Abstract. Mild Cushing's syndrome was diagnosed in a 35 year old woman. Elevated plasma and urinary cortisol levels were unsuppressible with up to 32 mg dexamethasone per day. Aldosterone, 18-OH-corticosterone and testosterone in plasma were normal and dehydroepiandrosterone-sulphate was low. No adrenal tumour was found by CT or adrenal venography, and bilateral cortisol secretion was demonstrated by steroid measurements in adrenal venous blood. A circadian rhythm of plasma cortisol was absent. Plasma ACTH was suppressed, even after injection of CRH, during insulininduced hypoglycaemia and after metyrapone administration, which led to a large fall in plasma cortisol but to a subnormal rise of plasma 11-deoxy-cortisol. The clinical diagnosis of primary micronodular adenomatosis of the adrenal gland was histologically confirmed, when the patient finally underwent bilateral adrenalectomy. In vitro, the adrenal cells did not produce more cortisol and aldosterone than adrenal cells from cadaver kidney donors. In vivo and in vitro, cortisol was slightly less than normally responsive to ACTH. Intermittent treatment of the patient with 800 mg/day of ketoconazole led to a rapid fall of cortisol secretion and clinical signs of adrenocortical insufficiency. Treatment for 7 weeks with 200–400 mg ketoconazole per day reduced plasma and urinary cortisol less dramatically into the normal range. This case unequivocally documents autonomous dysfunction of the adrenal cortex in this rare form of Cushing's syndrome and the efficacy of ketoconazole in the treatment of ACTH-independent hypercortisolism.

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The diurnal variations of the plasma concentrations of eleven steroid hormones and of corticotropin (ACTH) were studied in ten young healthy males. The plasma steroids progesterone, pregnenolone, deoxycorticosterone, 17-OH-progesterone, 17-OH-pregnenolone, deoxycortisol, 18-OH-deoxycorticosterone, corticosterone, aldosterone, cortisol and 18-OH-corticosterone, as well as plasma ACTH, were measured at 30-min intervals in the morning and in the evening and at 2-h intervals during the rest of the day. Steroids were extracted from 1 ml plasma, fractionated by high-pressure liquid chromatography (HPLC) and finally quantified by radioimmunoassay (RIA). Plasma concentrations of ACTH were radioimmunoassayed after extraction from 2 ml plasma. More or less pronounced circadian and episodic variations were apparent for plasma levels of all steroids studied, as well as of ACTH. According to related profiles of diurnal variations of plasma concentrations, three different categories of steroids were tentatively crystallized. Category 1 includes 17-OH-pregnenolone, deoxycortisol, corticosterone, 18-OH-deoxycorticosterone, deoxycorticosterone, cortisol and 18-OH-corticosterone, exhibiting a rhythm partly synchronous with that of the pituitary secretory activity of ACTH. Category 2, including progesterone, pregnenolone and 17-OH-progesterone, exhibited a time course of plasma concentrations assuming a regulation predominantly dictated by the testicular secretory activity. Lastly, aldosterone exerted a variation of plasma concentrations which was obviously regulated by the renin-angiotensin system under the present conditions.