Abstract
Seven children with salt-losing CAH who had been off mineralo-corticoid for several years were studied. 9 children with non-salt-losing CAH were controls. The 7 subjects showed clinical evidence of poor control despite suppressive doses of hydrocortisone; most showed coincidental signs of glucocorticoid excess. Chemical assessment of control included serum 17-OHP, PRA, and ACTH and 24-hour urine 17-KS and PT. The salt-losers were then placed on Florinef, 0.1 mg bid with improvement in all measured parameters(see table), decreased fatigue and salt craving, and in some, a decreased cortisol requirement without hypertension.
We thus conclude: 1)the renin-angiotensin system can stimulate all zones of the adrenal cortex, and elevated PRA can lead to poor control in salt-losing CAH just as can inadequate suppression of ACTH; 2)salt-losers need mineralocorticoid as well as glucocorticoid replacement for optimal control; and 3)salt-losers should be maintained on mineralocorticoid for life.
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Horner, J., Hintz, R. 303 THE ROLE OF RENIN AND ANGIOTENSIN IN SALT-LOSING CAH. Pediatr Res 12 (Suppl 4), 414 (1978). https://doi.org/10.1203/00006450-197804001-00308
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DOI: https://doi.org/10.1203/00006450-197804001-00308