Das Gilbert-Syndrom ist eine indirekte Hyperbilirubinämie ohne Krankheitswert. Sie wird auf Störungen des vom UGT1A-Gen kodierten Enzyms zurückgeführt. Die Vererbung kann sowohl dominant als auch rezessiv sein.
Mit Phenobarbital kann eine Enzyminduktion und damit eine Verbesserung der Bilirubinwerte erzielt werden.
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None (1962) Chronic unconjugated hyperbilirubinemia without overt signs of hemolysis in adolescents and adults. ![]() |
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None (1967) Nonhemolytic unconjugated hyperbilirubinemia with hepatic glucuronyl transferase deficiency: a genetic study in four generations. ![]() |
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Koiwai O et al. (1995) Gilbert's syndrome is caused by a heterozygous missense mutation in the gene for bilirubin UDP-glucuronosyltransferase. ![]() |
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Bosma PJ et al. (1995) The genetic basis of the reduced expression of bilirubin UDP-glucuronosyltransferase 1 in Gilbert's syndrome. ![]() |
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None (2008) Pharmacogenetics of Gilbert's syndrome. ![]() |
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Hsieh TY et al. (2007) Molecular pathogenesis of Gilbert's syndrome: decreased TATA-binding protein binding affinity of UGT1A1 gene promoter. ![]() |
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Orphanet article Orphanet ID 2824![]() |
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OMIM.ORG article Omim 143500![]() |
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Wikipedia Artikel Wikipedia DE (Morbus_Meulengracht)![]() |