title: Adrenarche and puberty in children with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency.
abstract: There have been only a few studies on adrenarche in girls with classic congenital adrenal hyperplasia (CAH) showing that dehydroepiandrosterone sulfate (DHEAS) levels did not rise at the physiological age of adrenarche.
  
  
  OBJECTIVE
  Longitudinal analysis of serum DHEAS levels and Tanner stages in CAH children.
  
  
  METHODS
  We studied 98 CAH patients (52 females), aged between 1 month and 18.0 years. All patients had genetically proven classic CAH and received steroid substitution therapy.
  
  
  RESULTS
  Serum DHEAS levels did not differ between CAH children and healthy children from the age of 1 year until 5-6 years. Beginning at the age of 7-8 years, there was a continuous but blunted increase in DHEAS levels in CAH boys and girls compared to healthy children. There was no correlation of DHEAS levels with the genotype, glucocorticoid dosage, auxological data, or quality of metabolic control. Pubarche (PH2) as well as gonadarche (G2) and thelarche (B2) occurred significantly earlier in CAH boys and girls than in the reference group, but timing of menarche was normal.
  
  
  CONCLUSIONS
  Pubarche and adrenarche are dissociated in classic CAH: earlier pubarche, gonadarche and thelarche, respectively, in both sexes contrast with the absence of typical adrenarche.
authors: Division of Pediatric Endocrinology and Diabetology, Department of Pediatrics and Adolescent Medicine, Friedrich Alexander University of Erlangen-Nuremberg, Erlangen-Nuremberg, Germany.
authors: Thomas M K
authors: TM
authors: Völkl
authors: Lisa
authors: L
authors: Öhl
authors: Manfred
authors: M
authors: Rauh
authors: Christof
authors: C
authors: Schöfl
authors: Helmuth G
authors: HG
authors: Dörr
mesh_terms: D000293:Adolescent; D041923:Adolescent Development / Q000187:drug effects; D000312:Adrenal Hyperplasia, Congenital / Q000097:blood / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology*; D050499:Adrenarche / Q000187:drug effects; D002648:Child; D002657:Child Development / Q000187:drug effects; D002675:Child, Preschool; D015331:Cohort Studies; D019314:Dehydroepiandrosterone Sulfate / Q000097:blood; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008137:Longitudinal Studies; D008297:Male; D042361:Mammary Glands, Human / Q000187:drug effects / Q000473:pathology; D008499:Medical Records; D009929:Organ Size / Q000187:drug effects; D011627:Puberty / Q000187:drug effects; D012189:Retrospective Studies
chemical_list: D005938:Glucocorticoids; D019314:Dehydroepiandrosterone Sulfate