Paucity of intrahepatic bile ducts in pediatric practice

Abstract

Neonatal cholestasis is the main cause of hospitalization in a pediatric hepatology department and represents the main indication for liver transplantation in children. Besides the biliary atresia, infectious, metabolic or toxic causes, one of the important causes of neonatal cholestasis is the paucity of intrahepatic biliary ducts. Alagille syndrome represents the association of this paucity of the biliary ducts with cardiac, vascular, renal, skeletal, ocular manifestations and characteristic facies. Alagille syndrome raises important issues of diagnosis, treatment and prognosis, which makes these aspects necessary to be known by pediatricians. The differential diagnosis from biliary atresia or other causes of biliary ducts paucity is important for the management of a patient with cholestasis. In the last period, besides the clinical diagnosis, there is an increasing importance of the genetic confirmation of Alagille syndrome in a patient with cholestasis, important for the prognosis of the evolution. Liver transplantation may be an effective solution for patients with Alagille syndrome, but the particularities of post-transplant evolution should be considered.