In an 11 yr old male with the syndrome of primary hyperaldosteronism and hypertension due to adrenal hyperplasia, we have found evidence for abnormal ACTH regulation. Since renin is completely suppressed, it is not regulating aldosterone. Diurnal periodicity of F was normal and F, DOC and B were normally stimulable with ACTH and suppressible with dexamethasone. In contrast, though aldosterone was stimulable with prolonged ACTH administration it was not suppressible with high-dose, long-term dexamethasone. Circulating ACTH levels were repeatedly elevated and were also non-suppressible with dexamethasone despite complete suppression of F, DOC and B. The data suggest the presence of two hypothalamic-pituitary systems, one regulating F in the normal fashion, the second involving a species of non-suppressible ACTH which presumably does not regulate F secretion. Preliminary characterization of the patient's ACTH showed that it is of a higher molecular weight than the normal ACTH 1-39. Whether chronic elevation of this form of ACTH is related to the hyperaldosteronism and adrenal hyperplasia or, indeed, is the cause of selective hypersecretion of aldosterone in this patient is under investigation.
About this article
Cite this article
Schneider, B., Levine, L., Rosler, A. et al. Hyperaldosteronism with non-suppressible ACTH. Pediatr Res 12, 156 (1978). https://doi.org/10.1203/00006450-197802000-00059