Genetic syndrome of mild unconjugated hyperbilirubinaemia, by definition <102 micromol/L (<6 mg/dL) (rarely exceeding 68 micromol/L [4 mg/dL]). The liver function is otherwise normal.
Common syndrome affecting 8% of the general population.
Decreased uridine-diphosphoglucuronate glucuronosyltransferase (UDPGT) activity leads to decreased conjugation of unconjugated bilirubin. In general, the bilirubin clearance is reduced by 60%.
Presentation usually asymptomatic, with mild icterus (jaundice) seen during times of fasting or physiological stress.
No treatment is needed since there is no liver injury or progression to end stage liver disease.
Although prognosis continues to be excellent, there is evidence that some patients with Gilbert syndrome may have abnormalities in the metabolism of selective protease inhibitor drugs and monoclonal antibodies used in the treatment of rheumatological and autoimmune diseases.
Gilbert syndrome is a mild, non-haemolytic, unconjugated hyperbilirubinaemia, defined by bilirubin levels of <102 micromol/L (<6 mg/dL). It is an inherited disorder in which decreased levels of the enzyme uridine-diphosphoglucuronate glucuronosyltransferase (UDPGT) result in impaired conjugation of bilirubin.
Assistant Professor of Medicine
Gastroenterology & Hepatology
University of Nebraska Medical Center
MAOM declares that he has no competing interests.
Dr Marco A. Olivera-Martinez would like to gratefully acknowledge Dr Brian Ward and Dr John L. Gollan, the previous contributors to this monograph. BW declares that he has no competing interests. JLG is an author of a study referenced in the monograph.
Professor of Pediatrics
Division of Endocrinology and Metabolism
Baylor College of Medicine
Texas Children's Hospital
LPK declares that she has no competing interests.
St. Mark's Hospital
JTJ declares that he has no competing interests.
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