Hyperaldosteronismus

Hyperaldosteronism

Zusammenfassung

Aldosteron wird in der Nebennierenrinde produziert und reguliert den Volumen- und Elektrolythaushalt. Ein Hyperaldosteronismus kann entweder primär (reninunabhängig) oder sekundär (reninabhängig) auftreten. Der primäre Hyperaldosteronismus als häufigste Ursache der sekundären Hypertonie ist mit einem erhöhten kardiovaskulären Risiko assoziiert. Seine wichtigsten Subtypen sind das aldosteronproduzierende Adenom als häufigste unilaterale Form und der bilaterale Hyperaldosteronismus, selten treten eine unilaterale Hyperplasie, familiäre Formen und das Nebennierenkarzinom auf. Diagnostisch wird als Screeningparameter der Aldosteron/Renin-Quotient bestimmt. Ist dieser erhöht, folgen ein Bestätigungstest und eine Schnittbildgebung. Goldstandard zur Unterscheidung unilateraler von bilateralen Formen ist die seitengetrennte Nebennierenvenenkatheterisierung. Unilaterale Formen können mittels Adrenalektomie grundsätzlich kurativ therapiert werden, während bei Kontraindikation zur Operation und bei bilateralen Formen die Therapie mit Mineralokortikoidrezeptorantagonisten im Vordergrund steht. Studien der letzten zehn Jahre konnten somatische Mutationen, meist in Ionenkanälen oder -transportern, als Ursache von aldosteronproduzierenden Adenomen und sog. aldosteronproduzierenden Zellclustern nachweisen (aldosteronproduzierende Zellcluster sind womöglich Vorstufen von Adenomen und Korrelate der bilateralen Hyperplasie, aber auch eines subklinischen Hyperaldosteronismus). Ein familiärer Hyperaldosteronismus wird durch Keimbahnmutationen in überlappenden Genen ausgelöst. Ein sekundärer Hyperaldosteronismus kann im Rahmen einer Hypertonie als Folge einer Diuretikatherapie oder einer Nierenarterienstenose auftreten.

Abstract

Aldosterone is produced in the adrenal cortex and governs volume and electrolyte homeostasis. Hyperaldosteronism can occur either as primary aldosteronism (renin-independent) or secondary aldosteronism (renin-dependent). As the commonest cause of secondary hypertension, primary aldosteronism is associated with increased cardiovascular risk. Its most prevalent subtypes are aldosterone-producing adenomas as the most frequent unilateral form and bilateral hyperaldosteronism. Unilateral hyperplasia, familial hyperaldosteronism and aldosterone-producing carcinoma are rare. The aldosterone/renin ratio serves as a screening parameter for primary aldosteronism. If this ratio is elevated, confirmatory testing and adrenal imaging are performed. Adrenal venous sampling is considered the gold standard for the distinction of unilateral from bilateral disease. Unilateral disease can potentially be cured by adrenalectomy, whereas patients that are not candidates for surgery or have bilateral disease are treated with mineralocorticoid receptor antagonists. Over the past 10 years, somatic mutations in ion channels or transporters have been identified as causes of aldosterone-producing adenomas and so-called aldosterone-producing cell clusters (potential precursors of adenomas and correlates of bilateral hyperplasia, but also of subclinical hyperaldosteronism). In addition, germline mutations in overlapping genes cause familial hyperaldosteronism. Secondary hyperaldosteronism can occur in patients with hypertension treated with diuretics or in renal artery stenosis.

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Förderung

Gefördert durch die Stiftung Charité und die Deutsche Forschungsgemeinschaft (SFB 1365, SFB 1453).

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Correspondence to Univ.-Prof. Dr. med. U. I. Scholl.

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U.I. Scholl gibt an, dass kein Interessenkonflikt besteht.

Für diesen Beitrag wurden von der Autorin keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.

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H. Haller, Hannover

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Scholl, U.I. Hyperaldosteronismus. Internist 62, 245–251 (2021). https://doi.org/10.1007/s00108-021-00972-8

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Schlüsselwörter

  • Aldosteron
  • Renin
  • Nebennierenadenom
  • Mineralokortikoidrezeptorantagonisten
  • Mutation

Keywords

  • Aldosterone
  • Renin
  • Adrenocortical adenoma
  • Mineralocorticoid receptor antagonists
  • Mutation