[Congenital extrahepatic biliary atresia as a cause of cholestasis in newborns and infants]

Anna Liberek, Magdalena Góra-Gebka, Wanda Bako, Grazyna Sikorska-Wiśniewska, Agnieszka Szlagatys-Sidorkiewicz, Marek Królak, Andrzej Marek, Agnieszka Jankowska, Maria Korzon, Piotr Czauderna
Medycyna Wieku Rozwojowego 2006, 10 (2): 395-406

UNLABELLED: Congenital extrahepatic biliary atresia (CEBA) is one of the most common causes of cholestasis in newborns and infants.

THE AIM OF THE STUDY: was the analysis of clinical presentation, results of laboratory and imaging investigations as well as clinical outcome of children with extrahepatic cholestasis caused by CEBA.

MATERIALS AND METHODS: the analyzed group included 15 children aged from 2 weeks to 4 months. Data concerning: pregnancy and delivery, newborn's health condition, faeces color; jaundice onset, manifestation of coagulation disorders coexisting malformations and disorders of other systems were obtained. The following investigations were performed: biochemical tests evaluating the function of the liver and cholestasis (serum bilirubin concentration and fractions, bile acids in serum, AlAT, AspAT, GGTP, FALK activities, serum electrophoresis, prothrombin index). We also performed tests focusing on hepatotropic infections, - metabolic disorders tests and in all children - ultrasound of the abdomen, scintigraphy of the bile ducts - HEPIDA. 14 children had undergone hepatoportoenterostomy, modo Kasai.

RESULTS: jaundice, acholic stools and hepatomegaly were present in all children. The serum concentration of bilirubin and its conjugated fraction and bile acids in all children were increased. GGTP and FALK activities were markedly elevated in all children. Aminotransferases activities elevations were observed, more distinctively for AST. Active cytomagalovirus infection was detected in 3 children. Abdominal ultrasound revealed gallbladder in 7 children, whereas intrahepatic bile ducts were described as normal in 12 cases. In all cases the HEPIDA scintigraphy showed no passage of the tracer to the GI tract even after 24 hours delay. Hepatoportoenterostomy was performed in 14 children, 5 of them had liver transplantation.

CONCLUSIONS: 1. There is still not one effective and specific diagnostic method in differentiating between the causes of cholestasis in the newborns and infants. Thus many investigation methods should be run simultaneously. 2. Congenital atresia must be definitely excluded before cholestasis with other background is finally diagnosed. 3. The hepatoportoenterostomy should be considered as the first line treatment in children with CEBA. Most cases will need liver transplantation in the future.

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