Dissociation between plasma adrenal androgens and cortisol in Cushing's disease and ectopic ACTH-producing tumour: relation to adrenarche.
To assess the effect of chronic hypersecretion of corticotropin (ACTH) and other peptides derived from proopiomelanocortin , and of cortisol, on plasma adrenal androgen concentration, plasma dehydroepiandrosterone sulphate (DHEA-S), dehydroepiandrosterone (DHEA), androstenedione (delta 4 A), and cortisol were measured in 14 children and adolescents with Cushing's disease, a 9-year-old boy with an ectopic ACTH-producing tumour, and a group of normal, age-related individuals. The plasma DHEA-S concentration was normal for chronological age in 9 of 12 patients and for bone age in 7 of 10 patients. The plasma DHEA level was normal for chronological age in 12 of 14 patients and for bone age in 8 of 10 patients. In contrast, the concentration of plasma delta 4 A was raised for chronological age in 6 of 13 patients and for bone age in 7 of 10 patients. All patients had raised plasma cortisol levels in the afternoon and other laboratory and clinical signs of hypercortisolism. In the boy with an ectopic ACTH-producing tumour, plasma DHEA-S was moderately raised, plasma DHEA was normal, and plasma delta 4 A was very high. This patient's plasma ACTH levels ranged from 1340 to 1520 pg/ml and the cortisol levels from 51 to 95 micrograms/dl. The findings suggest that a factor other than ACTH is also required for adrenal androgen secretion. Since the other proopiomelanocortin -related peptides--ie, the N-terminal peptide (1-76), beta-endorphin (beta-EP), beta-lipotropin (beta-LPH), and gamma-lipotropin (gamma-LPH)--are raised in the plasma of patients with Cushing's disease, one of these is unlikely to be that putative factor.
D000182:ACTH Syndrome, Ectopic / Q000097:blood* / Q000503:physiopathology; D007516:Adenoma, Islet Cell / Q000097:blood / Q000378:metabolism; D000293:Adolescent; D000302:Adrenal Cortex / Q000378:metabolism*; D000324:Adrenocorticotropic Hormone / Q000097:blood; D000365:Age Determination by Skeleton; D000728:Androgens / Q000097:blood*; D000735:Androstenedione / Q000097:blood; D002648:Child; D003480:Cushing Syndrome / Q000097:blood* / Q000503:physiopathology; D003687:Dehydroepiandrosterone / Q000031:analogs & derivatives / Q000097:blood; D019314:Dehydroepiandrosterone Sulfate; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000097:blood* / Q000378:metabolism; D008297:Male; D010190:Pancreatic Neoplasms / Q000097:blood / Q000378:metabolism; D009384:Paraneoplastic Endocrine Syndromes / Q000097:blood*
D000728:Androgens; D000735:Androstenedione; D003687:Dehydroepiandrosterone; D019314:Dehydroepiandrosterone Sulfate; D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone