title: Salivary morning androstenedione and 17α-OH progesterone levels in childhood and puberty in patients with classic congenital adrenal hyperplasia.
abstract: BACKGROUND
  Treatment of congenital adrenal hyperplasia due to 21-hydroxylase deficiency can be monitored by salivary androstenedione (A-dione) and 17α-hydroxyprogesterone (17OHP) levels. There are no objective criteria for setting relevant target values or data on changes of 17OHP and A-dione during monitoring.
  
  
  METHODS
  We evaluated A-dione and 17OHP levels in nearly 2000 salivary samples collected during long-term treatment of 84 paediatric patients with classic 21-hydroxylase deficiency.
  
  
  RESULTS
  A-dione and 17OHP levels and its ratio 17OHP/A-dione remained constant from 4 to 11 years with no sex-related differences. During puberty, A-dione and 17OHP levels both increased, starting at earlier age in girls than in boys. The ratio 17OHP/A-dione declined. Normalised A-dione concomitant with elevated 17OHP [1.43 nmol/L (0.46-4.41) during prepuberty; 2.36 nmol/L (0.63-8.89) for boys and 1.99 nmol/L (0.32-6.98) for girls during puberty] could be obtained with overall median glucocorticoid doses of 11-15 mg/m2/day. A-dione levels above the upper reference limit (URL), suggesting undertreatment, coincided with 17OHP levels ≥10 times URL. The percentage of A-dione levels above URL was 16% at ages 4-8 years, but increased to 31% for girls at 16 years and 46% for boys at 17 years.
  
  
  CONCLUSIONS
  Normalised A-dione consistent with 17OHP three times URL during prepuberty and normalised A-dione consistent with 4-6 times URL during puberty could be obtained by moderate glucocorticoid dosages. A constant 17OHP/A-dione ratio during prepuberty suggested absence of adrenarche. During puberty, a higher percentage of samples met the criteria for undertreatment, especially of boys.
authors.forename: Monique J M
authors.initials: MJ
authors.lastname: de Groot
authors.forename: Karijn J
authors.initials: KJ
authors.lastname: Pijnenburg-Kleizen
authors.forename: Chris M G
authors.initials: CM
authors.lastname: Thomas
authors.forename: Fred C G J
authors.initials: FC
authors.lastname: Sweep
authors.forename: Nike M M L
authors.initials: NM
authors.lastname: Stikkelbroeck
authors.forename: Barto J
authors.initials: BJ
authors.lastname: Otten
authors.forename: Hedi L
authors.initials: HL
authors.lastname: Claahsen-van der Grinten
mesh_terms: D019326:17-alpha-Hydroxyprogesterone / Q000032:analysis*; D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000188:drug therapy / Q000378:metabolism*; D000735:Androstenedione / Q000032:analysis*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005938:Glucocorticoids / Q000494:pharmacology / Q000627:therapeutic use; D006801:Humans; D008297:Male; D011627:Puberty / Q000378:metabolism*; D012189:Retrospective Studies; D012463:Saliva / Q000737:chemistry* / Q000187:drug effects
chemical_list: D005938:Glucocorticoids; D000735:Androstenedione; D019326:17-alpha-Hydroxyprogesterone