title: Aldosterone hypersecretion in "non-salt-losing" congenital adrenal hyperplasia.
abstract: Patients with the "non-salt-losing" form of the adrenogenital syndrome were studied before and after suppression of adrenal cortical activity with carbohydrate-active steroids. The response of aldosterone secretion to sodium deprivation was measured; in some patients response to adrenocorticotropic hormone (ACTH) was measured as well. The aldosterone secretion was normal and responded normally to sodium deprivation in all patients studied during suppression with carbohydrate-active steroids. This finding suggests that 21-hydroxylation of progesterone is normal in this syndrome. The sole abnormality in the production of aldosterone in these patients was found to be excessive secretion of aldosterone while they were not receiving suppressive doses of carbohydrate-active steroids. This finding strongly supports the view that the biogenetic pathways through which aldosterone is produced from progesterone are intact in this syndrome. No patient showed hypertension or hypokalemic alkalosis despite very high aldosterone secretion rates. This observation suggests that the hyper-aldosteronism is secondary to a tendency to sodium loss in the patient whose ACTH production is not suppressed. These studies provide additional evidence in support of the hypothesis that the salt-losing and "non-salt-losing" forms of adrenogenital syndrome are genetically and biochemically distinct.
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000037:antagonists & inhibitors / Q000096:biosynthesis*; D000312:Adrenal Hyperplasia, Congenital / Q000151:congenital* / Q000188:drug therapy / Q000378:metabolism; D000324:Adrenocorticotropic Hormone / Q000037:antagonists & inhibitors / Q000378:metabolism; D000328:Adult; D000450:Aldosterone / Q000096:biosynthesis; D002648:Child; D002675:Child, Preschool; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D006929:Hyperaldosteronism / Q000209:etiology*; D006965:Hyperplasia; D007010:Hyponatremia / Q000378:metabolism; D008297:Male; D011374:Progesterone / Q000096:biosynthesis; D012964:Sodium / Q000378:metabolism*; D012965:Sodium Chloride / Q000652:urine
chemical_list: D000305:Adrenal Cortex Hormones; D005938:Glucocorticoids; D012965:Sodium Chloride; D000450:Aldosterone; D011374:Progesterone; D000324:Adrenocorticotropic Hormone; D012964:Sodium