Health related quality of life in patients with biliary atresia surviving with their native liver

Abstract

Objectives: To quantify health related quality of life (HRQOL) of patients with biliary atresia with their native livers and compare them with healthy children and patients with biliary atresia post-liver transplant (LT) and to examine the relationship between HRQOL and medical variables.

Study design: A cross-sectional HRQOL study of patients with biliary atresia with their native livers (ages 2-25 years) was conducted and compared with healthy and post-LT biliary atresia samples using Pediatric Quality of Life Inventory 4.0 child self and parent proxy reports, a validated measure of physical/psychosocial functioning.

Results: 221 patients with biliary atresia with native livers (54% female, 67% white) were studied. Patient self and parent proxy reports showed significantly poorer HRQOL than healthy children across all domains (P < .001), particularly in emotional and psychosocial functioning. Child self and parent proxy HRQOL scores from patients with biliary atresia with their native livers and post-LT biliary atresia were similar across all domains (P = not significant). Child self and parent proxy reports showed moderate agreement across all scales, except social functioning (poor to fair agreement). On multivariate regression analysis, black race and elevated total bilirubin were associated with lower Total and Psychosocial HRQOL summary scores.

Conclusions: HRQOL in patients with biliary atresia with their native livers is significantly poorer than healthy children and similar to children with post-LT biliary atresia. These findings identify significant opportunities to optimize the overall health of patients with biliary atresia.

Keywords: ALT; Alanine aminotransferase; GI; Gastrointestinal; HPE; HRQOL; Health related quality of life; Hepatic portoenterostomy; ICC; Intra-class correlation coefficient; LT; Liver transplant; Pediatric Quality of Life Inventory; PedsQL.

Figure

Mean physical, psychosocial and total summary scores by child self report and parent proxy report were significantly lower (*p: <0.001) than the healthy comparison population, as well as the biliary atresia population post-liver transplant. Child self-report and parent proxy reports of PedsQL™ 4.0 Physical, Psychosocial and Total Summary Scores Mean physical, psychosocial and total summary scores by child self report and parent proxy report were significantly lower (*p: <0.001) than the healthy comparison population, as well as the BA population post-liver transplant.