Abstract
Four patients with salt-losing congenital adrenal hyperplasia (CAH) who had stopped mineralocorticoid therapy for several years, showed raised plasma concentrations of 17OH-progesterone and plasma renin activity, despite adequate glucoticoid therapy. One patient was able to reduce urinary sodium excretion when the sodium intake was restricted. Another patient who was a salt-loser, developed signs of an adrenal crisis when salt deprived. In comparison, one nonsalt-loser and 2 normal subjects decreased urinary sodium excretion in response to sodium restriction. The addition of fludrocortisone (100 micrograms) to usual maintenance doses of glucocorticoid, resulted in normal levels of plasma 17OH-progesterone and plasma renin activity in all 4 salt-losers. Two female salt-losers, with raised plasma testosterone concentrations, began menstruating when their plasma testosterone concentrations returned to normal after treatment with fludrocortisone. It is recommended that salt-losing CAH patients should be given mineralocorticoid, in addition to glucocorticoid therapy, at least until adult life.
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- Bartter F. C., Henkin R. I., Bryan G. T. Aldosterone hypersecretion in "non-salt-losing" congenital adrenal hyperplasia. J Clin Invest. 1968 Aug;47(8):1742–1752. doi: 10.1172/JCI105864. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Brook C. G., Zachmann M., Prader A., Mürset G. Experience with long-term therapy in congenital adrenal hyperplasia. J Pediatr. 1974 Jul;85(1):12–19. doi: 10.1016/s0022-3476(74)80277-5. [DOI] [PubMed] [Google Scholar]
- Chaussain J. L., Estrada Y., Roger M., Tea N. T., Scholler R., Canlorbe P., Job J. C. La 17-OH progestérone plasmatique. Mesure chez l'enfant normal et dans les hyperplasies surrénales congénitales par bloc de la 21-hydroxylation. Nouv Presse Med. 1974 Dec 21;3(44):2621–2624. [PubMed] [Google Scholar]
- Dillon M. J., Ryness J. M. Plasma renin activity and aldosterone concentration in children. Br Med J. 1975 Nov 8;4(5992):316–319. doi: 10.1136/bmj.4.5992.316. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Grant D. B., Dillon M. J., Atherden S. M., Levinsky R. J. Congenital adrenal hyperplasia: renin and steroid values during treatment. Eur J Pediatr. 1977 Aug 23;126(1-2):86–96. doi: 10.1007/BF00443127. [DOI] [PubMed] [Google Scholar]
- Haber E., Koerner T., Page L. B., Kliman B., Purnode A. Application of a radioimmunoassay for angiotensin I to the physiologic measurements of plasma renin activity in normal human subjects. J Clin Endocrinol Metab. 1969 Oct;29(10):1349–1355. doi: 10.1210/jcem-29-10-1349. [DOI] [PubMed] [Google Scholar]
- Hamilton W., McCandless A. E., Ireland J. T., Gray C. E. Hypoaldosteronism in three sibs due to 18-dehydrogenase deficiency. Arch Dis Child. 1976 Aug;51(8):576–583. doi: 10.1136/adc.51.8.576. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Hillier S. G., Brownsey B. G., Cameron E. H. Some observations on the determination of testosterone in human plasma by radioimmunoassay using antisera raised against testosterone-3-BSA and testosterone-11 -BSA. Steroids. 1973 May;21(5):735–754. doi: 10.1016/0039-128x(73)90139-6. [DOI] [PubMed] [Google Scholar]
- Hughes I. A., Winter J. S. The application of a serum 17OH-progesterone radioimmunoassay to the diagnosis and management of congenital adrenal hyperplasia. J Pediatr. 1976 May;88(5):766–773. doi: 10.1016/s0022-3476(76)81112-2. [DOI] [PubMed] [Google Scholar]
- Hughes I. A., Winter J. S. The relationships between serum concentrations of 17OH-progesterone and other serum and urinary steroids in patients with congenital adrenal hyperplasia. J Clin Endocrinol Metab. 1978 Jan;46(1):98–104. doi: 10.1210/jcem-46-1-98. [DOI] [PubMed] [Google Scholar]
- Kirkland J., Kirkland R., Librik L., Clayton G. Serum gonadotropin levels in female adolescents with congenital adrenal hyperplasia. J Pediatr. 1974 Mar;84(3):411–414. doi: 10.1016/s0022-3476(74)80728-6. [DOI] [PubMed] [Google Scholar]
- Limal J. M., Rappaport R., Bayard F. Plasma aldosterone, renin activity, and 17alpha-hydroxyprogesterone in salt-losing congenital adrenal hyperplasia. I. Response to ACTH in hydrocortisone treated patients and effect of 9alpha-fluorocortisol. J Clin Endocrinol Metab. 1977 Sep;45(3):551–559. doi: 10.1210/jcem-45-3-551. [DOI] [PubMed] [Google Scholar]
- Newns G. H. Congenital adrenal hyperplasia. Arch Dis Child. 1974 Jan;49(1):1–3. doi: 10.1136/adc.49.1.1. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Rayyis S. S., Horton R. Effect of angiotensin II on adrenal and pituitary function in man. J Clin Endocrinol Metab. 1971 Apr;32(4):539–546. doi: 10.1210/jcem-32-4-539. [DOI] [PubMed] [Google Scholar]
- Rose G. Epidemiology of familial factors and salt intake in man. Postgrad Med J. 1977;53 (Suppl 2):139–143. [PubMed] [Google Scholar]
- Rösler A., Levine L. S., Schneider B., Novogroder M., New M. I. The interrelationship of sodium balance, plasma renin activity and ACTH in congenital adrenal hyperplasia. J Clin Endocrinol Metab. 1977 Sep;45(3):500–512. doi: 10.1210/jcem-45-3-500. [DOI] [PubMed] [Google Scholar]