Study of the etiology and pathogenesis of low grade nonhemolytic unconjugated hyperbilirubinemia (Gilbert's disease).

172 adolescent and adult patients with low grade nonhemolytic unconjugated hyperbilirubinaemia (n.u.h) were examined. Authors have come to the conclusion of the absence of an acquired (posthepatic) form of n.u.h., i.e of the existence of a single --constitutional--form (Gilbert's disease). Viral hepatitis is not likely to play the role of an etiological factor of n.u.h. but of a factor which manifests a congenital defect of bilirubin metabolism. The study of the glucuronidisation found out that its decrease is an important factor in the pathogenesis of the low grade n.u.h. that therefore cannot opposed to the n.u.h. with glucuronyl transferase deficiency group II according to Arias et al. In 61% of patients a moderately shortened erythrocyte life span has been revealed. However the increased bile pigment production in these cases does not speak against Gilbert's disease. The results of biochemical assays have shown that in n.u.h not only the intrahepatic bilirubin metabolism is disturbed but other functions of the liver cell as well. The morphological study of hepatocytes has revealed certain signs of their dystrophy. Based on their investigation the authors propose to single out a group of hereditary pigment hepatoses which include besides Gilbert's disease the syndromes of Crigler-Najjar, Dubin-Johnson and Rotor.