Long-Term Follow-Up Study of Patients with Cholangitis after Successful Kasai Operation in Biliary Atresia

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Abstract
Since the introduction of cyclosporine A, liver transplantation has become accepted as the therapy for end-stage liver disease. However, there are no definite criteria for liver replacement in biliary atresia. We investigated (a) the survival rate after hepatic portoenterostomy (n = 131), (b) liver function tests in fatal cases after an initially successful hepatic portoenterostomy (n = 9), and (c) liver function tests in the patients with episodes of cholangitis after a successful surgical treatment (n = 8). Patients with persisting jaundice after the surgery cannot be expected to survive long, and therefore they definitely should undergo liver transplantation. When total bilirubin concentration was above 10 mg/dl in patients with cholangitis after a successful operation, conservative therapy had almost no effect. Therefore, patients with total bilirubin levels above 10 mg/dl should be considered for liver transplantation. Of the liver function tests, only total bilirubin was reliable as a marker for hepatic failure in the end stage of biliary atresia. Prolongation of thrombo test and episodes of gastrointestinal bleeding also were used in selection for patients of liver replacement.

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