Affiliation:
1. Postgraduate Institute of Medicine , University of Colombo, Colombo , Sri Lanka
2. Samson Gemmell Chair of Child Health, University of Glasgow , Glasgow G12 8QQ , UK
3. Department of Paediatrics, Faculty of Medicine, University of Colombo , Colombo , Sri Lanka
Abstract
Abstract
Objective
To conduct a systematic review (SR) and meta-analysis (MA) on health-related quality-of-life (QoL) and associated factors among children/adolescents with congenital adrenal hyperplasia (CAH).
Method
Following registration in the PROSPERO International Prospective Register of Systematic Reviews(reg no: CRD42022313389), Google Scholar, PubMed, LILACS, Cochrane, and Scopus databases were searched up to March 5, 2022, using predefined search strategy/MESH terms to identify original studies describing/assessing self-reported/parent-reported health-related QoL in patients with CAH ≤21 years. Methodological quality was assessed by Newcastle-Ottawa Quality Assessment Scale (NOS), and heterogeneity by I2 statistics. MA assessed mean difference (MD) in QoL between children/adolescents with CAH and healthy children/adolescents.
Results
Among 1308 publications, the 12 studies eligible for the SR (CAH n = 781) showed NOS scales of 3 to 7/9, and the 6 eligible for MA (CAH n = 227) showed moderate-considerable heterogeneity. MA showed that parent-reported psychosocial QoL (MD 9.9 [−12.6,7.3], P ≤ .001) {consisting of school (MD 7.4[−12.2, −2.5], P = .003), emotional (MD 5.6 [−10.2, −0.9], P = .02) and social domains (MD 4.3 [−8.1, −0.5], P = .03), and self-reported school domain QoL (MD 8.5 [−15.9, −1.2], P = .02) was lower in children/adolescents with CAH while parent-reported and self-reported physical QoL were similar to controls.
Factors associated with lower QoL among children/ adolescents with CAH included poor disease control, poor medication compliance, and complications including hyperpigmentation, virilization, hypertension, hospital admission, and urinary incontinence.
Conclusion
Based on available data, children/adolescents with CAH had preserved physical QoL but impaired psychosocial QoL, especially in the school domain. Factors associated with lower QoL included poor disease control and disease/treatment-related complications. There is a need for further high-quality research that investigates the relationship between disease control, provision of psychosocial support, and improvement in QoL in children/adolescents with CAH.