ePrints Repository

Health status of adults with congenital adrenal hyperplasia: a cohort study of 203 patients

Arlt, Wiebke and Willis, Debbie S and Wild, Sarah H and Krone, Nils and Doherty, Emma J and Hahner, Stefanie and Han, Thang S and Carroll, Paul V and Conway, G. S. and Rees, D Aled and Stimson, Roland H and Walker, Brian R and Connell, John M C and Ross, Richard J (2010) Health status of adults with congenital adrenal hyperplasia: a cohort study of 203 patients. The Journal of clinical endocrinology and metabolism, 95 (11). pp. 5110-5121. ISSN 1945-7197

Loading
PDF (1257Kb)

URL of Published Version: http://jcem.endojournals.org/content/95/11/5110.abstract?sid=3a2ed353-d6b9-47f1-bd80-bea8c7cf9349

Identification Number/DOI: 10.1210/jc.2010-0917

Context: No consensus exists for management of adults with congenital adrenal hyperplasia (CAH) due to a paucity of data from cohorts of meaningful size. Objective: Our objective was to establish the health status of adults with CAH. Design and Setting: We conducted a prospective cross-sectional study of adults with CAH attending specialized endocrine centers across the United Kingdom. Patients: Participants included 203 CAH patients (199 with 21-hydroxylase deficiency): 138 women, 65 men, median age 34 (range 18-69) years. Main Outcome Measures: Anthropometric, metabolic, and subjective health status was evaluated. Anthropometric measurements were compared with Health Survey for England data, and psychometric data were compared with appropriate reference cohorts. Results: Glucocorticoid treatment consisted of hydrocortisone (26%), prednisolone (43%), dexamethasone (19%), or a combination (10%), with reverse circadian administration in 41% of patients. Control of androgens was highly variable with a normal serum androstenedione found in only 36% of patients, whereas 38% had suppressed levels suggesting glucocorticoid overtreatment. In comparison with Health Survey for England participants, CAH patients were significantly shorter and had a higher body mass index, and women with classic CAH had increased diastolic blood pressure. Metabolic abnormalities were common, including obesity (41%), hypercholesterolemia (46%), insulin resistance (29%), osteopenia (40%), and osteoporosis (7%). Subjective health status was significantly impaired and fertility compromised. Conclusions: Currently, a minority of adult United Kingdom CAH patients appear to be under endocrine specialist care. In the patients studied, glucocorticoid replacement was generally nonphysiological, and androgen levels were poorly controlled. This was associated with an adverse metabolic profile and impaired fertility and quality of life. Improvements in the clinical management of adults with CAH are required.

Type of Work:Article
Date:2010 (Publication)
School/Faculty:Colleges (2008 onwards) > College of Medical & Dental Sciences
Department:School of Clinical and Experimental Medicine
Subjects:RC Internal medicine
RA Public aspects of medicine
Institution:University of Birmingham
Copyright Holders:The Endocrine Society
ID Code:507
Refereed:YES
Local Holdings:
Export Reference As : ASCII + BibTeX + Dublin Core + EndNote + HTML + METS + MODS + OpenURL Object + Reference Manager + Refer + RefWorks
Share this item :
QR Code for this page

Repository Staff Only: item control page