Syndromes of Constitutional Hyperbilirubinemia

Abstract

The diagnosis of intermittent mild chronic jaundice of obscure origin may be difficult in the patient who has nonspecific complaints in the absence of clinical, laboratory or histologic evidence of progressive liver disease. The Gilbert, Dubin-Johnson, Rotor and Crigler-Najjar syndromes are the principal entities found in constitutional nonhemolytic hyperbilirubinemia and should be considered in the differential diagnosis. The typical clinical picture, laboratory and accessory findings, histologic features of liver biopsy, cause, therapy and prognosis of each syndrome are discussed.