title: Inherited forms of mineralocorticoid hypertension.
abstract: OBJECTIVE
  Inherited forms of mineralocorticoid hypertension are a group of monogenic disorders that, although rare, have enlightened our understanding of normal physiology, and subsequent processes implicated in the pathogenesis of 'essential' hypertension. They often present in early life and can be a cause of major morbidity and mortality that can be effectively treated with simple but targeted pharmacological therapy. Interestingly, all the conditions centre on the regulation of sodium transport through its epithelial channel, either directly or through mediators that act via the mineralocorticoid receptor.
  
  
  RESULTS
  In recent years, molecular mechanisms of these conditions and their functional consequences have been elucidated. Diagnosis has been facilitated by plasma and urinary biomarkers.
  
  
  CONCLUSIONS
  We provide an overview and diagnostic approach to apparent mineralocorticoid excess, glucocorticoid remediable aldosteronism, familial hyperaldosteronism type 2, Liddle's syndrome, Gordon's syndrome, activating mutations of the mineralocorticoid receptor, generalized glucocorticoid resistance and hypertensive forms of congenital adrenal hyperplasia.
authors.affiliation: Centre for Endocrinology, Diabetes and Metabolism, School of Clinical and Experimental Medicine, University of Birmingham, Birmingham, UK.
authors.forename: Zaki
authors.initials: Z
authors.lastname: Hassan-Smith
authors.forename: Paul M
authors.initials: PM
authors.lastname: Stewart
mesh_terms: D000312:Adrenal Hyperplasia, Congenital / Q000175:diagnosis / Q000235:genetics / Q000378:metabolism; D015415:Biomarkers / Q000097:blood; D006801:Humans; D006929:Hyperaldosteronism / Q000175:diagnosis / Q000235:genetics / Q000378:metabolism; D006973:Hypertension / Q000175:diagnosis / Q000235:genetics / Q000378:metabolism*; D056929:Liddle Syndrome / Q000175:diagnosis / Q000235:genetics / Q000378:metabolism; D043204:Mineralocorticoid Excess Syndrome, Apparent / Q000175:diagnosis / Q000235:genetics / Q000378:metabolism; D008901:Mineralocorticoids / Q000097:blood*; D009154:Mutation; D011546:Pseudohypoaldosteronism / Q000175:diagnosis / Q000235:genetics / Q000378:metabolism; D018161:Receptors, Mineralocorticoid / Q000235:genetics
chemical_list: D015415:Biomarkers; D008901:Mineralocorticoids; D018161:Receptors, Mineralocorticoid