title: Inadequate cortisol response to the tetracosactide (Synacthen®) test in non-classic congenital adrenal hyperplasia: an exception to the rule?
abstract: OBJECTIVE
  To describe cortisol response to tetracosactide and to review the literature on adrenal function in non-classic congenital adrenal hyperplasia (NCCAH) patients.
  
  
  METHODS
  We compared cortisol responses to tetracosactide (250 μg) between NCCAH patients and a comparison group (CG) of patients with premature pubarche and normal tetracosactide test. An adequate cortisol response was defined as a peak ≥18 μg/dl.
  
  
  RESULTS
  We included 35 NCCAH patients (26 girls, 9 boys), whose mean age at testing was 7.0 years (0.8-15.6), and 47 patients in the CG (39 girls, 8 boys), whose mean age was 7.2 years (0.5-9.9). Baseline cortisol was significantly higher in the NCCAH group than in the CG [12.9 (4.3-22.2) vs. 9.7 (4.2-16.2) μg/dl, respectively; p = 0.0006]. NCCAH patients had lower cortisol peak response compared to the CG [18.2 (6.3-40) vs. 24.9 (12-30.3) μg/dl, respectively; p < 0.0001]. Peak cortisol was <18 μg/dl in 21/35 (60%) NCCAH patients versus 1/47 (2.1%) in the CG. No NCCAH patients had acute adrenal insufficiency, but 2 reported severe fatigue that improved with hydrocortisone.
  
  
  CONCLUSIONS
  The cortisol response to tetracosactide was inadequate (<18 μg/dl) in 60% of patients with NCCAH. Hydrocortisone therapy may deserve consideration when major stress (surgery, trauma, childbirth) or objectively documented fatigue occurs in NCCAH patients with inadequate cortisol response.
authors: Service d'Endocrinologie, Gynécologie et Diabétologie Pédiatriques, Hôpital Universitaire Necker-Enfants Malades, Paris, France.
authors: Athanasia
authors: A
authors: Stoupa
authors: Laura
authors: L
authors: González-Briceño
authors: Graziella
authors: G
authors: Pinto
authors: Dinane
authors: D
authors: Samara-Boustani
authors: Caroline
authors: C
authors: Thalassinos
authors: Isabelle
authors: I
authors: Flechtner
authors: Jacques
authors: J
authors: Beltrand
authors: Maud
authors: M
authors: Bidet
authors: Albane
authors: A
authors: Simon
authors: Marie
authors: M
authors: Piketty
authors: Kathleen
authors: K
authors: Laborde
authors: Yves
authors: Y
authors: Morel
authors: Christine
authors: C
authors: Bellanné-Chantelot
authors: Philippe
authors: P
authors: Touraine
authors: Michel
authors: M
authors: Polak
mesh_terms: D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000097:blood / Q000175:diagnosis*; D002648:Child; D002675:Child, Preschool; D003366:Cosyntropin; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D010912:Pituitary-Adrenal Function Tests; D012189:Retrospective Studies; D012680:Sensitivity and Specificity
chemical_list: D003366:Cosyntropin