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Progressive adrenal failure in polyglandular autoimmune disease

Abstract

We describe here the clinical course of a boy who developed isolated failure of the zona glomerulosa as part of polyglandular autoimmune disease(PAD)and in whom function of the zona fasciculata remains intact.Initially the patient presented with hypoparathy-roidism and mucocutaneous candidiasis.ACTH tests at age 8 and 11 yrs resulted in a normal response of both mineralo- and glucocorticoids.The constellation of hyponatremia, hyperkalemia and growth failure at age 14 yrs prompted a reevaluation. A repeat ACTH test, assessing individual contributions of zona fasciculata and glomerulosa, showed normal plasma cortisol, DOC and B responses and a normal urinary response of 18 OH DOC and TH DOC.Urinary 18 OH B and urinary as well as plasma aldosterone were undetectable. Plasma renin activity(PRA)was markedly elevated.The ACTH response of adrenal androgens,presumably metabolic products of the zona reticularis was also deficient.Antiadrenal antibodies against all three layers of the adrenal cortex were positive. Mineralo-corticoid therapy resulted not only in normalization of electrolytes and PRA but also in catch up growth.The course of this patient suggests that in addition to monitoring the electrolyte status, periodic tests for both mineralo- and glucocorticoid synthesis should be performed in children with PAD because progressive adrenal insufficiency may go unrecognized.

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Saenger, P., Levine, L., Irvine, W. et al. Progressive adrenal failure in polyglandular autoimmune disease. Pediatr Res 15, 1559 (1981). https://doi.org/10.1203/00006450-198112000-00146

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