6 Mineralocorticoid resistance

摘要: Summary Mineralocorticoid resistance, or pseudohypoaldosteronism (PHA), is a rare cause of salt wasting in young children. It may be inherited as an autosomal dominant recessive trait, it occur sporadically or, rarely, develop secondary to other conditions. characterized by episodes dehydration and hyponatraemia the face high aldosterone levels. In most cases, after short period supplementation no further ill effects are experienced. The condition great interest because provides insights into both mechanisms which water balance controlled actions aldosterone. This article reviews normal physiology aldosterone, with particular reference its biosynthesis specific target tissues. Current knowledge regarding molecular involved action discussed some detail. clinical features PHA reviewed diagnostic issues management considered. Finally, current views pathophysiology presented. Here, considerable uncertainty remains. Whilst many cases there greatly reduced binding receptor, underlying abnormality yet identified; particular, spite strong reasons for suspecting defect defects mineralocorticoid so far direct evidence support this hypothesis. concludes discussion possible explanations PHA.