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Abstract

Objectives: Biliary atresia is a chronic cholestatic disease resulting from irreversible extrahepatic bile duct occlusions, which leads to liver transplantation in childhood. Solid prognostic values are lacking.

Methods: We investigated the prognostic validity of the PELD score and other laboratory parameters that concern the transplantation-free survival of children with biliary atresia. We correlated the degree of liver fibrosis and neoproliferation of bile ducts at the time of Kasai hepatic portoenterostomy (KPE) and liver transplantation with serological and clinical parameters. Liver biopsies were obtained at the time of KPE in 26 children and directly before the liver transplantation (LTx) of 22 children. The ISHAK score was used to calculate liver fibrosis. Neo-proliferation of the biliary tract was shown by expression of cytokeratin 7.

Results:The survival rate with their own liver was significantly high in children who underwent KPE in less than 28 days of age (p<0.01, cut off 30 days, sensitivity 95.5%, specificity 100%, negative predictive value 80%). Patients with liver enzyme values AST < 83 U/l before surgery also pointed to a significantly better result than patients with higher values (p<0.01, sensitivity 100%, specificity 100%). The bilirubin clearance in children in the group without LTx three months after KPE was significantly better (0.6 (0.1-1.2) vs. 10.18 (0.3-21.9) mg/dl, p<0.05). PELD score and histology had no predictive value.

Conclusions: This study confirms that histological findings do not correlate with serological markers at KPE. However, age and serological markers at the time of KPE appear to be a predictive factor for a positive outcome.

Keywords: ISHAK score, Liver fibrosis, Prognostic markers, Pediatric liver transplantation, PELD score