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Biliary atresia and pregnancy: puberty may be an important point for predicting the outcome
Tatsuo Kuroda1, Morihiro Saeki, Nobuyuki Morikawa
1Department of Surgery, National Center for Child Health and Development, Tokyo 157-8535, Japan. kuroda-t@ncchd.go.jp
Insights
Biliary atresia patients with good liver function at puberty can safely experience pregnancy. Post-puberty liver function, not Kasai operation timing, predicts long-term outcomes and pregnancy safety.
Area of Science:
- Hepatology
- Pediatric Surgery
- Reproductive Medicine
Background:
- Biliary atresia is a severe neonatal liver disease.
- Long-term outcomes and reproductive health in survivors are not well-defined.
Purpose of the Study:
- To investigate the late clinical features of biliary atresia survivors.
- To assess the safety and outcomes of pregnancy in women with a history of biliary atresia.
Main Methods:
- Retrospective analysis of 49 adult biliary atresia patients (age > 15 years).
- Review of clinical data, including liver function tests and pregnancy outcomes.
- Comparison of liver function between patients with different clinical courses.
Main Results:
- Six patients successfully delivered 7 newborns; 5 with native livers, 1 post-transplant.
- Newborns were generally healthy, though often small-for-date.
- Adult liver function at puberty, particularly enzyme levels, predicted later outcomes and pregnancy safety, independent of Kasai operation age.
Conclusions:
- Liver function during puberty is a key predictor of long-term health and pregnancy safety in biliary atresia survivors.
- Management strategies for these patients require reassessment after puberty.
- Pregnancy can be a safe outcome for carefully selected patients with well-managed biliary atresia.
Purpose:
The aim of this study was to study the late clinical features of biliary atresia in relation to pregnancy.
Materials And Methods:
Of the 175 patients with biliary atresia operated on at our department, the data for 49 patients (18 males, 31 females) older than 15 years were reviewed and analyzed.
Results:
Six patients delivered 7 newborns (5 with their native liver and 1 with a transplanted liver). Although one of the patients delivered twice after transplantation, another underwent transplantation after delivery. None of the 7 newborns showed any congenital anomalies; however, 6 were small-for-date newborns. Their birth weight tended to be inversely related to the mothers' age at delivery. An additional 13 patients required liver transplantation, of whom 1 died after transplantation and 1 died before transplantation. The remaining 30 patients showed uneventful courses without transplantation. The age at Kasai operation was significantly higher in the 5 patients who delivered babies with their native liver compared with that in these 30 patients (88.0 vs 68.6 days). A significant difference between these 5 patients and those who died or required transplantation before pregnancy was recognized even at the age of puberty in the alanine aminotransferase (36.5 +/- 26.5 vs 126.4 +/- 56.1 IU/L), aspartate aminotransferase (36.2 +/- 26.5 vs 114.2 +/- 48.9 IU/L), and choline esterase (249.7 +/- 64.8 vs 163.5 +/- 40.2 IU/L).
Conclusions:
The age at Kasai operation may not necessarily impact the late clinical course; however, liver function at puberty may predict the subsequent clinical course, including the safety of pregnancy. The management strategies should be revised after puberty.
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