山梨医科大学雑誌　第4巻4号　179-183(1989)

ACTH Secretory Mechanism in Congenital Adrenal Hypoplasia.
II. Paradoxical Responses of Plasma Bioactive ACTH
to Thyrotropin-Releasing Hormone ( TRH) and
Luteinizing Hormone-releasing Hormone (LH-RH)

Kenji OHYAMA and Kiyohiko KATO

Abstract: The ACTH secretory mechanism was studied in four patients with congenital adrenal hypoplasia. Plasma ACTH levels were measured by bioassay using isolated rat adrenal cells. In three subjects, the plasma bioactive (b-) ACTH levels increased in response to TRH or LH-RH loadings. In another patient, we examined the plasma b-ACTH response to TRH under a hydrocortisone constant infusion. Plasma b-ACTH levels were decreased slightly during the infusion and increased in response to TRH. These findings suggest that the intermediate lobe is the source of ACTH secretion by TRH in congenital adrenal hypoplasia.
Our hypothesis that ACTH is secreted from both the anterior and intermediate lobes of the pituitary in congenital adrenal hypoplasia is supported by the present study.

Key words: congenital adrenal hypoplasia, ACTH, TRH, LH-RH, Nelson's syndrome.




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