Genetic and Potential Autoimmune Triggers of Primary Aldosteronism

Williams, Tracy Ann; Mulatero, Paolo; Bidlingmaier, Martin; Beuschlein, Felix; Reincke, Martín

doi:10.1161/hypertensionaha.115.05643

Cited by 10 publications

(12 citation statements)

References 59 publications

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“…Previous studies have shown that a high serum aldosterone concentration and potassium deficiency, the common clinical manifestations of PA, positively correlate with the metabolic disorders 7‐9 . PA has two main subtypes: unilateral, largely represented by aldosterone‐producing adenoma (APA), and bilateral, usually idiopathic hyperaldosteronism (IHA); which have differing characteristics and pathogenesis 10 . APA is characterized by more severe hyperaldosteronism and hypokalemia, 11 which may theoretically lead to higher prevalence of MetS than that of IHA.…”

Section: Introductionmentioning

confidence: 99%

Higher prevalence of metabolic disorders in patients with bilateral primary aldosteronism than unilateral primary aldosteronism

Zhang

Luo

Tuersun

et al. 2020

Clinical Endocrinology

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Objective: Metabolic syndrome (MetS) is a frequent clinical condition in hypertension patients and is more frequently reported in primary aldosteronism (PA). This study is aimed at investigating the prevalence of MetS and its components in the two major types of patients with adrenal venous sampling (AVS)-confirmed unilateral PA and bilateral PA. Design and patients: This was a retrospective cross-section study. We analysed metabolic parameters from 169 PA patients subtyped by AVS, including 85 unilateral PA patients and 84 bilateral PA patients, and we also included 169 non-PA patients matched for age and sex. Results: Patients with unilateral PA had higher concentrations of aldosterone and lower serum potassium than patients with bilateral PA. However, patients with bi

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Section: Introductionmentioning

confidence: 99%

Higher prevalence of metabolic disorders in patients with bilateral primary aldosteronism than unilateral primary aldosteronism

Zhang

Luo

Tuersun

et al. 2020

Clinical Endocrinology

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“…With increased screening and detection, the incidence of PA among hypertensive patients is currently reported at approximately 10% . This syndrome has multiple etiologies, including inherited gene mutations, development of autoantibodies, and ectopic aberrant functional adrenal receptors . The clinical management is guided by the definition of unilateral vs bilateral adrenal involvement.…”

mentioning

confidence: 99%

Proportion of Patients With Hypertension Resolution Following Adrenalectomy for Primary Aldosteronism: A Systematic Review and Meta‐Analysis

Benham

Eldoma

Khokhar

et al. 2016

J of Clinical Hypertension

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Unilateral primary aldosteronism (PA) is often treated with adrenalectomy, but hypertension resolution rates are variable. A valid estimate of the postoperative normotension rate is necessary to inform the utility of PA testing and treatment. The authors searched MEDLINE In-Process & Other Non-Indexed Citations, Embase, and Cochrane Central Register of Controlled Trials. Prospective adult cohort studies with surgically treated PA that reported resolution of hypertension without the aid of medications were included. Among 2620 abstracts identified by the search, 25 studies in the systematic review with data on 1685 patients were investigated. The pooled proportion of normotension following adrenalectomy was 52% (95% confidence interval, 0.44-0.60). Meta-regression demonstrated a significant negative association between length of follow-up and proportion of normotension, with normotension dropping by 6.7% per year of follow-up (coefficient À0.006; 95% confidence interval, À0.01 to 0.002). Overall, approximately half of the patients experienced hypertension resolution, although this outcome may not be durable in all patients.

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“…The discrepancy between the studies might be caused by methodological differences and deserves further investigation in large cohorts. As discussed earlier, the chronic stimulation of the ZG by AT1-AA with a higher predisposition to somatic mutations might possibly elucidate the development of IAH and APA [58] ( • ▶ Fig. 1).…”

Section: Genetics Of Primary Aldosteronism ▼mentioning

confidence: 85%

Recent Developments in Primary Aldosteronism

Asbach

Williams

Reincke

2016

Exp Clin Endocrinol Diabetes

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Primary aldosteronism (PA) is the most frequent endocrine cause of secondary arterial hypertension. Sporadic forms of PA caused mainly by an aldosterone producing adenoma (APA) or idiopathic adrenal hyperplasia (IAH) predominate; in contrast, familial forms (familial hyperaldosteronism types I, II and III) affect only a minor proportion of PA patients. Patient based registries and biobanks, international networks and next generation sequencing technologies have emerged over recent years. Somatic hot-spot mutations in the potassium channel GIRK4 (encoded by KCNJ5), in ATPases and a L-type voltage-gated calcium-channel correlate with the autonomous aldosterone production in approximately half of all APAs. The recently discovered form FH III is caused by different germline KCNJ5 mutations with variable clinical presentations and severity. Autoantibodies to the angiotensin II Type 1 receptor have been identified in patients with PA and possibly play a pathophysiological role in the development of PA. Adrenal vein sampling (AVS) represents the gold standard in differentiating unilateral and bilateral forms of PA. Recent consensus papers have tried to implement current guidelines in order to standardise the technique of AVS. New techniques like segmental AVS might allow a finer mapping of the aldosterone production within the adrenal gland. The measurement of the steroids 18-hydroxycortisol and 18-oxocortisol by liquid chromatography tandem mass spectrometry has been shown to be useful to distinguish between unilateral and bilateral forms of PA.

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Genetic and Potential Autoimmune Triggers of Primary Aldosteronism

Cited by 10 publications

References 59 publications

Higher prevalence of metabolic disorders in patients with bilateral primary aldosteronism than unilateral primary aldosteronism

Higher prevalence of metabolic disorders in patients with bilateral primary aldosteronism than unilateral primary aldosteronism

Proportion of Patients With Hypertension Resolution Following Adrenalectomy for Primary Aldosteronism: A Systematic Review and Meta‐Analysis

Recent Developments in Primary Aldosteronism

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