Biliary atresia, the next generation: a review of liver function, social activity, and sexual development in the late postoperative period.
OBJECTIVE
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.
Kuroda
Tatsuo
T
Department of Surgery, National Center for Child Health and Development, Tokyo, Japan.
Saeki
Morihiro
M
Nakano
Miwako
M
Morikawa
Nobuyuki
N
D000293:Adolescent; D001656:Biliary Atresia / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008111:Liver Function Tests / Q000706:statistics & numerical data*; D016031:Liver Transplantation / Q000706:statistics & numerical data; D008297:Male; D008599:Menstruation Disturbances / Q000453:epidemiology*; D011171:Portoenterostomy, Hepatic / Q000706:statistics & numerical data*; D011183:Postoperative Complications / Q000453:epidemiology*; D011247:Pregnancy; D011256:Pregnancy Outcome / Q000453:epidemiology; D011295:Prenatal Care; D011606:Psychosexual Development; D012189:Retrospective Studies; D012951:Socialization; D016896:Treatment Outcome