Health-Related Quality of Life in Patients with Biliary Atresia: A Systematic Review and Meta-Analysis

Objectives: In this systematic review and meta-analysis, for the first time, the existing evidence on health-related quality of life (HRQOL) was evaluated in patients with biliary atresia (BA) and compared with that of healthy population. Methods: A systematic search was done on PubMed, Scopus, Embase, Cochrane Library and Web of Science from 1990 up to April 2018 in order to identify all articles that assess the HRQOL in patients with BA. Meta-analysis was done using comprehensive metaanalysis and the standard difference of mean (SDM) was calculated. Results: A total of 188 publications were identified from the initial search. After removing duplicates and irrelevant studies, 32 articles remained for full-text evaluation. Out of this number, 22 articles were excluded due to non-relevance to the study scope leaving 10 studies for systematic review and three articles for meta-analysis. HRQOL was assessed using PedsQL4 in three studies, SF-36C in two studies and by assessing different parameters including social status, school, university, and/or sports performance in five studies. HRQOL in BA patients living with their own liver was significantly lower than that of the general population [SDM = -0.55 (95% CI: -0.69, -0.40)]. In the terms of PedsQL4 sub-scores, compared with healthy population, the levels of physical [SDM = -0.40 (95% CI: -0.55, -0.28)], social [SDM = -0.25 (95% CI: -0.40, -0.11)] and school functioning [SDM = -1.19 (95% CI: -2.35, 0.07)] were significantly lower in the group of biliary atresia.