Pigmentary hepatosis. Clinical features, puncture biopsy, electronic introscopy, diagnosis, prognosis

Abstract

The term "pigmentary hepatosis” has become a collective concept, including various disorders of bilirubin metabolism. The main diseases are the syndromes Gilbert, Kriegler-Nayyar, Dabin-Johnson and Rotor. With Gilbert s syndrome, the sequence of the gene responsible for the formation of phosphate hydroge-nase has been violated, Kriegler-Nayyar syndrome - congenital chronic non-hemolytic jaundice with increased unbound bilirubin, Dabin-Johnson syndrome - a disease that is hereditary benign conjugated bilirubinemia due to impaired excretion of pigments in bile, Rotor syndrome is similar to the Dabin-Johnson syndrome, but the excretion defect is less pronounced. The article deals with morphology, clinical picture, main syndromes, differential diagnosis, prognosis and treatment regimens specific for each syndrome.