Research on Quality of Life in Female Patients with Congenital Adrenal Hyperplasia and Issues in Developing Nations

Zainuddin, Ani Amelia; Grover, Sonia; Shamsuddin, Khadijah; Mahdy, Zaleha Abdullah

doi:10.1016/j.jpag.2012.08.004

Cited by 46 publications

(54 citation statements)

References 91 publications

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“…Previous research on HRQOL in CAH patients has primarily focused on adults [2,3,4,5,6,7,14], whereas pediatric data have been limited. A recent study from the Netherlands documented few negative effects of the disease on physical, social, and societal functioning [8].…”

Section: Discussionmentioning

confidence: 99%

Health-Related Quality of Life in Children with Congenital Adrenal Hyperplasia

et al. 2015

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Background/Aims: Factors in congenital adrenal hyperplasia (CAH) that may affect quality of life (QOL) include the need for lifelong medication, the risk of adrenal crisis, and hyperandrogenic symptoms. The objectives were to evaluate health-related QOL (HRQOL) in children with CAH, and whether CAH poses an additional burden compared to other endocrine disorders. Methods: The validated PedsQL 4.0 generic core scales were administered to subjects (8-18 years) with CAH and hypothyroidism and their parents. The minimal clinically important difference (MCID) was determined for each scale score, allowing a comparison with the healthy population. A score of >1 standard deviation below the population mean was considered at risk for impaired HRQOL. Results: In CAH, the mean total HRQOL scores were >1 MCID below the population mean, and a higher percentage than expected had scores considered at risk. Conclusion: Compared to subjects with hypothyroidism, subjects with CAH self-reported lower school domain scores. CAH subjects more frequently reported peers not wanting to be friends.

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Section: Discussionmentioning

confidence: 99%

Health-Related Quality of Life in Children with Congenital Adrenal Hyperplasia

et al. 2015

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“…Moreover, there are added difficulties given the fact that these patients live in a developing country. Poverty, ignorance, lack of accessibility to specialized medical care, and lack of psychological services may all contribute negatively to these patients’ outcomes [25]. …”

Section: Discussionmentioning

confidence: 99%

Quality of Life in Children with Disorders of Sex Development

Selveindran¹,

Zakaria

Jalaludin

et al. 2017

Horm Res Paediatr

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Background/Aims: Disorders of sex development (DSD) are a heterogeneous group of rare conditions. Evidence-based treatment is challenged by a lack of clinical longitudinal outcome studies. We sought to investigate the quality of life of children with DSD other than congenital adrenal hyperplasia. Methods: The participants (aged 6–18 years) were 23 patients raised as males and 7 patients raised as females. Control data were obtained from representatives of the patients’ siblings matched for age and gender. The Pediatric Quality of Life Inventory^TM Version 4.0 (PedsQL) Generic Core Scales were used as the study tool. Results: In comparison with the reference data, the patient group had significantly lower overall PedsQL (p < 0.01) and school functioning (p < 0.01) scores. Also, the total PedsQL score was significantly lower in patients with DSD who were of female social sex as compared to the controls who were females. Family income, surgical procedures, degree of virilization, and mode of puberty did not influence the PedsQL scores. Conclusion: This study revealed a poorer quality of life for patients with DSD as compared to the age-matched control group. This highlights the need for a skilled multidisciplinary team to manage this group of patients.

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“…These facts are best demonstrated by studies in developing countries, where financial concerns, such as having to buy essential medications at high cost or to travel from far to reach a specialist, constitute primary burdens for affected families [5]. A study performed in a Malaysian tertiary center revealed that approximately one-third of patients with CAH had poor knowledge of their condition or did not realize that their medications were required to sustain life [6]. Additional issues impacting CAH families in these countries include lack of newborn screening, which may lead to late diagnosis and gender assignment without appropriate evaluation.…”

Section: Introductionmentioning

confidence: 99%

Advances in diagnosis and care of persons with DSD over the last decade

Lee

Wisniewski

Baskin

et al. 2014

Int J Pediatr Endocrinol

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It is clear that the major issues raised by the Chicago Disorders of Sex Development (DSD) Consensus meeting primarily the need of more data, especially outcome information, are still not available. Hence, there are insufficient data to merit another consensus statement. However, there has been a major shift in the thinking and approach to the care of patients with DSD. This was a consequence of the emphasis of the need to reconsider the criteria for sex assignment, to incorporate new genetic and hormonal knowledge in the care, and to investigate impact of surgical timing and techniques. Much of the reconsideration is in response to patient, family, social and legal demands, including the need for full disclosure and family or individual participation in decisions. Further, there remains a lack of sufficient information to provide predictors for future gender development. Hence, it still is impossible to develop specific clinical guidelines to apply to patients generally or individually. Thus, it is pertinent to periodically evaluate and assess some of these multifaceted changes. This report discusses cultural and social forces, legal factors, surgical perspectives, treatment shifts including psychological approaches, progress regarding genetic diagnosis, gender issue comparisons with transgender patients, and on-going research studies occurring since the consensus conference.

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Research on Quality of Life in Female Patients with Congenital Adrenal Hyperplasia and Issues in Developing Nations

Cited by 46 publications

References 91 publications

Health-Related Quality of Life in Children with Congenital Adrenal Hyperplasia

Health-Related Quality of Life in Children with Congenital Adrenal Hyperplasia

Quality of Life in Children with Disorders of Sex Development

Advances in diagnosis and care of persons with DSD over the last decade

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