The syndrome of juxtaglomerular hyperplasia with aldosteronism, hypokalemic alkalosis and normal blood pressure.
In the syndrome of hypokalemic alkalosis, aldosteronism, elevated plasma renin and normal blood pressure, the juxtaglomerular apparatus shows hypertrophy and hyperplasia of all elements. Renal handling of sodium is abnormal: despite excessive delivery of proximal fluid to distal sites so that potassium secretion is excessive, free water formation is subnormal. The syndrome is frequently familial, but no inherited case is known. In erythrocytes low sodium efflux coefficient and high sodium concentration may serve as "markers" for the syndrome. In two cases, products of consanguineous relations, autosomal recessive inheritance is suggested.
D000471:Alkalosis / Q000150:complications*; D001794:Blood Pressure; D006801:Humans; D006929:Hyperaldosteronism / Q000150:complications*; D006965:Hyperplasia; D006984:Hypertrophy; D007008:Hypokalemia / Q000150:complications*; D007606:Juxtaglomerular Apparatus / Q000473:pathology*; D007674:Kidney Diseases / Q000150:complications / Q000235:genetics / Q000378:metabolism; D012083:Renin / Q000378:metabolism; D012964:Sodium / Q000378:metabolism; D013577:Syndrome
D012964:Sodium; D012083:Renin