Growth failure and outcomes in infants with biliary atresia: A report from the Biliary Atresia Research Consortium
✍ Scribed by Patricia A. DeRusso; Wen Ye; Ross Shepherd; Barbara A. Haber; Benjamin L. Shneider; Peter F. Whitington; Kathleen B. Schwarz; Jorge A. Bezerra; Philip Rosenthal; Saul Karpen; Robert H. Squires; John C. Magee; Patricia R. Robuck; Ronald J. Sokol
- Publisher
- John Wiley and Sons
- Year
- 2007
- Tongue
- English
- Weight
- 388 KB
- Volume
- 46
- Category
- Article
- ISSN
- 0270-9139
No coin nor oath required. For personal study only.
✦ Synopsis
Malnutrition is a significant clinical problem in infants with biliary atresia. The natural history of poor growth and its potential association with early transplantation or death in children with biliary atresia was determined. Serial weight-and length-for-age z-scores were computed as part of a retrospective study of 100 infants who underwent hepatoportoenterostomy (HPE) for biliary atresia at 9 U.S. pediatric centers between 1997 and 2000. Poor outcome was defined as transplantation or death by 24 months of age (n ؍ 46) and good outcome was defined as survival with native liver at 24 months of age with total serum bilirubin less than 6 mg/dL (n ؍ 54). Growth velocity was significantly slower in the poor outcome group compared to the good outcome group (P < 0.001 for both weight and length). Mean weight z-scores were significantly lower by 6 months after HPE in the poor outcome group (؊2.1 ؎ 1.4) compared to the good outcome group (؊1.2 ؎ 1.4) (P < 0.001). In a subgroup with total bilirubin between 2 and 6 mg/dL at 3 months after HPE (n ؍ 28), the weight z-scores at 3 months after HPE were significantly lower in the poor outcome group (؊2.0 ؎1.2) compared to the good outcome group (؊1.0 ؎ 1.2) (P ؍ 0.04) despite similar bilirubin concentrations. Conclusion: Growth failure after HPE was associated with transplantation or death by 24 months of age. The combination of intermediate bilirubin concentrations and poor mean weight z-scores 3 months after HPE was also associated with poor clinical outcome. (HEPATOLOGY 2007;46:1632-1638.) See Editorial on Page 1329
B iliary atresia is a neonatal cholangiopathy of unknown etiology that affects 1 in 8,000 to 18,000 newborns. 1,2 It is the most frequent indication for liver transplantation in children accounting for almost 50% of pediatric liver transplants in the United States. 1,2 Early surgery with hepatoportoenterostomy (HPE) to re-lieve extrahepatic biliary obstruction and restore bile flow is critical. Despite surgery, however, progressive hepatic fibrosis often ensues and liver transplantation is eventually necessary in 70%-80% of patients. At least half of infants with biliary atresia are likely to undergo liver transplantation within the first 2 years of life. [3][4][5] The evaluation of outcomes in infants with biliary atresia after HPE has been one focus of the Biliary Atresia Research Consortium (BARC), which was formed in