title: [Update on endocrine hypertension].
abstract: Endocrine hypertension is the most common cause of secondary hypertension affecting ~3 % of the population, with primary hyperaldosteronism and pheochromocytoma being the principal conditions. Both diseases share an increased cardiovascular risk in comparison with essential hypertension patients (at the same blood pressure level). This augmented cardiovascular risk as well as the availability of specific treatment emphasize the importance of timely and correct diagnosis. Primary hyperaldosteronism, representing one tenth of hypertensive patients, is an under-diagnosed disease partly because of difficult diagnostic steps and absence of standard criteria. Recently, the description of somatic mutations in KCNJ5 gene in Conn adenomas had precipitated a resurgence of research activity to understand the pathophysiology of this common disease. Research had confirmed the role of these mutations in aldosterone hypersecretion; however, its role in adenoma formation is still to be elucidated. Elsewhere, much remains to be done in order to understand the pathogenesis of bilateral idiopathic hyperaldosteronism, the other common subtype of primary hyperaldosteronism. In pheochromocytoma, the revolution of genetics has led to major advances in the characterization of this rare disease. It is now clear that up to 50 % of patients with pheochromocytoma have a genetic abnormality and that different pheochromocytomas segregate into two clusters with distinct genotypes, signal transduction pathways and expression of biomarkers (phenotype). This continuing progress has huge effects on patient's management and follow-up. In this article we will shed light on the recent developments in both diseases with emphasis on their role in patient care.
authors.affiliation: Service d'Endocrinologie, Diabétologie et Maladies Métaboliques, Hôpital Avicenne, AP-HP, 125 route de Stalingrad, 93009 Bobigny, France. abdallahalsalameh@hotmail.com
authors.forename: A
authors.initials: A
authors.lastname: Al-Salameh
authors.forename: R
authors.initials: R
authors.lastname: Cohen
authors.forename: P
authors.initials: P
authors.lastname: Chanson
authors.forename: P F
authors.initials: PF
authors.lastname: Plouin
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000150:complications / Q000175:diagnosis / Q000235:genetics; D018246:Adrenocortical Adenoma / Q000150:complications / Q000175:diagnosis / Q000235:genetics; D000450:Aldosterone / Q000378:metabolism; D002318:Cardiovascular Diseases; D004700:Endocrine System Diseases / Q000150:complications*; D006801:Humans; D006929:Hyperaldosteronism / Q000150:complications / Q000175:diagnosis / Q000235:genetics; D006973:Hypertension / Q000209:etiology*; D009154:Mutation; D010673:Pheochromocytoma / Q000150:complications / Q000175:diagnosis / Q000235:genetics; D012307:Risk Factors
chemical_list: D000450:Aldosterone