title: Syndromes that Mimic an Excess of Mineralocorticoids.
abstract: Pseudohyperaldosteronism is characterized by a clinical picture of hyperaldosteronism with suppression of renin and aldosterone. It can be due to endogenous or exogenous substances that mimic the effector mechanisms of aldosterone, leading not only to alterations of electrolytes and hypertension, but also to an increased inflammatory reaction in several tissues. Enzymatic defects of adrenal steroidogenesis (deficiency of 17α-hydroxylase and 11β-hydroxylase), mutations of mineralocorticoid receptor (MR) and alterations of expression or saturation of 11-hydroxysteroid dehydrogenase type 2 (apparent mineralocorticoid excess syndrome, Cushing's syndrome, excessive intake of licorice, grapefruits or carbenoxolone) are the main causes of pseudohyperaldosteronism. In these cases treatment with dexamethasone and/or MR-blockers is useful not only to normalize blood pressure and electrolytes, but also to prevent the deleterious effects of prolonged over-activation of MR in epithelial and non-epithelial tissues. Genetic alterations of the sodium channel (Liddle's syndrome) or of the sodium-chloride co-transporter (Gordon's syndrome) cause abnormal sodium and water reabsorption in the distal renal tubules and hypertension. Treatment with amiloride and thiazide diuretics can respectively reverse the clinical picture and the renin aldosterone system. Finally, many other more common situations can lead to an acquired pseudohyperaldosteronism, like the expansion of volume due to exaggerated water and/or sodium intake, and the use of drugs, as contraceptives, corticosteroids, β-adrenergic agonists and FANS. In conclusion, syndromes or situations that mimic aldosterone excess are not rare and an accurate personal and pharmacological history is mandatory for a correct diagnosis and avoiding unnecessary tests and mistreatments.
authors.affiliation: Department of Medicine (DIMED), Endocrinology, University of Padua, Via Ospedale 105, 35128, Padua, Italy.
authors.forename: Chiara
authors.initials: C
authors.lastname: Sabbadin
authors.affiliation: Department of Medicine (DIMED), Endocrinology, University of Padua, Via Ospedale 105, 35128, Padua, Italy. decio.armanini@unipd.it.
authors.forename: Decio
authors.initials: D
authors.lastname: Armanini
mesh_terms: D000450:Aldosterone / Q000097:blood*; D015415:Biomarkers / Q000097:blood; D003480:Cushing Syndrome / Q000097:blood / Q000188:drug therapy / Q000209:etiology; D003907:Dexamethasone / Q000627:therapeutic use; D004032:Diet / Q000009:adverse effects; D020022:Genetic Predisposition to Disease; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D006929:Hyperaldosteronism / Q000097:blood* / Q000188:drug therapy / Q000209:etiology / Q000235:genetics; D006973:Hypertension / Q000097:blood* / Q000188:drug therapy / Q000209:etiology / Q000235:genetics; D056929:Liddle Syndrome / Q000097:blood / Q000188:drug therapy / Q000235:genetics; D000451:Mineralocorticoid Receptor Antagonists / Q000627:therapeutic use; D010641:Phenotype; D012084:Renin-Angiotensin System / Q000187:drug effects; D012307:Risk Factors; D049993:Sodium Chloride Symporter Inhibitors / Q000627:therapeutic use; D015854:Up-Regulation
chemical_list: D015415:Biomarkers; D005938:Glucocorticoids; D000451:Mineralocorticoid Receptor Antagonists; D049993:Sodium Chloride Symporter Inhibitors; D000450:Aldosterone; D003907:Dexamethasone
keywords: 11 Hydroxysteroid dehydrogenase type 2; Aldosterone; Apparent mineralocorticoid excess syndrome; Deoxycorticosterone; Hypertension; Licorice; Mineralocorticoid receptor; Pseudohyperaldosteronism