TY - JOUR
T1 - Biliary atresia, the next generation
T2 - A review of liver function, social activity, and sexual development in the late postoperative period
AU - Kuroda, Tatsuo
AU - Saeki, Morihiro
AU - Nakano, Miwako
AU - Morikawa, Nobuyuki
N1 - Copyright:
Copyright 2017 Elsevier B.V., All rights reserved.
PY - 2002/12/1
Y1 - 2002/12/1
N2 - 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 v159 ± 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 v3.9 ± 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.
AB - 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 v159 ± 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 v3.9 ± 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.
KW - Biliary atresia
KW - Fertility
KW - Liver function
KW - Menstrual disturbance
UR - http://www.scopus.com/inward/record.url?scp=0036895779&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0036895779&partnerID=8YFLogxK
U2 - 10.1053/jpsu.2002.36702
DO - 10.1053/jpsu.2002.36702
M3 - Article
C2 - 12483637
AN - SCOPUS:0036895779
VL - 37
SP - 1709
EP - 1712
JO - Journal of Pediatric Surgery
JF - Journal of Pediatric Surgery
SN - 0022-3468
IS - 12
ER -