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| Dubin-Johnson syndrome ： ウィキペディア英語版 |
Dubin–Johnson syndrome
Dubin–Johnson syndrome (DJS) is an autosomal recessive disorder that causes an increase of conjugated bilirubin in the serum without elevation of liver enzymes (ALT, AST). 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.
==Pathophysiology==
The conjugated hyperbilirubinemia is a result of defective endogenous and exogenous transfer of anionic conjugates from hepatocytes into the bile.〔Suzanne M Carter, MS 〕 Impaired biliary excretion of bilirubin glucuronides is due to a mutation in the canalicular multiple drug-resistance protein 2 (MRP2). A darkly pigmented liver is due to polymerized epinephrine metabolites, not bilirubin.
抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』
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