Hereditary defects of bilirubin metabolism

Last updated on: 18.12.2020

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DefinitionThis section has been translated automatically.

Familial hepatocellular disorder of bilirubin metabolism caused by a microsomal (bilirubin conjugation disorder - Crigler-Najjar syndrome type I and type II) or postmicrosomal preterminal disorder (isolated secretion disorder of conjugated bilirubin - Dubin-Johnson syndrome, Rotor syndrome).

ClassificationThis section has been translated automatically.

With elevated unconjugated (indirect) bilirubin

Crigler-Najjar syndrome type I
Crigler-Najjar syndrome type II (Arias syndrome)
Gilbert-Meulengracht syndrome

With elevated conjugated (direct) bilirubin

Dubin-Johnson syndrome (E 80.6)
Rotor syndrome (E 80.6)
Idiopathic recurrent cholestasis (Summerskill-Tygstrup) E83.1

Last updated on: 18.12.2020