Mineralocorticoid Unresponsiveness with Severe Neonatal Hyponatremia and Hyperkalemia
- 1 February 1980
- journal article
- case report
- Published by The Endocrine Society in Journal of Clinical Endocrinology & Metabolism
- Vol. 50 (2) , 401-404
- https://doi.org/10.1210/jcem-50-2-401
Abstract
An infant with severe neonatal hyponatremia and hyperkalemia is described. Although marked elevations of urinary 17-hydroxycorticosteroids suggested an 18-dehydrogenase aldosterone biosynthetic defect, the infant proved to have mineralocorticoidunresponsiveness, or pseudohypoaldosteron-ism. Dietary sodium supplementation and ion exchange resin administration resulted in normalization of serum electrolytes and urinary 17-hydroxycorticosteroids. ACTH infusion produced natriuresis, suggesting the need for additional sodium supplementation during the stress of illness, with a resultant increase i n ACTH secretion. Determinations of the relative amounts of urinary 18-hydroxy and aldosterone metabolites appear necessary for early definitive diagnosis of the disorder. (J Clin En-docrinol Metab50: 401, 1980)Keywords
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