Aggravation of salt loss due to hydrocortisone in the first days of treatment of congenital adrenal hyperplasia caused by 21-hydroxylase deficiency
Bey-Omar, F.; Feit, J.P.; Forest, M.G.; David, M.
Pediatrie 38(2): 77-86
1983
ISSN/ISBN: 0031-4021 PMID: 6622141 Document Number: 211827
The longitudinal study of five infants affected with congenital hyperplasia (CAH) due to 21-hydroxylase deficiency illustrate the aggravation of the salt losing syndrome during the initial period of replacement therapy with hydrocortisone alone, associated or not with salt supplementation as demonstrated by weight loss or failure to thrive, hyponatremia and rise in plasma renin activity. Concomitantly, the plasma levels of both ACTH and aldosterone decreased monkedly. The sodium loss was rapidly normalized following the association of small doses (25 gamma/day) of 9 alpha fluorohydrocortisone to the same glucocorticoid replacement dosage. Initial treatment of infants affected with CAH should therefore include both mineral and glucocorticoid hormones.