title: Coexistence of 21-hydroxylase and 11 beta-hydroxylase deficiency in adrenal incidentalomas and in subclinical Cushing's syndrome.
abstract: OBJECTIVE
  The prevalence of steroid secretion abnormalities was studied by evaluating the 17-hydroxyprogesterone (17-OHP) and 11-deoxycortisol (S) responses to adrenocorticotropic hormone (ACTH) stimulation in 48 patients with 'nonfunctioning' incidentalomas and in 10 patients with 'subclinical' Cushing's syndrome.
  
  
  METHODS
  In all patients the cortisol, 17-OHP, and S levels were measured after ACTH test. Eight patients were reinvestigated after surgery.
  
  
  RESULTS
  In patients with nonfunctioning lesions, the ACTH test induced 17-OHP and S peaks higher than in normals (p < 0.005). In 10 cases an augmented rise of 17-OHP and S was observed. In patients with subclinical Cushing's syndrome, the 17-OHP peak after ACTH was greater than in patients with nonfunctioning lesions and in normals (p < 0.005); the S peak was also higher than in controls (p < 0.005). In 7 of 8 operated patients, the exaggerated 17-OHP peak was normalized.
  
  
  CONCLUSIONS
  A combined impairment of different enzyme activities is frequently present in adrenal incidentalomas; the alteration of enzymatic pathways can also coexist with the presence of partial cortisol autonomy.
authors.affiliation: Institute of Endocrine Sciences, Ospedale Maggiore IRCCS, Milan, Italy.
authors.forename: Chiara
authors.initials: C
authors.lastname: Dall'Asta
authors.forename: Laura
authors.initials: L
authors.lastname: Barbetta
authors.forename: Rossella
authors.initials: R
authors.lastname: Libé
authors.forename: Elena
authors.initials: E
authors.lastname: Passini
authors.forename: Bruno
authors.initials: B
authors.lastname: Ambrosi
mesh_terms: D019326:17-alpha-Hydroxyprogesterone / Q000097:blood; D000236:Adenoma / Q000201:enzymology*; D000310:Adrenal Gland Neoplasms / Q000201:enzymology*; D000312:Adrenal Hyperplasia, Congenital; D000324:Adrenocorticotropic Hormone; D000328:Adult; D000368:Aged; D003350:Cortodoxone / Q000097:blood; D003480:Cushing Syndrome / Q000201:enzymology*; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D008297:Male; D008875:Middle Aged
chemical_list: D019326:17-alpha-Hydroxyprogesterone; D000324:Adrenocorticotropic Hormone; D003350:Cortodoxone; D006854:Hydrocortisone