Assessment of health-related quality of life in Egyptian children and adolescents with congenital adrenal hyperplasia

Noha Musa; Noha Asem; Shaza Basyony; Lubna Fawaz

doi:10.1515/jpem-2019-0345

Article

Assessment of health-related quality of life in Egyptian children and adolescents with congenital adrenal hyperplasia

Noha Musa , Noha Asem , Shaza Basyony and Lubna Fawaz

Published/Copyright: February 5, 2020

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From the journal Journal of Pediatric Endocrinology and Metabolism Volume 33 Issue 2

Abstract

Background

Congenital adrenal hyperplasia (CAH) is a chronic disorder causing adrenal insufficiency and hyperandrogenism affecting the quality of life (QOL). The objective of the study was to assess the health-related QOL (HRQOL) in Egyptian children and adolescents with CAH and to identify factors affecting it.

Methods

This cross-sectional study included 200 CAH patients (with 21-hydroxylase deficiency [21-OHD]) who were assessed according to their age, sex, clinical phenotype, timing of genitoplasty, hospital admissions within the last year, compliance to treatment, regularity of follow-up, presence of complications and hormonal control. HRQOL was assessed using the World Health Organization (WHO)QOL-BREF questionnaire with four domains analyzed independently including physical, psychological, social and environmental domains, with higher scores indicating better QOL.

Results

The study included 140 females and 60 males with a mean age of 6.6 ± 4.5 years, and 88% were salt-wasting (SW). Older patients had significantly lower QOL scores (r = − 0.151, p = 0.033). The physical domain correlated significantly with the degree of virilization (r = − 0.491, p = 0.001) and frequency of hospitalization (r = − 0.495, p < 0.001). The psychological domain was affected by age (r = − 0.157, p = 0.026) and timing of genitoplasty (r = − 0.326, p = 0.001), while the social domain was affected by age (r = −0.277, p < 0.005) and pubertal stage (r = − 0.195, p = 0.006). Females had lower scores at the psychological domain (p < 0.001), whereas males had lower scores at the physical domain (p = 0.003). Salt-losing patients had lower scores at the physical domain (p = 0.001). Patients with good hormonal control had higher scores at the physical domain (p = 0.03). Genitoplasty affected both psychological and social domains (p = 0.003 and 0.01, respectively). Patients with hypertension and hirsutism had lower QOL scores (p < 0.05).

Conclusions

HRQOL was relatively more affected in CAH patients with older age, poor hormonal control, high frequency of hospital admissions and those who developed complications.

Keywords: 21-hydroxylase deficiency; congenital adrenal hyperplasia; genitoplasty; hormonal control; quality of life; WHOQOL-BREF questionnaire

Author contributions: Lubna Fawaz gave the study idea and design, and revised the results. Noha Musa performed the study design, data collection, patients’ recruitment, revising results, writing the manuscript and submitting it. Shaza Basiony performed patients’ recruitment, data collection and finalizing the excel sheet. Noha Asem did the statistical analysis and revised the results.
Research funding: None declared.
Employment or leadership: None declared.
Honorarium: None declared.
Competing interests: The funding organization(s) played no role in the study design; in the collection, analysis, and interpretation of data; in the writing of the report; or in the decision to submit the report for publication.
Conflict of interest: The authors have no potential conflict of interest to disclose.
Ethical approval: All procedures performed in studies involving human participants were in accordance with the ethical standards of Cairo University and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.
Informed consent: Informed consent was obtained from every individual participant included in the study.

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Received: 2019-07-28

Accepted: 2019-11-18

Published Online: 2020-02-05

Published in Print: 2020-02-25

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Articles in the same Issue

https://doi.org/10.1515/jpem-2019-0345

Keywords for this article

21-hydroxylase deficiency; congenital adrenal hyperplasia; genitoplasty; hormonal control; quality of life; WHOQOL-BREF questionnaire