Abstract
Subjects (8) with SL-CAH, 21-hydroxylase deficiency, on glucocorticoid therapy were studied after mineralocorticold therapy was discontinued. They had similar neonatal salt wasting. On constant sodium (Na) intake, all demonstrated negative Na balance with increasing plasma renin. Although plasma Aldo was often in normal range, it was low considering the degree of renin elevation, indicating a biosynthetic defect. They may be divided into 3 groups: I-normal Aldo concentrations and relatively effective Na conservation, near normal urinary pregnanetriol (P'triol); II-high Aldo, elevated renin with significant Na loss and high P'triol; and III-low Aldo and Na loss despite high renin. These data indicate that while the most severe salt-losing defects are associated with very low Aldo. clinically significant salt loss on a normal Na intake may result from relative Aldo insensitivity possibly due to steroid antagonists of adrenal origin.
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Keenan, B., Kirkland, R. & Clayton, G. SODIUM WASTING WITH NORMAL PLASMA ALDOSTERONE (ALDO) CONCENTRATIONS IN SALT LOSING CONGENITAL ADRENAL HYPERPLASIA (SL-CAH). Pediatr Res 11, 427 (1977). https://doi.org/10.1203/00006450-197704000-00346
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DOI: https://doi.org/10.1203/00006450-197704000-00346