology

Peritoneum and peritoneal cavity
Upper gastrointestinal tract disorders
Lower gastrointestinal tract disorders
Liver, gallbladder and pancreas disorders
Gastrointestinal system pathology review

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Dubin-Johnson syndrome

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High Yield Notes
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Dubin-Johnson syndrome

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USMLE® Step 1 style questions USMLE

2 questions

USMLE® Step 2 style questions USMLE

1 questions
Preview

A 5-month-old infant comes for an office visit. Laboratory results show congenital hyperbilirubinemia. AST and ALT concentrations are normal, and his coproporphyrin I to coproporphyrin III ratio is greatly increased. A liver biopsy shows a dark green macroscopic specimen. Which of the following is the most likely diagnosis? 

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