FP03-1 Quality of Life in Adults With Congenital Adrenal Hyperplasia Relates to Glucocorticoid Treatment, Adiposity and Insulin Resistance: United Kingdom Congenital Adrenal Hyperplasia Adult Study Executive (CaHASE)

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: FP03-Glucocorticoids & Glucocorticoid Actions
Saturday, June 15, 2013: 11:00 AM-11:30 AM
Presentation Start Time: 11:00 AM
Room 130 (Moscone Center)

Poster Board SAT-1
Thang S Han1, Nils Krone2, Debbie S Willis3, Gerard Stephen Conway4, Stephanie Hahner5, Aled Rees6, Roland H Stimson7, Brian R Walker8, Wiebke Arlt9 and Richard J. Ross*10
1University College London, London, United Kingdom, 2CEDAM Ctr for Endo, Diab and Met, Birmingham, United Kingdom, 3British Endocrine Society, 4Department of Endocrinology, London, United Kingdom, 5Univ Hosp of Wuerzburg, Wurzburg, Germany, 6Cardiff University, Cardiff, United Kingdom, 7Univ of Edinburgh, Edinburgh Scotland, United Kingdom, 8University of Edinburgh, Edinburgh, United Kingdom, 9University of Birmingham, Birmingham, United Kingdom, 10Univ of Sheffield, Sheffield, United Kingdom
Context: Quality of life (QoL) has been variously reported as normal or impaired in adults with congenital adrenal hyperplasia (CAH). To explore the reasons for this discrepancy we investigated the relationship between QoL, glucocorticoid treatment and other health outcomes in CAH adults.

Methods: Cross-sectional analysis of 151 adults with 21-hydroxylase deficiency (50M: 47 with classic and 3 with non-classic CAH; 101F: 75 with classic and 26 with non-classic CAH) aged 18-69 years in whom QoL (SF-36), glucocorticoid regimen, anthropometric and metabolic measures were recorded. Relationships were examined between QoL, type of glucocorticoid (hydrocortisone, prednisolone and dexamethasone), and dose of glucocorticoid expressed as prednisolone dose equivalent (PreDEq). QoL was expressed as z-scores calculated from matched controls (14,430 subjects from UK population). Principal components analysis (PCA) was undertaken to identify clusters of associated clinical and biochemical features and the principal component (PC) scores used in regression analysis as predictor of QoL.

Results: QoL scores were associated with type of glucocorticoid treatment for vitality (P=0.002) and mental health (P=0.011), with higher z-scores indicating better QoL in patients on hydrocortisone than in patients receiving prednisolone or dexamethasone (P<0.05). QoL did not relate to PreDEq or mutation severity. PCA identified three PCs (PC1, disease control; PC2, adiposity and insulin resistance; PC3, blood pressure and mutations) that explained 61% of the variance in observed variables. Stepwise multiple regression analysis demonstrated that PC2 (comprising waist circumference, serum triglycerides, HOMA-IR and HDL-cholesterol) was associated with QoL scores, specifically impaired physical functioning, bodily pain, general health, Physical Component Summary Score (P<0.001) and vitality (P=0.002).

Conclusions: Increased adiposity, insulin resistance and use of prednisolone or dexamethasone are associated with impaired QoL in adults with CAH independently of mutation severity. Intervention trials are required to establish whether choice of glucocorticoid treatment and/or weight loss can improve QoL in CAH adults.

Disclosure: SH: Advisory Group Member, Viropharma. RJR: Founder, Diurnal. Nothing to Disclose: TSH, NK, DSW, GSC, AR, RHS, BRW, WA

*Please take note of The Endocrine Society's News Embargo Policy at http://www.endo-society.org/endo2013/media.cfm

Sources of Research Support: CaHASE are grateful to The Clinical Endocrinology Trust for its financial support and the Society for Endocrinology for management of the project. R.J.R. is a founding director and equity holder in Diurnal Ltd that is developing new hydrocortisone preparations for patients with CAH.
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