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CUSHING'S SYNDROME BY ECTOPIC ACTH SECRETION: EFFECT OF MIFEPRISTONE

1986; Springer Nature; Volume: 20; Issue: 11 Linguagem: Inglês

10.1203/00006450-198611000-00174

1530-0447

B. Beaufrère, Pierre Chatelain, G. Sassolas, Yves Morel, N Catbeh, M Aguercif, F Bey-Omar, François Rassendren,

Hormonal Regulation and Hypertension

Mifepristone (RU 486 - Roussel-Udlaf), an antiglucorticoid receptor with no agonist activity, has been proposed as treatment of hyper-cortisolism in adults. A 27 month old girl presented a Cushing's syndrome, ongoing for the past 12 months. She exhibited obesity, delayed growth, moon facies and severe hypertension (200 mm Hg). Urinary free cortisol (UFC) (6275 nmol/day), plasma cortisol (C) (2435 nmol/ml) and ACTH (2016 pg/ml) were elevated and did not respond to dexamethasone suppression test. Plasma CRF was undetectable. Cranial CT scans were normal. The very high ACTH levels were consistent with an ectopic ACTH secretion although its origin could not be located on repeated extensive investigations. RU 486 was administered for 2 months at increasing dosages (5 to 25 mo/kg.d) The patient improved dramatically, lost 3 kg and normalized her blood pressure. UFC (118 nmol/day), C (595 nM) and ACTH (233pg/ml) all decreased. No side effect was observed. Six weeks after discontinuation of RU 486, there was no clinical evidence of relapse, and UFC (147 nmol/day), C (418 nmol/ml) and ACTH (397 pg/ml) were unchanged. Although spontaneous cure or intermittent ACTH secretion cannot be excluded, this observation could suggest a therapeutic effect of RU 486 on ectopic ACTH secretion. However, its mechanism remains unclear since we observed a fall of cortisol and ACTH, contrary to what has been previously described in adult Cushing's syndrome treated with RU 486.