Biliary atresia, the next generation: a review of liver function, social activity, and sexual development in the late postoperative period

Purpose: The current study aimed to establish the management for biliary atresia (BA) patients in the late postoperative period. Methods: Of 165 BA patients operated on in the authors' department, 44 patients (16 boys, 28 girls) with a follow-up period of more than 15 years were reviewed retrospectively. Results: Forty-one of 44 patients (93.2%) currently are employed or highly educated, 7 are married, whereas 2 (4.5%) died, and 10 (22.7%) required liver transplantation after puberty. Four babies have been born from BA parents without congenital anomalies. Four girls conceived 5 times and delivered 3 newborns weighing 2,330 to 2,474 g including one delivered after transplantation. Maternal portal hypertension uniformly deteriorated during pregnancy, and one pregnancy was terminated. Menstrual disorder correlated significantly with the biochemical data related to liver function at puberty such as serum choline esterase (266 ± 70.4 in 19 normal patients v 159 ± 34.3 IU/L in 9 abnormal patients, P =.00057), asparate aminotransferase (42 ± 30.8 v 96.0 ± 63.6 IU/L; P =.0031), and serum albumin (4.6 ± 0.4 v 3.9 ± 0.6 g/dL; P =.013). Conclusions: The long-term survivors of Kasai's operation, with or without liver transplantation, have reached the next generation. Transgenerational follow-up and management including conception and perinatal care should be required for BA patients. J Pediatr Surg 37:1709-1712. Copyright 2002, Elsevier Science (USA). All rights reserved.