Quality of life after living donor liver transplant for biliary atresia in
Department of Family Nursing, Division of Health Sciences and Nursing, Graduate School of Medicine, The University of
Department of Transplant Surgery, Jichi Medical University and
Center for Department of Transplantation
and Regeneration, Jichi Medical University Hospital, Shimotsuke City, Tochigi, Japan
Abstract Background: Health-related quality of life (HRQOL) is an important outcome in solid organ transplantation. This
study evaluated and explored the factors of generic and transplant-speciﬁc HRQOL in Japanese pediatric and
adolescent patients with biliary atresia (BA) after living donor liver transplant (LDLT).
Methods: A cross-sectional survey using anonymous questionnaires was completed between April and July 2015.
Patient medical records were accessed. The Japanese version of Pediatric Quality of Life Inventory
Scales and Transplant Modules (child self-report and parent proxy-report) was administered.
Results: Participants consisted of 75 patients (mean age at survey, 9.6 years) and 74 parents. Japanese patients
reported higher generic and transplant-speciﬁc HRQOL (total score) than that reported by US patients with BA after
LT (US I; age at survey, 7.2 years) and by US patients after solid organ transplant (US II; age at survey,
11.3 years; LT, 53.8%; effect size, 0.55–0.96). Japanese parents, however, rated their children’s generic HRQOL
(total score) similar to that rated by the US I and II parents (0.13 and 0.30, respectively) and reported lower trans-
plant-speciﬁc HRQOL (total score) than that reported by US II (0.26). Although the number of types of prescribed
drugs was a common factor in HRQOL, most demographic and medical factors (e.g. child’s age at survey and
consultation frequency) varied with reporter (i.e. patients and parents).
Conclusions: The levels and factors of generic and transplant-speciﬁc HRQOL of Japanese pediatric and adolescent
patients with BA after LDLT varied with reporter (i.e. patients or parents).
Key words biliary atresia, health-related quality of life, liver transplantation, patient-reported outcome, Pediatric Quality of Life
Liver transplantation (LT) is one of the treatment options to
improve survival rate and health-related quality of life
(HRQOL) in pediatric and adolescent patients with end-stage
liver failure. In Japan, biliary atresia (BA) frequently necessi-
tates LT in children and adolescents, with >1,700 pediatric
and adolescent patients having undergone LT, most of whom
received living donor liver transplantation (LDLT).
LT for BA achieves good outcomes, and the 5 year survival
rate in Japanese patients with BA after LT is approximately
In the assessment of outcomes of LT for pediatric and
adolescent BA, objective indexes, such as patient and graft
survival rate, are usually used, but patient-reported outcomes
in the Japanese population have not been assessed. Organ
transplant aims to cure the primary disease and entails a tran-
sition to a new chronic condition.
Compared with other
diseases, for BA, LT is most often required during infancy,
thereby prolonging the number of post-transplant years. There-
fore, in transplantation, patient outcomes cannot be assessed
only using objective indexes, and it is important to assess
HRQOL outcomes from the perspective of the patient.
The HRQOL integrates domains of objective assessments
of personal functioning or health status as well as subjective
perceptions of health.
These domains vary according to
respondent personal characteristics (e.g. age and disease). In
pediatric and adolescent solid organ transplant patients,
HRQOL encompasses not only the general aspects of physical,
emotional, social, and school functioning but also the trans-
plant- and disease-speciﬁc HRQOL.
The aim of this study
was therefore to evaluate and explore the factors of generic
and transplant-speciﬁc HRQOL in Japanese pediatric and
adolescent patients with BA after LDLT. To evaluate the Japa-
nese HRQOL, two coeval US populations were used from pre-
vious studies: patients with BA after LT (US I) and patients
after solid organ (e.g. liver, kidney, and heart) transplant (US
Correspondence: Kiyoko Kamibeppu, RN PHN PhD, Department
of Family Nursing, Division of Health Sciences and Nursing,
Graduate School of Medicine, The University of Tokyo, 7-3-1
Hongo, Bunkyo-ku, Tokyo 113-0033, Japan. Email: kkamibeppu-
Received 7 November 2016; revised 3 October 2017; accepted
20 October 2017.
© 2017 Japan Pediatric Society
Pediatrics International (2018) 60, 183–190 doi: 10.1111/ped.13442