Dissociation between plasma adrenal androgens and cortisol in Cushing's disease and ectopic ACTH-producing tumour: relation to adrenarche.

Abstract

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.

• Authors: B P Hauffa, S L Kaplan, M M Grumbach
• Journal: Lancet (London, England) (Lancet) Vol. 1 Issue 8391 Pg. 1373-6 (Jun 23 1984) ISSN: 0140-6736 [Print] England
• PMID: 6145833 (Publication Type: Case Reports, Comparative Study, Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Androgens
• Androstenedione
• Dehydroepiandrosterone
• Dehydroepiandrosterone Sulfate
• Adrenocorticotropic Hormone
• Hydrocortisone

Topics

• ACTH Syndrome, Ectopic (blood, physiopathology)
• Adenoma, Islet Cell (blood, metabolism)
• Adolescent
• Adrenal Cortex (metabolism)
• Adrenocorticotropic Hormone (blood)
• Age Determination by Skeleton
• Androgens (blood)
• Androstenedione (blood)
• Child
• Cushing Syndrome (blood, physiopathology)
• Dehydroepiandrosterone (analogs & derivatives, blood)
• Dehydroepiandrosterone Sulfate
• Female
• Humans
• Hydrocortisone (blood, metabolism)
• Male
• Pancreatic Neoplasms (blood, metabolism)
• Paraneoplastic Endocrine Syndromes (blood)