ESPE Abstracts

Introduction: Congenital adrenal hyperplasia (CAH) is a lifelong condition associated with long term medical and psychosocial issues, which can adversely affect Quality of Life (QoL). There is paucity of high-grade evidence on health-related QoL in children and adolescents with CAH, with available studies being limited by small study samples. We conducted a systematic-review(SR) and meta-analysis(MA) to assess factors associated with health-related QoL among children and adolescent with CAH worldwide, and compare self-reported and parent-reported QoL of children/adolescents with CAH with healthy children/adolescents.

Method: This study was registered prospectively in the PROSPERO International-prospective-register-of-systematic-reviews (reg no: CRD42022313389). Google-scholar, PubMed, LILACS, Cochrane and Scopus databases were searched up to 03/05/2022, using a pre-defined search strategy and MESH terms, to identify original studies describing/assessing self-reported/parent-reported health-related QoL in patients with CAH ≤21 years. Studies comparing QoL of children/ adolescents with CAH with healthy controls using standardised validated questionnaires were included in MA. Methodological quality of studies were assessed by the Newcastle-Ottawa quality-assessment Scale (NOS), and heterogeneity by I2 statistics.

Results: A total of 1308 publications were screened, from which ten studies (CAH n= 593) were eligible for the SR (NOS scores ranging from 3-7/9), while six studies were eligible for the MA, and they showed moderate-considerable heterogeneity. MA showed that compared to healthy controls, children and adolescents with CAH (n= 227) had lower parent-reported QoL in their overall psychosocial health (MD-9.9[-12.6,7.3], P=<0.001)), as well as in all three individual psychosocial domains: school domain ((MD-7.4[-12.2, -2.5], P=0.003); emotional domain (MD-5.6[-10.2, -0.9], P=0.02) and social domain (MD-4.3[-8.1, -0.5], P =0.03); while self-reported QoL in children/ adolescents with CAH was lower in school domain only (MD-8.5[-15.9, -1.2], P=0.02). Children/ adolescents did not show any difference to healthy controls in parent- and self-reported health-related physical QoL. Factors associated with lower overall QoL among children/adolescents with CAH included: poor disease control, poor compliance to medication, higher number of hospital admissions, greater virilization, and presence of hyperpigmentation, hypertension and urinary incontinence.

Conclusion: Children/ adolescents with CAH had impaired overall psychosocial QoL from parenteral perceptive, and impaired QoL in school domain from self-report, while their physical QoL was similar to healthy children. Factors associated with lower QoL included poor disease-control, and disease/treatment-related complications. Further research is needed to determine if better disease-control and increased psychosocial support could help improve QoL in children with CAH.