Growth failure and outcomes in infants with biliary atresia: a report from the Biliary Atresia Research Consortium

Abstract

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.

Fig. 1

Mean weight-for-age z-scores in infants with biliary atresia in the good and poor outcome groups at presentation, at HPE, 3 months after HPE and at 6 months after HPE. Dark bars represent the good outcome group and hatched bars represent poor outcome group. A significant difference in mean weight z-scores was seen at 6 months after HPE between the good and poor outcome groups (P < 0.01). HPE, hepatoportoenterostomy.

Fig. 2

Weight for age z-scores in infants with biliary atresia comparing good and poor outcome groups on each graph. (A) Original data for all 100 subjects; (B) Trimmed data for all 100 subjects; (C) Original data for only subjects without BASM or ascites; (D) Trimmed data for only subjects without BASM or ascites. Good outcome group is represented by solid lines and poor outcome group is represented by dashed lines. The dotted vertical line on each graph represents time of HPE. The 2 outer lines are the 95% confidence intervals. Among the 100 subjects, the number of children followed (with measurements) at HPE were 52 (good outcome) and 46 (poor outcome), at 6 months after HPE 48 and 32, at 12 months after HPE 47 and 13 and at 18 months after HPE 45 and 4, respectively. Analysis on the complete dataset (n = 100) shows a significant difference in the shape of the growth curves between the good and poor outcome group (P < 0.0001). Excluding children with BASM and ascites, the significant difference remains (P = 0.0001). HPE, hepatoportoenterostomy.

Fig. 3

Length for age z-scores in infants with biliary atresia comparing good and poor outcome groups on each graph. (A) Original data for all 100 subjects; (B) Trimmed data for all 100 subjects; (C) Original data for only subjects without BASM or ascites. (D) Trimmed data for only subjects without BASM or ascites. The good outcome group is represented by solid lines and poor outcome group is represented by dashed lines. The dotted vertical line on each graph represents time of HPE. The 2 outer lines are the 95% confidence intervals. Among the 100 subjects, the number of children followed (with measurements) at HPE were 51 (good outcome) and 46 (poor outcome), at 6 months after HPE 47 and 30, at 12 months after HPE 47 and 12, and at 18 months after HPE 44 and 4, respectively. Analysis on the complete dataset (n = 100) shows a significant difference in the shape of the growth curves between the good and poor outcome group (P < 0.0001). Excluding children with BASM and ascites, the significant difference remains (P < 0.0001). HPE, hepatoportoenterostomy.

Fig. 4

Mean weight-for-age z-scores at 3 and 6 months after HPE in the subgroup with total bilirubin concentrations between 2 and 6 mg/dl at 3 months after HPE. Dark bars represent the good outcome group and hatched bars represent poor outcome group. A significant difference in mean weight z-scores were seen between the good and poor outcome groups at 3 months (P = 0.04) and 6 months (P = 0.02) after HPE. HPE, hepatoportoenterostomy.

Fig. 5

Weight-for-age z-scores in subgroup (n = 28) with total bilirubin between 2 and 6 mg/dl at 3 months after HPE; a comparison of the good and poor outcome groups on each graph. The original data are shown on the left graph and trimmed data are shown on the right graph. The good outcome group is represented by solid lines and poor outcome group is represented by dashed lines. The dotted vertical line on each graph represents time of HPE. The 2 outer lines are the 95% confidence intervals. The number of children followed (with measurements) at HPE were 10 (good outcome) and 18 (poor outcome), at 6 months after HPE 10 and 15, at 12 months after HPE 10 and 7, and at 18 months after HPE 8 and 2, respectively. There was a significant difference in the shape of the growth curves between the good and poor outcome group (P < 0.05). HPE, hepatoportoenterostomy.