Improvement of health-related quality of life in adult women with 21-hydroxylase deficiency over a seven-year period

Bleicken, Benjamin; Ventz, Manfred; Hinz, Andreas; Quinkler, Marcus

doi:10.1507/endocrj.ej12-0183

Cited by 6 publications

(3 citation statements)

References 34 publications

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“…Proper treatment might have a greater influence on the patients than the genotype, as indicated in a recent study [ 22 ]. A longitudinal study on CAH also showed that daily-reduced hydrocortisone dose might be helpful for the improvement of QOL [ 23 ]. (3) To protect their personal privacy, we adopt patient-centred service concept.…”

Section: Discussionmentioning

confidence: 99%

The Investigation of Quality of Life in 87 Chinese Patients with Disorders of Sex Development

Wang

Tian

2015

BioMed Research International

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Objective. In the process of care for disorders of sex development (DSD), clinical decisions should focus on the long-term quality of life (QOL). We sought to investigate the QOL of patients with DSD in China. Design. Case-control study was carried out. Patients. 90 patients of DSD participated in the study. Finally, 87 patients were analyzed including Turner's syndrome (23), Noonan syndrome (2), androgen insensitivity syndrome (22), testicular regression syndrome (2), congenital adrenal hyperplasia (16), and pure gonadal dysgenesis (22). Measurements. The WHOQOL-BREF questionnaire was chosen for the present investigation. Four domain scores were analyzed independently including physical, psychological, and social relationship and environmental domains. Results. The average age of the DSD group is 22.34 ± 4.97 years, and only 13.79% patients ever had sexual life. The scores of psychological and environmental domains were lower than that of the physical and social relationship domains, but the difference was not significant (P > 0.05). Compared with the Chinese urban population, the QOL scores of DSD patients in China were not significantly lower. Conclusions. With proper treatment, including the follow-up and psychological support, the QOL of DSD patients cannot be significantly reduced. For DSD patients, more attention should be paid to the potential psychological and sexual problems.

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Section: Discussionmentioning

confidence: 99%

The Investigation of Quality of Life in 87 Chinese Patients with Disorders of Sex Development

Wang

Tian

2015

BioMed Research International

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“…However, high doses of glucocorticoids might worsen QoL as well, probably through metabolic changes (e.g., obesity), suppression of the gonadal axis and neuropsychological changes, e.g observed in patients with Cushing’s syndrome. Reduction in high doses of glucocorticoids seems to improve QoL in CAH patients [ 49 ].…”

Section: Discussionmentioning

confidence: 99%

Body Image and Quality of Life in Women with Congenital Adrenal Hyperplasia

Tschaidse

Quinkler²,

Grinten

et al. 2022

JCM

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Objective: Women with congenital adrenal hyperplasia due to 21-hydroxylase deficiency (CAH) may have poor quality of life (QoL) and low satisfaction with body appearance. We investigated the influence of the patients’ satisfaction with their support on their QoL and body image. Design: Retrospective, comparative, Europe-wide study as part of the multicenter dsd-LIFE study. Methods: 203 women with CAH were included in this study. We investigated the patients’ QoL and body image compared to a healthy control group. The patients’ satisfaction with their treatment and support in childhood and adolescence as well as in adulthood was assessed by questionnaire and its influence on the patients’ body image and QoL was analyzed by multiple regression models. Results: Women with CAH showed worse body image and poorer physical, psychological and social QoL compared to a healthy reference population. The patients’ satisfaction with professional care in the last 12 months was a significant positive predictor for all four domains of QoL (psychological, physical, social, environmental). Dissatisfaction with care in childhood and adolescence and with general support through different stages of life was a significant negative predictor for QoL and body image. Conclusions: These results show that women with CAH have poor QoL and body image compared to a healthy reference population. Psychosocial factors such as general and family support, and social interactions with professionals have a substantial impact on QoL and body image in adult females with CAH. This should be taken into account regarding patient care and multimodal therapy.

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“…As increasing the daily drug doses the bad impact on physical, psychological and social wellbeing increases. This was emphasized by a longitudinal study on CAH patients that revealed increased quality of life by reducing the daily drug doses (Bleicken et al, 2012).…”

Section: Proper and Early Psychosocial Management Is Very Important F...mentioning

confidence: 99%

Study of the Quality of Life in children and adolescents with Disorders of Sex Development (DSD)

Zamzam,

Mazen,

Abdel Latif

et al. 2023

Middle East Journal of Medical Genetics

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Background: Disorders of sex development (DSD), are medical conditions in which development of chromosomal, gonadal, or anatomical sex is atypical. Quality of life (QOL) includes important aspects of physical and mental health as well as social interactions, economic conditions, personal views, and their connections the environment. limited researches are available regarding QOL in DSD patients especially children. However, recent studies showed reduction in QOL in DSD patients. Aim of the work:The aim of the study is to assess the quality of life in DSD patients to improve clinical practice in the management of disorders of sex development. Patients and Methods:This cross-sectional study to assess the health-related QOL in 50 DSD children and adolescents who attended the endocrinology clinic at Cairo University Children's Hospital and National Research Centre (NRC) from March 2018 to December 2019, in addition to 50 healthy controls. Results: While comparison between DSD patients who were reared as males and those who were reared as females as regards WHOQOL domains score showed a significant difference in all WHOQOL domains and total score except in physical domain only there was no significant difference. There was a significant difference between 46, XX DSD, 46, XY DSD and Sex chromosome DSD in psychological (D2), environmental domain (D4) and to lesser extent in social domain (D3). Sex chromosome DSD patients were the most affected patients in the 4 WHOQOL domains, which was significantly lower than 46, XX DSD (217.50±66.76) and 46, XY DSD (255.06±48.95) with P value= 0.001. Comparison between 46, XX DSD, 46, XY DSD and sex chromosome DSD patients in age, family history, consanguinity, educational level, surgical and hormonal treatment showed significant difference between the 3 groups in family history and consanguinity with higher percentage of positive family history and consanguinity in 46, XX DSD then 46, XY DSD. DSD patients who received hormonal treatment and underwent surgical correction had significant higher scores in WHOQOL. Conclusion: Pediatric patients with DSD have significantly lower HRQOL in psychological and social aspects rather than in physical and environmental aspects. HRQOL is relatively more affected in Sex chromosome DSD patients. DSD patients reared as males had a significant better HRQOL than DSD patients reared as females. DSD patients who underwent surgical correction showed a statistically significant positive effect on HRQOL.

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Improvement of health-related quality of life in adult women with 21-hydroxylase deficiency over a seven-year period

Cited by 6 publications

References 34 publications

The Investigation of Quality of Life in 87 Chinese Patients with Disorders of Sex Development

The Investigation of Quality of Life in 87 Chinese Patients with Disorders of Sex Development

Body Image and Quality of Life in Women with Congenital Adrenal Hyperplasia

Study of the Quality of Life in children and adolescents with Disorders of Sex Development (DSD)

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