Abstract
Dexamethasone-suppressible hyperaldosteronism(DSH),a form of low renin hypertension,is a familial disorder of aldosterone secretion which is transmitted by an autosomal dominant gene. With dexamethasone(DEX) administration there is a prompt suppression of aldosterone excretion to unmeasurable levels and return to normal blood pressure in young subjects. With prolonged ACTH administration, unlike normal subjects in whom aldosterone excretion decreases after an initial rise, the DSH patient demonstrates a sustained and continuous increase of aldosterone excretion.This unusual aldosterone response mimics that of cortisol(F). These findings suggest that in DSH, aldosterone may be a product of the adrenal fasciculata or that ACTH regulates aldosterone secretion by the adrenal glomerulosa. We studied adrenal glomerulosa function in ten patients with DSH treated continuously with DEX 2mg/d and studied on a regular Na+diet(87meq/m2)and on a 10meq Na+ diet. With DEX treatment all patients showed a prompt suppression of adrenal fasciculata function as evidenced by suppression of serum F,corticosterone,desoxycorticosterone and urinary 18-OH-desoxy-corticosterone. The complete suppression of urinary pH 1 aldosterone by DEX was paralleled by a prompt suppression of urinary 18-OH-corticosterone. With continued DEX administration plasma renin levels rose to the normal range. Dietary sodium restriction resulted in a further rise in plasma renin activity and a rise in urinary pH 1 aldosterone and 180Hcorticosterone. Thus, in DSH the adrenal glomerulosa is responsive to the stimulation of reninangiotensin II.
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Oberfield, S., Levine, L., Chow, D. et al. 433 ADRENAL GLOMERULOSA FUNCTION IN DSH. Pediatr Res 15 (Suppl 4), 512 (1981). https://doi.org/10.1203/00006450-198104001-00444
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DOI: https://doi.org/10.1203/00006450-198104001-00444