The clinical phenotype resembles primary hyperaldosteronism, and the presenting feature is typically hypertension in teenage years. Patients are often asymptomatic. Renal impairment may occur due to hypertension. Muscle weakness in combination with severe hypertension has been reported in elderly population with the syndrome . The defining factor in the diagnosis is evidence of suppressed aldosterone levels, and the lack of response to treatment with the mineralocorticoid receptor blocker spironolactone [6;7]. Metabolic abnormalities can be corrected by dietary salt restriction, and administration of antagonists of the epithelial sodium channel such as amiloride or triamterene . Renal transplantation has been used as treatment.
|Original language||English (US)|
|Journal||Anasthesiologie und Intensivmedizin|
|State||Published - Sep 1 2016|
ASJC Scopus subject areas
- Critical Care and Intensive Care Medicine
- Anesthesiology and Pain Medicine