doiSerbia

Hereditary hyperbilirubinemias

Radlović Nedeljko (Faculty of Medicine, Belgrade + University Children’s Hospital, Belgrade)

Inherited disorders of bilirubin metabolism involve four autosomal recessive syndromes: Gilbert, Crigler- Najjar, Dubin-Johnson and Rotor, among which the first two are characterized by unconjugated and the second two by conjugated hyperbilirubinemia. Gilbert syndrome occurs in 2%-10% of general population, while others are rare. Except for Crigler-Najjar syndrome, hereditary hyperbilirubinemias belong to benign disorders and thus no treatment is required.

Keywords: hereditary hyperbilirubinemias, UGT1A1 gene, uridine diphosphate glucuronosyltransferase 1A1, MRP2 gene