Hyporeninemic hypoaldosteronism


Hyporeninemic hypoaldosteronism: A rare condition where low levels of renin result in insufficient aldosterone being produced


Hyperkalemia Heart arrhythmia Muscle weakness Low plasma rennin activity Low plasma aldosterone


he most common causes of hypoaldosteronism are hyporeninemic hypoaldosteronism or primary adrenal insufficiency, an adrenal enzyme defect (most often CYP11B2 [aldosterone synthase, P450c11as] deficiency), or pseudohypoaldosteronism, a rare genetic disorder of aldosterone resistance, the autosomal recessive form of which is due to a mutation in the collecting tubule sodium channel gene. These disorders can be differentiated by measurement of plasma renin activity (PRA) and serum aldosterone and cortisol. These tests should be performed after the administration of a loop diuretic or three hours in the upright position, which will increase renin and aldosterone release in normals, but not in these disease states. Hyporeninemic hypoaldosteronism most often occurs in patients 50 to 70 years of age with diabetic nephropathy or chronic interstitial nephritis who have mild to moderate renal insufficiency.