Results of Treatment in 108 Patients with Cushing's Syndrome

Follow-up study on treatment in 27 patients with Cushing's disease: Adrenalectomy, transsphenoidal adenomectomy and medical treatment.

“…Subtotal or unilateral adrenalectomy as surgical treatment for Cushing's disease was unsatisfactory, as demonstrated in our early cases and also as reported by others (Orth and Liddle, 1971). Since then, we had performed total bilateral adrenalectomy as a major treatment for this disease but it is well known that this procedure has several disadvantages, such as extensive surgery, lifelong cortisol substitution therapy postoperative and the subsequent development of Nelson's syndrome (Nelson et al, 1960).…”

Metyrapone in long-term management of Cushing's disease.

“…2 This ability to lower the level of circulating corticosteroids led to its being adopted in two main diagnostic clinical roles: as a test of ACTH reserve' 3-7 and in the differential diagnosis of Cushing's syndrome.3 7 Reports of its use in treatment have been few and virtually confined to the treatment of adrenal tumours3 8 and the ectopic ACTH syndrome.9 10 It has been claimed that the compensatory rise in plasma ACTH that occurs with the use of adrenocortical enzyme blocking agents in patients with pituitary-dependent Cushing's disease would effectively overcome the blockade.1" 12 Our object was to show that long-term control of patients with pituitary-dependent disease is possible. We elected to treat all patients with oral metyrapone and to give them also a course of external pituitary irradiation (unless contraindicated).…”

Medical management of Cushing disease

“…31 Of note, however, the criterion for "cortisol control" in the latter study was a mean serum level less than 400 nmol/L. When the more accepted, stringent criterion of Orth and Liddle 21 is applied (280 nmol/L cutoff), only 19% of patients experienced an effective short-term response. Overall, Orth 20 concluded that metyrapone is only useful as adjunctive treatment for Cushing disease.…”