Biliary atresia registry, 1976 to 1989

摘要: The Registry provides information about 904 children with biliary atresia from more than 100 institutions. There was a 1.4 to 1 female predominance; racial distribution 62% caucasian, 20% black, 11% Hispanic, 4.2% asian, and 1.5% American Indian. Eight hundred sixteen (90%) underwent corrective surgery (median age at operation, 69 days). Intraoperatively, 70% had totally obliterated extrahepatic bile ducts, 22% patency of the gallbladder distal common duct, whereas only 8% "correctable" (proximal duct patency). A variety reconstructions were used, but majority patients Roux-en-Y portoenterostomy or without exteriorization. Follow-up available for 670 (74%) average length follow-up 5 years (range, 16 years). Five-year actuarial survival 48% following Kasai's less 10% (at 3 years) if no operative correction done. Survival unaffected by sex, type reconstruction, cholangitis. Predictors bad outcome (1) caucasian race; (2) greater 60 days; (3) presence cirrhosis initial biopsy; (4) nonpatent ducts; (5) absent ducts level transection in liver hilus; (6) subsequent development varices ascites. Identification factors predictive ultimate provide basis either continued efforts management operation early referral transplantation.