Coexistence of 21-hydroxylase and 11 beta-hydroxylase deficiency in adrenal incidentalomas and in subclinical Cushing's syndrome

Horm Res. 2002;57(5-6):192-6. doi: 10.1159/000058381.

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • 17-alpha-Hydroxyprogesterone / blood
  • Adenoma / enzymology*
  • Adrenal Gland Neoplasms / enzymology*
  • Adrenal Hyperplasia, Congenital*
  • Adrenocorticotropic Hormone
  • Adult
  • Aged
  • Cortodoxone / blood
  • Cushing Syndrome / enzymology*
  • Female
  • Humans
  • Hydrocortisone / blood
  • Male
  • Middle Aged

Substances

  • 17-alpha-Hydroxyprogesterone
  • Adrenocorticotropic Hormone
  • Cortodoxone
  • Hydrocortisone