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Turkish Journal of Gastroenterology, vol.9, no.2, pp.198-200, 1998 (SCI-Expanded)
Paucity of interlobular bile ducts, which is defined as a reduction in the number of interlobular bile ducts, is a significant abnormality causing intrahepatic cholestasis in children. The nonsyndromic form which is seen rarely, may be idiopathic or associated with various specific clinical conditions. The syndromic form usually shows clinical improvement, while development of cirrhosis is common in the nonsyndromic form. We describe two children with nonsyndromic paucity of interlobular bile ducts diagnosed in the neonatal period, in whom clinical and biochemical improvement occurred in about six months.