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[Predictive parameters in children with biliary atresia]
J Leonhardt1, J F Kuebler, C Turowski
1Klinik für Kinderchirurgie, Medizinische Hochschule, Hannover, Germany. Johannes_leonhardt@yahoo.de
Insights
Biliary atresia (BA) patients often need liver transplants. This study found few predictive factors for long-term survival with native livers, suggesting primary transplantation may be premature.
Area of Science:
- Pediatric Hepatology
- Gastroenterology
- Transplantation Immunology
Context:
- Biliary atresia (BA) is a severe neonatal liver disease.
- Most BA patients require liver transplantation for long-term survival.
- Predictive factors for native liver survival in BA are currently limited.
Purpose:
- To identify prognostic factors for long-term native liver survival in biliary atresia patients.
- To evaluate the correlation between pre-Kasai operation variables and patient outcomes.
- To assess the utility of post-Kasai liver function tests in predicting native liver survival.
Summary:
- This retrospective study analyzed 85 biliary atresia patients from 1993-2003.
- Age at Kasai operation, liver fibrosis, and pre-Kasai lab results did not correlate with outcome.
- Normal bilirubin levels at 3, 6, and 12 months post-Kasai, and AST/gammaGT at 6 months, were associated with native liver survival.
Impact:
- Findings suggest that current predictive factors are insufficient to guide primary liver transplantation decisions in BA.
- Highlights the importance of monitoring post-Kasai liver function tests for assessing prognosis.
- Emphasizes the need for further research into reliable prognostic markers for biliary atresia.
Abstract:
Most children with biliary atresia require liver transplantation, and only about 20% survive in the long term with their native livers. Prognostic factors that determine disease progression are still lacking. This retrospective survey of 85 BA patients from 1993 to 2003 was aimed to evaluate prognostic factors using the log rank test. After 5 years 40% of the patients are alive with their native livers (35/85), 26 of them with normal bilirubin (31%). Age at Kasai operation (P=0.46), degree of liver fibrosis (P=0.95), and all laboratory test results before Kasai failed to correlate with outcome. Normal levels of bilirubin 3, 6, and 12 months after Kasai and of aspartate aminotransferase with gammaGT after 6 months are associated with survival with native liver. In conclusion our data demonstrate that a lack of predictive factors must prevent primary liver transplantation in BA patients.
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