Dubin-Johnson Syndrome

Dubin-Johnson syndrome is an autosomal recessive disease which presents shortly after birth with an increase of conjugated bilirubin without elevation of liver enzymes (ALT, AST).
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In Dubin-Johnson(autosomal recessive) and Rotor(possibly autosomal dominant) syndromes there are defects in bilirubin handling in the liver.there is conjugated hyperbilirubinaemia. prognosis is good in both. In the Dubin -Johson syndrome a mutation in the cMOAT tranpsorter gene has recently been discribed. The liver is black owing to melanin deposition.