Psychosexual Quality of Life in Adult Intersexuality: The Example of Congenital Adrenal Hyperplasia (CAH)

2000 ◽  
pp. 200-208 ◽  
Author(s):  
Heino F. L. Meyer-Bahlburg ◽  
Sonia Gidwani ◽  
Ralf W. Dittmann ◽  
Curtis Dolezal ◽  
Susan W. Baker ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Congenital Adrenal Hyperplasia ◽  
Adrenal Hyperplasia

Metabolic profile, cardiovascular risk factors and health-related quality of life in children, adolescents and young adults with congenital adrenal hyperplasia

2019 ◽  
Vol 32 (8) ◽  
pp. 871-877 ◽  
Author(s):  
Roopa Vijayan ◽  
Nisha Bhavani ◽  
Praveen V. Pavithran ◽  
Vasantha Nair ◽  
Usha V. Menon ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Risk Factors ◽  
Cardiovascular Risk ◽  
Congenital Adrenal Hyperplasia ◽  
Cardiovascular Risk Factors ◽  
Metabolic Profile ◽  
Good Control ◽  
Adrenal Hyperplasia ◽  
Age And Sex

Abstract Background The present study was designed to evaluate the metabolic profile, cardiovascular risk factors and quality of life in children with congenital adrenal hyperplasia (CAH) and compare it with age- and sex-matched controls. Methods Fifty-two patients aged 3–21 years with classic CAH due to 21-hydroxylase deficiency were included in the study. Metabolic profiling was done for 36 cases and compared with 28 healthy age- and sex-matched controls. Quality of life was assessed in all 52 children and their parents using a validated Pediatric Quality of Life Inventory (PedsQL) questionnaire and was compared with normative data from the same population. Results The median age was 12 years with 14 (27%) males and 38 (73%) females. Out of the total 52 patients, 35 (67%) had salt wasting and 17 (33%) had simple virilising CAH. The median height standard deviation score (SDS) of cases was similar to that of controls (−0.72 vs. −0.64, p = 0.57) and 81% of females had normal pubertal status indicating a good control of the disease. Weight SDS, body mass index (BMI) SDS, mean diastolic blood pressure and insulin resistance were significantly higher in cases when compared to controls (0.31 vs. −0.3; 0.96 vs. 0.17; 67.8 ± 10.49 vs. 61 ± 8.49 and 2.1 vs. 0.95, respectively). The quality of life was significantly reduced in all domains as per parents’ perspective, whereas the children reported reduced quality of social and school functioning. There was no significant correlation between quality of life and metabolic parameters. Conclusions Children with CAH despite a reasonably good control of the disease have a higher cardiovascular risk and reduced quality of life when compared to healthy controls.

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

2020 ◽  
Vol 33 (2) ◽  
pp. 295-304
Author(s):  
Noha Musa ◽  
Noha Asem ◽  
Shaza Basyony ◽  
Lubna Fawaz
Keyword(s):  
Quality Of Life ◽  
Congenital Adrenal Hyperplasia ◽  
Children And Adolescents ◽  
Hospital Admissions ◽  
Hormonal Control ◽  
Physical Domain ◽  
Adrenal Hyperplasia ◽  
Egyptian Children ◽  
Health Related

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

scholarly journals Impact of transition on quality of life in patients with congenital adrenal hyperplasia diagnosed during childhood

2017 ◽  
Vol 6 (7) ◽  
pp. 422-429 ◽  
Author(s):  
Anne Bachelot ◽  
Magaly Vialon ◽  
Amandine Baptiste ◽  
Isabelle Tejedor ◽  
Caroline Elie ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Congenital Adrenal Hyperplasia ◽  
Psychological Health ◽  
Adult Patients ◽  
Adrenal Hyperplasia ◽  
Adult Care ◽  
Related Quality ◽  
The Impact

Background Health-related quality of life (QoL) in adult patients with congenital adrenal hyperplasia (CAH) has been variously reported. However, there is no study evaluating the impact of transition on quality of life. Methods Adult patients with classic or non-classic CAH diagnosed during childhood CAH, born between 1970 and 1990, were recruited from the registers of Pediatric departments belonging to the French reference center for endocrine rare disease. Primary end point was the QoL (WHOQOL-BREF). Results Seventy-three patients were included in the study, among them 59/73 were transferred to adult endocrinologist by their pediatricians for transition. WHOQOL-BREF scores were similar between patients with or without transition to specialist adult services, except for environment dimension score, which was slightly higher in CAH patients without transition. However, CAH patients with a regular follow-up had a better physical health, psychological health and environment score and item global QoL than the group without regular follow-up after transition. Conclusion Regular medical follow-up in adulthood is associated with the transition between pediatric and adult care and is associated with better QoL in adults with CAH.

