الفهرس يوجد فقط 14 صفحة متاحة للعرض العام
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المستخلص
Background: Congenital Adrenal Hyperplasia (CAH) is a chronic endocrinal disorder affecting both sexes and characterized by enzymatic deficiency in adrenal steroidogenesis, leading to adrenal insufficiency and hyperandrogenism with a wide range of clinical severity. Recent studies have demonstrated a reduction in the quality of life (QOL) in patients with CAH. Objectives: To assess the health-related QOL in Egyptian children and adolescents with CAH and to study different factors affecting their QOL. Method: In this cross-sectional study, 200 patients with CAH due to 21- hydroxylase deficiency (21-OHD) regularly attending the Diabetes, Endocrine and Metabolism Pediatric Unit (DEMPU) at Cairo University Children’s Hospital were included. HRQOL was assessed using the WHOQOL-BREF questionnaires with 4 domains analyzed independently including physical, psychological, social relationship and environmental domains with higher scores indicating better QOL. Results: The study included 140 females and 60 males between 1 to 18 years old. Older children and adolescents had significantly lower total QOL scores (r=-0.151, p=0.033). Females had lower scores at psychological domain (p<0.001), whereas males showed lower scores at physical domain (p=0.003). Salt losing CAH patients had shown lower scores than simple virilizing at physical domain (p=0.003). Patients on regular follow up scored higher in the environmental domain (p=0.037). Females who had undergone clitroplasty showed statistically significant higher scores at psychological domain (p=0.003). Although, those who had undergone vaginoplasty after clitroplasty showed statistically significant lower scores at social domains compared to those who are not (p=0.010). Conclusion: HRQOL was relatively more affected in CAH patients with older age, high frequency of hospital admissions and those who developed complications