Growth failure and outcomes in infants with biliary atresia: a report from the 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.


CONCLUSIONS
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.
DeRusso
Patricia A
PA
Johns Hopkins School of Medicine and The Johns Hopkins Children's Center, Baltimore, Maryland, USA.
Ye
Wen
W
Shepherd
Ross
R
Haber
Barbara A
BA
Shneider
Benjamin L
BL
Whitington
Peter F
PF
Schwarz
Kathleen B
KB
Bezerra
Jorge A
JA
Rosenthal
Philip
P
Karpen
Saul
S
Squires
Robert H
RH
Magee
John C
JC
Robuck
Patricia R
PR
Sokol
Ronald J
RJ
D001656:Biliary Atresia / Q000150:complications* / Q000401:mortality / Q000601:surgery; D001663:Bilirubin / Q000097:blood; D015331:Cohort Studies; D005260:Female; D006130:Growth Disorders / Q000209:etiology*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D016031:Liver Transplantation; D008297:Male; D010288:Parenteral Nutrition; D012189:Retrospective Studies; D012307:Risk Factors; D016896:Treatment Outcome; D014481:United States / Q000453:epidemiology
D001663:Bilirubin