Periodic remission in Cushing's disease with paradoxical dexamethasone response: an expression of periodic hormonogenesis.
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.
D015065:17-Hydroxycorticosteroids / Q000652:urine; D015068:17-Ketosteroids / Q000652:urine; D000324:Adrenocorticotropic Hormone; D000328:Adult; D003366:Cosyntropin; D003480:Cushing Syndrome / Q000188:drug therapy / Q000378:metabolism / Q000503:physiopathology*; D003907:Dexamethasone / Q000627:therapeutic use*; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D007016:Hypophysectomy; D008236:Lypressin; D008297:Male; D008797:Metyrapone; D010507:Periodicity; D012075:Remission, Spontaneous
D015065:17-Hydroxycorticosteroids; D015068:17-Ketosteroids; D003366:Cosyntropin; D008236:Lypressin; D003907:Dexamethasone; D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone; D008797:Metyrapone