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. 1979 May;54(5):350–355. doi: 10.1136/adc.54.5.350

Continuing need for mineralocorticoid therapy in salt-losing congenital adrenal hyperplasia.

I A Hughes, A Wilton, C A Lole, O P Gray
PMCID: PMC1545561  PMID: 475410

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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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. 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]
  2. 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]
  3. 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]
  4. 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]
  5. 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]
  6. 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]
  7. 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]
  8. 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]
  9. 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]
  10. 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]
  11. 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]
  12. 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]
  13. 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]
  14. 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]
  15. Rose G. Epidemiology of familial factors and salt intake in man. Postgrad Med J. 1977;53 (Suppl 2):139–143. [PubMed] [Google Scholar]
  16. 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]

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