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Pathophysiology of bilateral hyperaldosteronism.


ABSTRACT:

Purpose of review

Renin-independent aldosterone production from one or both affected adrenal(s), a condition known as primary aldosteronism (PA), is a common cause of secondary hypertension. In this review, we aimed to summarize recent findings regarding pathophysiology of bilateral forms of PA, including sporadic bilateral hyperaldosteronism (BHA) and rare familial hyperaldosteronism.

Recent findings

The presence of subcapsular aldosterone synthase (CYP11B2)-expressing aldosterone-producing micronodules, also called aldosterone-producing cell clusters, appears to be a common histologic feature of adrenals with sporadic BHA. Aldosterone-producing micronodules frequently harbor aldosterone-driver somatic mutations. Other potential factors leading to sporadic BHA include rare disease-predisposing germline variants, circulating angiotensin II type 1 receptor autoantibodies, and paracrine activation of aldosterone production by adrenal mast cells. The application of whole exome sequencing has also identified new genes that cause inherited familial forms of PA.

Summary

Research over the past 10 years has significantly improved our understanding of the molecular pathogenesis of bilateral PA. Based on the improved understanding of BHA, future studies should have the ability to develop more personalized treatment options and advanced diagnostic tools for patients with PA.

SUBMITTER: Nanba K 

PROVIDER: S-EPMC9202492 | biostudies-literature | 2022 Jun

REPOSITORIES: biostudies-literature

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Pathophysiology of bilateral hyperaldosteronism.

Nanba Kazutaka K   Rainey William E WE  

Current opinion in endocrinology, diabetes, and obesity 20220601 3


<h4>Purpose of review</h4>Renin-independent aldosterone production from one or both affected adrenal(s), a condition known as primary aldosteronism (PA), is a common cause of secondary hypertension. In this review, we aimed to summarize recent findings regarding pathophysiology of bilateral forms of PA, including sporadic bilateral hyperaldosteronism (BHA) and rare familial hyperaldosteronism.<h4>Recent findings</h4>The presence of subcapsular aldosterone synthase (CYP11B2)-expressing aldosteron  ...[more]

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