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

2013 ◽  
Vol 26 (6) ◽  
pp. 296-304 ◽  
Author(s):  
Ani Amelia Zainuddin ◽  
Sonia R. Grover ◽  
Khadijah Shamsuddin ◽  
Zaleha Abdullah Mahdy
Keyword(s):  
Quality Of Life ◽  
Congenital Adrenal Hyperplasia ◽  
Developing Nations ◽  
Adrenal Hyperplasia ◽  
Female Patients

scholarly journals Health-related quality of life in children with congenital adrenal hyperplasia

2017 ◽  
Vol 15 (1) ◽  
Author(s):  
Alyssa Halper ◽  
Mary C. Hooke ◽  
Maria Teresa Gonzalez-Bolanos ◽  
Nancy Vanderburg ◽  
Thang N. Tran ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Congenital Adrenal Hyperplasia ◽  
Health Related Quality ◽  
Adrenal Hyperplasia ◽  
Related Quality ◽  
Health Related

scholarly journals Health Status of Children and Young Persons With Congenital Adrenal Hyperplasia in the United Kingdom: Results of a Multi-Center Cohort Study

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A717-A717
Author(s):  
Irina-Alexandra Bacila ◽  
Neil Lawrence ◽  
Sundus Mahdi ◽  
Sabah Alvi ◽  
Timothy Cheetham ◽  
...  
Keyword(s):  
Quality Of Life ◽  
United Kingdom ◽  
Health Status ◽  
Congenital Adrenal Hyperplasia ◽  
Adrenal Hyperplasia ◽  
Hydroxylase Deficiency ◽  
The United Kingdom ◽  
Number Of Patients ◽  
21 Hydroxylase Deficiency

Abstract Introduction: The association between congenital adrenal hyperplasia (CAH) and increased morbidity and mortality in adult life has been well established, however, limited knowledge exists regarding the onset of co-morbidities during childhood. Objective: To establish the health status of children and young persons (CYP) with CAH in the United Kingdom. Methods: This cross-sectional multi-center study involved 14 tertiary endocrine units across the United Kingdom. We recruited 107 patients aged 8-18 years with 21-hydroxylase deficiency and 83 matched controls. We collected and analyzed demographic, clinical, and metabolic data, as well as psychological questionnaires (Strengths and Difficulties [SDQ], Paediatric Quality of Life [PedsQL]and Self-Image Profile [SIP]). Results: The majority of patients (62.2%) were diagnosed within the first month of life, most commonly presenting with ambiguous genitalia (32.7%) or salt losing crisis (25.2%). After diagnosis, 37.3% of patients required admission for adrenal crisis, 11.2% presenting three or more episodes. Of the female patients, 57.6% had undergone urogenital examination under anesthesia and 35.5% had genital surgery. Most CAH patients received glucocorticoid (GC) replacement therapy with hydrocortisone (HC) (94.3%) and the rest with prednisolone, with a mean for relative GC doses of 13.3 (±3.7) mg/m2 per day HC-equivalent. 76.6% of patients received treatment with fludrocortisone, with a mean dose of 105.0 (±50.2) µg/m2 per day. Comparing height-Standard Deviation Score (SDS), patients under 12 years were taller (p=0.011) and patients aged 12-18 years shorter (p=0.031) than controls. Bone age was advanced in patients, with a mean difference from the chronological age of 1.9 (±2.2) years. CAH patients were more frequently overweight (26.4%) or obese (22.6%) compared to controls (10.8% and 10.8% respectively, p&lt;0.001). Five patients had high blood pressure. A small number of patients had abnormal lipid profiles: raised total cholesterol (7%), low HDL (13%), raised LDL (4%) and triglycerides (15%). Insulin resistance as defined by HOMA-IR was found in 53.8% of patients with CAH, which was comparable to healthy controls (56.3%). 15.8% of patients had SDQ scores within the “high” and “very high” categories of concern. In the PedsQL questionnaires, ‘school functioning’ was the lowest scoring dimension with a median (IQR) of 70 (55 - 80), followed by ‘emotional functioning’ with a median score of 75 (65 - 85). The SIP scores were comparable to normative values, for all age and gender groups. Conclusion: Children with CAH have increased prevalence of growth and weight gain problems, metabolic co-morbidities, as well as reduced quality of life and mental wellbeing. There is a pressing need to optimize management and monitoring strategies in CYP with CAH order to improve long-term health outcomes.

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