[Constitutional hyperbilirubinemia and mixed bilirubin. Clinical and functional study]

Abstract

Three cases of non-haemolytic constitutional icterus with prevalently conjugated bilirubin have been studied: one case of Dubin-Johnson syndrome and two cases of Rotor's syndrome. Confirmation was obtained for the differences between the two syndromes from the physiopathological (by means of the BSF load test and determination of urinary excretion of porphyrin) and morphological (macroscopic, histological, ultrastructural) viewpoints. The following were carried out in all cases: a load curve with crystalline bilirubin, study of the course of the curve and application of compartmental analysis to the curve. Findings underlie the hypothesis that Rotor's syndrome contains an uptake defect and an accumulation change on the part of the liver cell of organic anions and that it is therefore possible to suggest some identity between Rotor's syndrome and the so-called "Hepatic storage disease".