Dexamethasone-suppressible hyperaldosteronism: a large new kindred.

Dexamethasone-suppressible hyperaldosteronism (DSH) is a rare familial variety of primary aldosteronism in which the biochemical features of mineralocorticoid excess are corrected by treatment with glucocorticoids. We report a large new kindred with this syndrome, the second such family described in the British Isles and the first in Ireland. The family has a dramatic history of premature cardiovascular death. We have documented DSH in four members and found evidence of aldosterone excess in two others. The kindred is unique in that we have documented DSH in distant relatives (fourth cousins). All patients described are well controlled on potassium-sparing diuretics. Our report underlines the importance of a detailed family history in the assessment of hypertension due to primary aldosteronism.