A new form of hereditary primary aldosteronism: familial hyperaldosteronism type III

In this issue of the Journal, Geller D and coll. report a newly described Mendelian form of familial hyperaldosteronism with distinctive clinical and biochemical features from previously described forms. This new familial form of PA, that could be called Familial Hyperaldosteronism Type III (FH-III), is characterized by severe hypertension in early childhood associated with marked aldosteronism, hypokalemia and significant target organ damage, that were resistant to aggressive anti-hypertensive therapy including spironolactone and amiloride thus requiring bilateral adrenalectomy. The future challenge therefore, will be the identification of the genes responsible for FH-II and FH-III. The early diagnosis in infant patients and in pre-clinical situations will allow the timed institution of a specific therapy thus preventing the development of target organ damage.