Congenital Hypoaldosteronism

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Abstract
Identical male twins suffering from congenital hypoaldosteronism due to a rare adrenal enzyme deficiency between corticosterone and aldosterone were followed up from birth until their present age of 13 yr. The symptoms of salt loss disappeared and normal growth rate resumed following treatment with DOCA [deoxycorticosterone acetate] and salt supplementation. Discontinuation of mineralocorticoid administration at the age of 7 yr resulted during a 5 yr period in a marked decline in their growth rate. Laboratory data revealed a persistent, although less pronounced, metabolic impairment. Mineralocorticoid administration was resumed and the twins entered normal puberty and increased their growth rate, emphasizing their need for continued mineralocorticoid administration to maintain adequate growth rate and development.