А case of a fulminant hepatitis on the background of the paucity of interlobular bile ducts

Abstract

Paucity of interlobular bile ducts (PIBD) ranks second place after biliary atresia in the structure of cholestatic liver disease in children. Morphologically PIBD is characterized by reduction of interlobular bile ducts number in correlation with portal hepatic tracts (ductopenia). Ductopenia can be found only on morphological examination. Liver biopsy is the single most informative investigational tool then. There are two known types of the paucity of biliary ducts: syndromic type which is associated with other congenital defects and nonsyndromic type without other anomalies detected at birth. It may be classified into secondary and primary. Cytomegalovirus is the most frequent infectious etiology in case of a secondary nonsyndromic hypoplasia. Nonsyndromic hypoplasia is also considered to be an inherited autosomal recessive disease, as in many cases parents of ill children are relatives, and their siblings have the same disorder. The presented clinical case of nonsyndromic form of PIBD was no an exclusion. In the patient, this congenital anomaly had complicated the course of an acute hepatitis. PIBD, which was revealed during the pathomorphology postmortally, had played a leading role in the fulminant course of an acute hepatitis in the child, that was thought to be healthy previously.