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An adrenal tumor was discovered fortuitously in a patient with no clinical features of Cushing's syndrome. On adrenal imaging, there was good uptake in the nodule but no visualization of the contralateral adrenal. The latter was seen, however, in a second scan performed under ACTH treatment. In the hormone assessment, basal cortisol and 17-hydroxycorticoids were normal and cortisol diurnal variation was near normal, but a dexamethasone suppression test and ACTH responses to metyrapone and insulin hypoglycemia were abnormal. Eight months after excision of a spongiocytic-type adenoma, the remaining adrenal was visible on scintigram and the hormonal tests were normal. This pattern suggests that the clinical Cushing's syndrome but enough to suppress partially ACTH and, consequently, visualization of the contralateral gland.

A case of Cushing's disease in a ten year old girl with bilateral diffuse hyperplasia of the adrenal cortex and postoperative enlargment of the sella turcica is presented. The administration of dexamethasone elicited a paradoxical response with a clear elevation of the already high excretion of 17-hydroxycorticoids. The possible mechanisms for this previously described, but infrequent, response to dexamethasone are discussed. It is concluded that: 1) Interpretation of the classical dexamethasone suppression test can occasionally be misleading; and 2) Periodic hormonogenesis may account for this type of paradoxical response.

Sixteen consecutive cases of Cushing's disease were treated by radical transphenoidal hypophysectomy. Surgical removal was not possible in two cases. Of the other 14, there was total clinical and biochemical remission of the disease in 13 and partial remission in one. Some form of replacement therapy has been necessary in 11 out of the 14 cases. During a long period of postoperative clinical and biochemical assessment extending over 5-14 years (average 8 years) there have been no recurrences.

A 47-year-old woman affected by Cushing's syndrome due to an adrenal adenoma is described. An altered but rhythmometrically apparent cortisol secretory rhythm was detected using the single-cosinor computation. In fact serum cortisol levels and urinary excretion of 17-OHCS were elevated in the PM hours, particularly between 14:00-18.00 h and 18:00-22:00 h, and normal between 02:00-10:00 h. The patient was cured by unilateral adrenalectomy and one year later the circadian rhythm of corticosteroids secretion was investigated again. A normal rhythm of cortisol secretion and of 17-OHCS urinary excretion was found. Though it may be hypothesized that factors intrinsic to the tumoral adrenal cells were responsible for the rhythmic, but phase-shifted, hormonal release, the cause of the persistent and abnormal cortisol secretory rhythm is unknown.

Aseptic necrosis of bone is a known complication of corticosteroid therapy. We report a case of aseptic necrosis of the femoral head as a presenting symptom of Cushing's disease. The case demonstrates that endogenous steroids, like exogenous steroids, may bear the same relationship to the pathogenesis of aseptic necrosis of bone. It also indicates that Cushing's disease must be considered in every case of so-called idiopathic aseptic necrosis of bone.

A study was made of the content of cortisol, corticosterone, 11-deoxycortisol, estradiol, testosterone and progesterone in the blood plasma using radioimmunoassay and of the content of these hormones or their main metabolites in the daily urine using physicochemical methods in 77 patients with an active stage of the Icenko-Cushing disease. An acute increase in cortisol secretion, a noticeable rise of progesterone secretion, and significant deviations in testosterone production having opposite nature in men and women were established: in men androgen content in the body was lowered, in women raised. The adrenals were shown to be the main site of testosterone and progesterone secretion in female patients. One-type disorders of the rhythm of secretion of hormones determined during one day (disappearance or reduction of daily variations) were revealed.

In a group of 22 unselected patients with Cushing's disease 3 had a moderately enlarged pituitary fossa, while 6 patients showed asymmetry of the sella turcica at lateral tomography. This group of 9 patients with radiologically evident pituitary adenomas had:i) a higher average basal urinary excretion of 17-hydroxycorticosteroids which showed a greater day to day variation;ii) a greater (although insufficient) decrease of plasma cortisol in response to a single oral dose of 1 mg dexamethasone and larger postdexamethasone rebound excretion of urinary 17-hydroxycorticosteroids after 8 mg dexamethasone daily for two days; and iii) a larger maximal increase of plasma cortisol after lysine vasopressin administration than the other 13 patients. The increase in the size of a pituitary (micro-) adenoma in patients with Cushing's disease is accompanied by a more severe hypercortisolism and an increased responsiveness of the pituitary-adrenal axis to the manipulations mentioned.

The results of study of the hypothalamo-hypophysio-adrenal system in patients with Itsenko-Cushing's disease under the influence of high reserpine doses are presented. An increase of plasma ACTH and cortizol level and disturbance of the 24-hour rhythm of their secretion was noted in the patients. High reserpine doses led to the fall of ACTH and cortizol secretion, without any normalization of their secretion rhythm in the course of 24 hours. A test with metopyron carried out before and after the reserpine prescription pointed to the increase of the ACTH reserve under the influence of high reserpine doses.

A 36-year-old female was admitted to Asahi General Hospital because of hypertension. Laboratory findings showed elevated levels of urine 17 (11)-hydroxycorticosteroid and blood cortisol. Computerized tomography and ultrasound sonography demonstrated a round tumor at the suprarenal region. The black tumor, 5 X 3 X 2 cm, was removed by left adrenalectomy. Microscopic findings revealed adrenal adenoma consisting of compact cells containing numerous pigmented granules. Postoperative course was uneventful. Hormonal condition was recovered 11 months after operation.