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

Shikha S. Sundaram*, Estella M. Alonso, Barbara Haber, John C. Magee, Emily Fredericks, Binita Kamath, Nanda Kerkar, Philip Rosenthal, Ross Shepherd, Christine Limbers, James W. Varni, Patricia Robuck, Ronald J. Sokol

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

31 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)1052-1057.e2
JournalJournal of Pediatrics
Volume163
Issue number4
DOIs
StatePublished - Oct 2013

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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