Research progress of apparent mineralocorticoid excess syndrome / 中国基层医药

ABSTRACT

Apparent mineralocorticoid excess (AME) is an autosomal recessive inheritance caused by 11β－hydroxysteroid dehydrogenase 2 gene mutation.It may occur in newborn and adult.AME was first reported in 1977 by Werder et al.Its clinical features include hyporenin type hypertension , hypoaldosteronemia, metabolic alkalosis, hypernatremia and hypokalemia.In recent years,with the improvement of clinical diagnosis ,especially gene detection , AME has been reported one after another.In this paper,the pathogenesis,clinical manifestation ,diagnosis and therapy of AME were reviewed in order to improve the understanding of this disease and provide reference for its clinical diagnosis and treatment.