Cushing\u27s Disease: Dilemmas of Diagnosis and Management

Determining the cause of Cushing\u27s disease and correcting the abnormality presents a continuing challenge to the clinician despite remarkable advances in diagnostic and therapeutic techniques. We present seven cases to illustrate 1) the classic disorder cured by pituitary adenomectomy: 2) persistence of the disease after adenomectomy: 3) Cushing\u27s disease manifesting in the puerperium and remitting with dopamine agonist therapy: 4) a patient whose disease relapsed at least five times during 20 years of treatment by adrenalectomy, pituitary radiation, mitotane, and pituitary adenomectomy; 5) the Nelson syndrome; 6) the ectopic adrenocorticotropic hormone (ACTH) syndrome in a patient with dexamethasone suppressible urinary cortisol who had a pituitary adenoma which stained positively for ACTH hut who was not cured by total hypophysectomy; and 7) a patient whose ACTH-secreting tumor proved fatal despite repeated surgical, radiologic, and pharmacologic measures

Aldosterone-Producing Adenomas Responsive to Angiotensin Pose Problems in Diagnosis

1. A subgroup of patients with aldosterone‐producing adenoma (APA) have been identified who lack many of the biochemical features regarded as characteristic of APA and used to distinguish APA from bilateral adrenal hyperplasia. 2. In these patients, aldosterone is responsive to infused angiotensin II (angiotensin‐responsive APA), which explains their uncharacteristic responses to upright posture, saline infusion and fludrocortisone acetate administration. 3. The angiotensin‐responsiveness of these patients may derive from the contra‐lateral adrenal gland, since renin levels are less completely suppressed in angiotensin‐responsive APA than in angiotensin‐unresponsive APA. 4. However, while the excretion of 18‐oxo‐cortisol was consistently increased in angiotensin‐unresponsive APA, it was normal in angiotensin‐responsive APA, consistent with biochemical and biosynthetic distinctiveness residing in the tumours. 5. Angiotensin‐responsive APA should always be considered as an alternative diagnosis to bilateral hyperplasia causing primary aldosteronism. Copyrigh

Angiotensin-Responsive Aldosterone-Producing Adenomas - Postoperative Disappearance of Aldosterone Response to Angiotensin

1. Nineteen out of 47 patients (40%) with confirmed unilateral aldosterone-producing adenoma (APA) were responsive to low-dose angiotensin II infusion (AII-R), as defined by an increase in plasma aldosterone concentration of > 50% over basal at 2 ng/ kg per min for 60 min