Periodic remission in Cushing's disease with paradoxical dexamethasone response: an expression of periodic hormonogenesis.

Abstract

A patient with Cushing's disease due to a chromophobe adenoma was studied for 243 days before pituitary surgery and evidence for periodicity in cortisol steroid production was found with cycles occurring every 85.8 days (peak-to-peak length), associated with laboratory remissions and paradoxical response to dexamethasone. The autonomy of ACTH secretion was suggested by the nonresponsiveness to repeated lysine-vasopressin stimulation tests and lack of increase in urinary 170HCS following metyrapone. A distinct response of the hyperplastic glands (as demonstrated by percutaneous adrenal venography) was obtained on several B1-24 corticotropin stimulation. The patient's hypercortisolism disappeared following removal of the chromophobe adenoma through transphenoidal hypophysectomy.

• Authors: B Liberman, B L Wajchenberg, M A Tambascia, C H Mesquita
• Journal: The Journal of clinical endocrinology and metabolism (J Clin Endocrinol Metab) Vol. 43 Issue 4 Pg. 913-8 (Oct 1976) ISSN: 0021-972X [Print] United States
• PMID: 185234 (Publication Type: Case Reports, Journal Article)

Chemical References

• 17-Hydroxycorticosteroids
• 17-Ketosteroids
• Cosyntropin
• Lypressin
• Dexamethasone
• Adrenocorticotropic Hormone
• Hydrocortisone
• Metyrapone

Topics

• 17-Hydroxycorticosteroids (urine)
• 17-Ketosteroids (urine)
• Adrenocorticotropic Hormone
• Adult
• Cosyntropin
• Cushing Syndrome (drug therapy, metabolism, physiopathology)
• Dexamethasone (therapeutic use)
• Humans
• Hydrocortisone (blood)
• Hypophysectomy
• Lypressin
• Male
• Metyrapone
• Periodicity
• Remission, Spontaneous