山梨医科大学雑誌　第4巻3号　119-124(1989)

ACTH Secretory Mechanism in Congenital Adrenal Hypoplasia. I.
- Responses of Plasma Bioactive and Immunoreactive ACTH
to Insulin-induced Hypoglycemia and Dexamethasone -

Kenji OYHYAMA and Kiyohiko KATO

Abstract: The ACTH secretory mechanism was studied in five patients with congenital adrenal hypoplasia. Plasma ACTH levels were measured by bioassay using isolated rat adrenal cells and by radioimmunodssay using N-terminal antibody. Gel chromatography (0.9×25 cm Sephadex G.50 column) was performed on plasma extracts from 3 patients. Although all of the subjects were treated with adequate hydrocortisone replacement therapy,they had elevated basal plasma ACTH levels, augmented responses to insulin-induced hypoglycemia, and a poor suppressive effect of dexamethasone. Moreover, plasma i-ACTH levels were much higher than plasma b-ACTH levels, and in gel chromatography, the big form of ACTH was found in plasma. These findings suggest that a large amount of biologically inactive ACTH exists with b-ACTH in plasma in congenital adrenal hypoplasia, and some of these ACTHs are independent of negative feedback inhibition.
Thus, we hypothesize that in congenital adrenal hypoplasia hypertrophic or hyperplastic anterior and intermediate lobes exist and secrete a biologically inactive ACTH with b-ACTH.

Key words: Congenital adrenal hypoplasia, ACTH, Big ACTH, Dexamethasone, Pituitary gland




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