title: 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).
abstract: BACKGROUND
  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 aged 18-69 years in whom QoL (assessed using the Short Form Health Survey), 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 monotherapy (P<0.05). QoL did not relate to PreDEq or mutation severity. PCA identified three PCs (PC1, disease control; PC2, adiposity and insulin resistance and PC3, blood pressure and mutations) that explained 61% of the variance in observed variables. Stepwise multiple regression analysis demonstrated that PC2, reflecting adiposity and insulin resistance (waist circumference, serum triglycerides, homeostasis model assessment of insulin resistance and HDL-cholesterol), related to 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. Intervention trials are required to establish whether choice of glucocorticoid treatment and/or weight loss can improve QoL in CAH adults.
authors.affiliation: Department of Endocrinology, University College London Hospitals, London, UK.
authors.forename: Thang S
authors.initials: TS
authors.lastname: Han
authors.forename: Nils
authors.initials: N
authors.lastname: Krone
authors.forename: Debbie S
authors.initials: DS
authors.lastname: Willis
authors.forename: Gerard S
authors.initials: GS
authors.lastname: Conway
authors.forename: Stefanie
authors.initials: S
authors.lastname: Hahner
authors.forename: D Aled
authors.initials: DA
authors.lastname: Rees
authors.forename: Roland H
authors.initials: RH
authors.lastname: Stimson
authors.forename: Brian R
authors.initials: BR
authors.lastname: Walker
authors.forename: Wiebke
authors.initials: W
authors.lastname: Arlt
authors.forename: Richard J
authors.initials: RJ
authors.lastname: Ross
mesh_terms: D050154:Adiposity / Q000502:physiology*; D000312:Adrenal Hyperplasia, Congenital / Q000188:drug therapy* / Q000378:metabolism / Q000503:physiopathology; D000328:Adult; D000368:Aged; D003430:Cross-Sectional Studies; D003907:Dexamethasone / Q000627:therapeutic use; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D007333:Insulin Resistance / Q000502:physiology*; D008297:Male; D008875:Middle Aged; D011788:Quality of Life; D006113:United Kingdom; D055815:Young Adult
chemical_list: D005938:Glucocorticoids; D003907:Dexamethasone