Fine Structure of the Liver of Constitutional Hyperbilirubinemia

Abstract

Constitutional hepatic dysfunction showing an increase in the free form of the plasma bilirubin, was first reported by Gilbert1) under the term of cholemie simple familiale in 1901. Similarly in 1948, Rotor2) reported the other type of constitutional hepatic disorder which showed mainly an increase in the conjugated form of the plasma bilirubin. However, histological examination of the liver in these two types of diseases has been reported to be within normal limits. In 1954, Dubin and Johnson3) described another type of the disorder under the term of chronic idiopathic jaundice with unidentified pigment in the liver which showed characteristic pigment in the liver cells and an increase in the conjugated form of the plasma bilirubin. These three disorders have many problems yet to be dissolved. In Rotor syndrome, despite of quite similarities to Dubin-Johnson syndrome in clinical and laboratory findings, there has been no report in which the confirmation of the pigment in the liver was made. These made us to study the two conditions with an use of electronmicroscope to find whether they are the same or different in their etiology and to delineate the particular pigmentation in the liver of Dubin-Johnson syndrome, as to its origin or nature and difference from ordinary brown colored pigmentation in the liver seeing in cases of cirrhosis, hepatitis or even in normal cases. In addition, we studied the cases of Gilbert's disease under the same circumstances.