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Characterization of Adrenocorticotropin Secretion in a Patient with 17-Hydroxylase Deficiency^*

Endocrinology Division, Department of Medicine, Faculty of Medicine 14049 Ribeirão Preto SP, Brazil

Address all correspondence and requests for reprints to: Ayrton C. Moreira, M.D., Department of Medicine, Faculty of Medicine, 14049, Ribeirão Preto SP, Brazil.

17-Hydroxylase deficiency (17-OHDS) is a peculiar type of adrenal insufficiency because of elevated cortico-sterone (B) production. Episodic and circadian ACTH secretion and the responses to ovine CRH and metyrapone were examined in a female with 17-OHDS under conditions of normal sodium (NSI) and low sodium (LSI) intake. Plasma ACTH and B were higher on LSI than on NSI (mean ± SD: ACTH, 58 ± 20 vs. 33 ± 14 pmol/L; B, 508 ± 107 vs. 381 ± 69 nmol/L). ACTH pulses were concomitant with or followed by B pulses. An increase in the amplitude, but not the number, of ACTH and B pulses was found on LSI. Circadian ACTH and B rhythms persisted on LSI. Aldosterone levels were higher on LSI than on NSI. After metyrapone, plasma B decreased in the afternoon (NSI, 220 ± 10; LSI, 275 ± 35 nmol/L) and rose on the following morning. Plasma ACTH increased substantially on the following morning (NSI, 135 ± 47; LSI, 307 ± 77 pmol/L). The ACTH peak levels after ovine CRH increased after metyrapone administration. These data indicate that B may also have a negative feedback effect at the pituitary level. In all maneuvers, there were no changes in PRA, plasma sodium and potassium, or cortisol. We conclude that in 17-OHDS, in the absence of cortisol production, ACTH and B may interact to modulate the brain-pituitary-adrenocortical system.

^* This work was supported by Conselho Nacional de Desenvolvimento Científico e Tecnològico (CNPq Grant 30.0834/82-9) and Fundação de Amparo á Pesquisa São Paulo (FAPESP Grant 87/3448-6).

Received October 18, 1989.