Biliary atresia in Turkish children

AIM: To evaluate surgical success, survival rate and relationship of outcome parameters with time at diagnosis and operation of extrahepatic biliary atresia (EHBA) patients in Izmir, Turkey. METHODS: Clinical and laboratory data were reviewed from case reports of 27 EHBA patients. Twenty-five patients were operated on using Kasai procedure and two cases received liver transplants without portoenterostomy due to decompansated liver cirrhosis on diagnosis. Post operational success was defined as clearance of jaundice (bilirubine level <2 mg/dL). Patients were studied in two groups: I (jaundice free and/or compensated liver disease with liver transplant if needed after 3 years of age) and II (progressive liver disease with death or liver transplant if needed before 3 years of age). Kasai success, age at diagnosis and operation, survival and correlation of outcome with age, preoperative liver functions were evaluated. Eight patients received liver transplants. RESULTS: Median age at diagnosis was 63.5 days (21-212) and portoenterostomy was performed at median age of 67.5 days (25-220). Kasai operation was successful in two cases (8%) and the operation was performed at 35 and 42 days. Age at diagnosis (P = 0.13) and operation (P = 0.2) was not different between the two groups. Group I and II consisted of seven (28%) and 18 (72%) infants. Pre-existing ascites, serum alanine aminotransferase (ALT) and globulin levels were significantly higher in group II patients (P = 0.008, P = 0.04, P = 0.017, respectively). CONCLUSIONS: The results of the present study indicate that general practitioners should pay close attention to the evaluation of infants with prolonged jaundice in Turkey. Because of frequent late diagnosis presenting with cirrhosis at admission and also because of the low organ donation rates in Turkey, living related liver transplantation is an option and is currently undergoing detailed ethical consideration.