GLUCOCORTICOID SUPPRESSIBLE HYPERALDOSTER ONISM - AN UNUSUAL CAUSE OFFAMILIAL HYPERTENSION

The glucocorticoid suppressible hyperaldosteronism (GSH) is a rare for m of systemic hypertension. We report a family a father and his two ch ildren - with this disease. GSH represents a peculiar form of primary hyperaldosteronism, with usually an absence of increase of aldosterone in response to upright posture, a sustained responsiveness of aldoste rone to prolonged ACTH stimulation and high levels of two steroids, th e 18-hydroxy- and the 18-oxocortisol. But the two main features of GSH which distinguish it from other causes of hyperaldosteronism are the prompt reversal of the features of mineralocorticoids excess by glucoc orticoid therapy and the autosomal dominant mode of inheritance. Recen t studies demonstrate that this disorder is caused by an abnormal stru cture of the aldosterone synthase gene. Treatment by glucocorticoid (u sually 0,30 to 0,75 mg dexamethasone daily) can reverse hypertension a nd hypokaliemia.