Document Detail


Gilbert-Meulengracht's syndrome and pharmacogenetics: is jaundice just the tip of the iceberg?
MedLine Citation:
PMID:  20070246     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Gilbert's syndrome is characterized by mild unconjugated nonhemolytic hyperbilirubinemia, without hepatic inflammation, fibrosis, chronic liver disease, or liver failure. It is readily diagnosed by genetic variants of the UGT1A1 gene, mainly UGT1A1*28, and is also associated with abnormalities of hepatobiliary transport and additional UGT1A gene variants. Apart from representing a potential risk factor in irinotecan and protease inhibitor therapy, it appears to exert protective effects in Hodgkin's lymphoma and cardiovascular disease. Gilbert's syndrome is part of a continuous spectrum of altered glucuronidation that extends to fatal Crigler-najjar disease. The complexity hidden behind this pharmacogenetic abnormality is of profound significance for drug development and therapy.
Authors:
Christian P Strassburg
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Drug metabolism reviews     Volume:  42     ISSN:  1097-9883     ISO Abbreviation:  Drug Metab. Rev.     Publication Date:  2010 Feb 
Date Detail:
Created Date:  2010-01-14     Completed Date:  2010-05-03     Revised Date:  2011-01-19    
Medline Journal Info:
Nlm Unique ID:  0322067     Medline TA:  Drug Metab Rev     Country:  England    
Other Details:
Languages:  eng     Pagination:  168-81     Citation Subset:  IM    
Affiliation:
Department of Gastroenterology, Hepatology, and Endocrinology, Hannover Medical School, Hannover, Germany. strassburg.christian@mh-hannover.de
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MeSH Terms
Descriptor/Qualifier:
Crigler-Najjar Syndrome / genetics*
Gene Frequency
Genetic Predisposition to Disease
Gilbert Disease / genetics*
Glucuronosyltransferase / genetics*,  metabolism
Hyperbilirubinemia / epidemiology
Jaundice / complications,  genetics*
Pharmacogenetics*
Polymorphism, Single Nucleotide
Risk Factors
Chemical
Reg. No./Substance:
EC 2.4.1.-/bilirubin uridine-diphosphoglucuronosyl transferase 1A1; EC 2.4.1.17/Glucuronosyltransferase

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