title: Assessment of early atherosclerosis and left ventricular dysfunction in children with 21-hydroxylase deficiency.
abstract: OBJECTIVE
  We analysed 25 children with 21-hydroxylase deficiency who received glucocorticoid and/or mineralocorticoid treatment for at least 12 months to determine the effects of the disease and its treatment on vascular structures and ventricular function.
  
  
  METHODS
  Twenty-five patients with 21-hydroxylase-deficient congenital adrenal hyperplasia (CAH) and 25 control subjects were enrolled into this observational, cross-sectional study. The patients were investigated in terms of fasting blood glucose and insulin; fasting serum lipid profile; serum 17-hydroxyprogesterone; dehydroepiandrosterone sulphate; androstenedione; and adrenocorticotropic hormone. M-mode tracings of the wall motion of major arteries were obtained to measure carotid intima-media thickness (cIMT), as well as elasticity and distensibility of the aorta and carotid artery. Conventional and relatively new tissue Doppler imaging techniques were employed to assess ventricular systolic and diastolic functions.
  
  
  RESULTS
  The median age and weight of patients were 9·4 years (1·5-16·75) and 35·5 kg (7·5-76·3), respectively. The median duration of treatment was 52·2 months. Tissue Doppler imaging measurements revealed left ventricular diastolic impairment in the patient group compared to the controls. Carotid intima-media thickness, stiffness index, elastic modulus of the aorta and carotid artery were significantly higher; meanwhile, aortic distensibility and carotid distensibility were lower in the patient group, all of which indicates the presence of subclinical atherosclerosis. BMI was found to be an independent variable for cIMT (ß: 0·5, P = 0·01) and aortic stiffness index (ß: 0·52, P < 0·001).
  
  
  CONCLUSIONS
  Cardiovascular function and the elastic properties of major arteries are disturbed in children and adolescents with 21-hydroxylase-deficient CAH.
authors.affiliation: Division of Pediatric Cardiology, Izmir Dr. Behcet Uz Children's Hospital, Izmir, Turkey.
authors.forename: Rahmi
authors.initials: R
authors.lastname: Özdemir
authors.affiliation: Division of Pediatric Endocrinology, Izmir Dr. Behcet Uz Children's Hospital, Izmir, Turkey.
authors.forename: Hüseyin Anil
authors.initials: HA
authors.lastname: Korkmaz
authors.affiliation: Division of Pediatric Cardiology, Izmir Dr. Behcet Uz Children's Hospital, Izmir, Turkey.
authors.forename: Mehmet
authors.initials: M
authors.lastname: Küçük
authors.affiliation: Division of Pediatric Cardiology, Izmir Dr. Behcet Uz Children's Hospital, Izmir, Turkey.
authors.forename: Cem
authors.initials: C
authors.lastname: Karadeniz
authors.affiliation: Division of Pediatric Cardiology, Izmir Dr. Behcet Uz Children's Hospital, Izmir, Turkey.
authors.forename: Timur
authors.initials: T
authors.lastname: Meşe
authors.affiliation: Division of Pediatric Endocrinology, Izmir Dr. Behcet Uz Children's Hospital, Izmir, Turkey.
authors.forename: Behzat
authors.initials: B
authors.lastname: Özkan
mesh_terms: D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology*; D001011:Aorta / Q000503:physiopathology; D050197:Atherosclerosis / Q000175:diagnosis* / Q000209:etiology; D002339:Carotid Arteries / Q000503:physiopathology; D059168:Carotid Intima-Media Thickness; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D004548:Elasticity; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D018570:Risk Assessment; D059289:Vascular Stiffness; D018487:Ventricular Dysfunction, Left / Q000175:diagnosis* / Q000209:etiology