Primary cortisol resistance: a family study

J Clin Endocrinol Metab. 1983 Jun;56(6):1243-5. doi: 10.1210/jcem-56-6-1243.


Primary cortisol resistance is an autosomal disease characterized by increased plasma cortisol concentration and high urinary free cortisol, resistance to adrenal suppression by dexamethasone, and the absence of clinical stigmata of Cushing's syndrome. The proband with the severe form had hypertension and hypokalemic alkalosis. In subjects with a less severe resistance to cortisol, there are no clinical abnormalities and the condition is revealed only by detailed examination of several parameters of cortisol secretion.

MeSH terms

  • Adrenocortical Hyperfunction / blood
  • Adrenocortical Hyperfunction / drug therapy
  • Adrenocortical Hyperfunction / genetics*
  • Dexamethasone / therapeutic use
  • Drug Resistance
  • Female
  • Humans
  • Hydrocortisone / blood
  • Hydrocortisone / urine*
  • Male
  • Pedigree


  • Dexamethasone
  • Hydrocortisone