Also known as Meulengracht disease
A benign, inherited syndrome in which mild, intermittent unconjugated hyperbilirubinemia occurs in the absence of hemolysis or liver dysfunction
Pediatric Considerations
Rare for the disorder to be diagnosed before puberty
Pregnancy Considerations
The relative fasting that may occur with morning sickness can elevate bilirubin level.
Prevalence
The reported prevalence of Gilbert syndrome (GS) is between 5–10% in different populations, with most presentations during or after adolescence (1).
Indirect hyperbilirubinemia in GS results from impaired hepatic bilirubin clearance (<30% of normal) due to decreased levels of the enzyme uridine diphosphoglucuronate-glucuronosyltransferase (UDPGT). Hepatic bilirubin conjugation (glucuronidation) is thus reduced, although this may not be the only defect.
Genetics
GS is part of a spectrum of hereditary disorders that includes types I and II Crigler-Najjar syndrome. However, bilirubin levels in these cases will be >6 mg/dL. There is an associated increased risk of cholelithiasis in certain populations. A neonatal presentation may lead to breast milk jaundice in the 2nd week of life.
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