PubMed ID:
17929308
Public Release Type:
Journal
Publication Year: 2007
Affiliation: Johns Hopkins School of Medicine and The Johns Hopkins Children's Center, Baltimore, Maryland, USA.
DOI:
https://doi.org/10.1002/hep.21923
Authors:
Ye W,
Bezerra JA,
Biliary Atresia Research Consortium,
DeRusso PA,
Haber BA,
Karpen S,
Magee JC,
Robuck PR,
Rosenthal P,
Schwarz KB,
Shepherd R,
Shneider BL,
Sokol RJ,
Squires RH,
Whitington PF
Studies:
A Prospective Database of Infants With Cholestasis
,
Biliary Atresia Research Consortium
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.