Quality of Life in Children with Wilson Disease: A Single Center Study

Document Type : Original Research

Authors

1 Department of Pediatrics, Faculty of Medicine, Cairo University, Cairo, Egypt

2 Department of Pediatrics, National Institute of Health, Cairo, Egypt

Abstract

Background: Wilson disease (WD) is associated with compromised health-related quality of life (HRQoL) in both adults and children, even in the early stages of the liver disease.
Aim of the work: to assess HRQoL children and adolescents suffering from WD and to investigate factors that may affect the HRQoL among them.
Subjects and Methods: This was a case-control study including 30 WD patients and 30 age- and sex-matched normal healthy controls. The HRQoL was evaluated by the World Health Organization Quality of Life BREF questionnaire (WHOQoL-BREF) and chronic liver disease questionnaire (CLDQ) for both patients and controls.
Results: The mean ± SD age of the patients was 14.07 ± 3.36 years compared to 14.58± 3.391 years of the control group (p=0.96). At the initial presentation to our unit, 18 patients (60%) were symptomatic and 12 patients (40%) were asymptomatic. At the study enrollment, liver function tests were within normal ranges in 15 patients (50%), while the other 15 (50%) patients had deranged liver functions. Children with WD had significantly poorer HRQoL scores than normal controls regarding physical (54.3 ±14.2 vs 97.2 ±3.7) (p=0.000), psychological (33.4 ±9.9 vs 57.5 ± 7.6) (p=0.000), social (58 ±17 vs 83.5 ±9) (p=0.000), and environmental domains (40.3±11.2 vs 52.3 ±5.8) (p=0.000) of WHOQoL. Females exhibited significant declined scores in the worry domain of the CLDQ compared to males (p=0.017). Patients with longer disease duration had worse scores CLDQ in emotional (26.2 ± 2.9) and worry (13.2 ± 2.8) domains (p=0.050) and (p=0.044) respectively, but not the WHOQoL (physical p=0.2, psychological p=0.17, social P=0.85, environmental p=0.11).
Conclusion: Children with WD have significantly compromised HRQoL, compared to their healthy peers. The HRQoL in children with chronic liver disease complications were more affected in the physical aspects. Worry represents a significant morbidity among females with WD.

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