Health-related quality of life of female patients with congenital adrenal hyperplasia in Malaysia

Ani Amelia Zainuddin; Sonia Regina Grover; Nur Azurah Abdul Ghani; Loo Ling Wu; Rahmah Rasat; Mohd Rizal Abdul Manaf; Khadijah Shamsuddin; Zaleha Abdullah Mahdy

doi:10.1186/s12955-020-01515-9

Health-related quality of life of female patients with congenital adrenal hyperplasia in Malaysia

Health Qual Life Outcomes. 2020 Aug 1;18(1):258. doi: 10.1186/s12955-020-01515-9.

Authors

Ani Amelia Zainuddin¹, Sonia Regina Grover^{2

3

4}, Nur Azurah Abdul Ghani², Loo Ling Wu⁵, Rahmah Rasat⁵, Mohd Rizal Abdul Manaf⁶, Khadijah Shamsuddin⁶, Zaleha Abdullah Mahdy²

Affiliations

¹ Department of Obstetrics & Gynaecology, Faculty of Medicine, National University of Malaysia (UKM), Jalan Yaacob Latif, Bandar Tun Razak, Cheras, 56000, Kuala Lumpur, Malaysia. aniameliaz71@gmail.com.
² Department of Obstetrics & Gynaecology, Faculty of Medicine, National University of Malaysia (UKM), Jalan Yaacob Latif, Bandar Tun Razak, Cheras, 56000, Kuala Lumpur, Malaysia.
³ Faculty of Medicine, Dentistry and Health Sciences, Royal Children's Hospital, Melbourne, Australia.
⁴ Department of Paediatrics, University of Melbourne, Melbourne, Australia.
⁵ Department of Paediatrics, Faculty of Medicine, National University of Malaysia (UKM), Kuala Lumpur, Malaysia.
⁶ Department of Public Health, Faculty of Medicine, National University of Malaysia (UKM), Kuala Lumpur, Malaysia.

Abstract

Background: This study investigates the health-related quality of life (HRQOL) of female patients with congenital adrenal hyperplasia (CAH) in Malaysia. The objectives were to attain socio-demographic and medical data on these Malaysian females with CAH and establish their health-related quality of life (HRQOL) in comparison to age matched diabetic controls.

Methods: A cross-sectional study was conducted over 6 months in the two main tertiary centres for CAH patients in Malaysia. Participants including 59 female-raised CAH patients (mean age ± SD = 16.3 ± 4.2 years, range 10-28 years) compared to 57 age-matched female diabetic patients (mean age ± SD = 16.5 ± 3.4 years, range 10-26 years). Socio-demographic and medical profiles was obtained through semi-structured interviews. HRQOL of participants were evaluated utilising validated, Malay translated questionnaires which were age appropriate: Pediatric Quality of Life Inventory (PedsQL v4.0) scales for Child (8-12) and Adolescent (13-18) and Medical Outcome Survey 36-item Short Form version. These were then compared to the diabetic controls.

Results: The CAH participants consisted of children (ages 10-12 years, n = 12), adolescents (ages 13-17 years, n = 29) and adults (≥ 18 years, n = 18). The majority were Malays (64.4%) and had salt-wasting CAH (67.8%). There were no significant differences between the total mean score of the HRQOL of the combined children and adolescents CAH group (total mean score ± SD = 81.6 ± 17.9, 95% CI = 75.6-87.6) when compared to age-matched diabetic patients (total mean score ± SD = 80.8 ± 11.0, 95% CI = 77.0-84.5, P = 0.81, effect size = 0.05); no significant difference between the adult CAH and diabetic controls in the physical [median score (IQR) CAH vs diabetics; 49.3 (11.4) vs. 50.2 (6.1), P = 0.60, effect size = 0.09] and the mental composite scores [median score (IQR) CAH vs. diabetics; 47.8 (14.1) vs. 50.0 (10.8), P = 0.93, effect size = 0.01].

Conclusions: The HRQOL of the Malaysian CAH cohort were comparable to the diabetic controls.

Keywords: Congenital adrenal hyperplasia; Disorders of sex development; Health-related quality of life.

MeSH terms

Adolescent
Adrenal Hyperplasia, Congenital / psychology*
Adult
Age Factors
Case-Control Studies
Child
Cross-Sectional Studies
Diabetes Mellitus / psychology
Female
Humans
Malaysia
Quality of Life*
Surveys and Questionnaires
Young Adult

Grants and funding

FF-318-2011/UKMMC Fundamental Research Fund