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原发性醛固酮增多症的病理生理学与组织病理学

Pathophysiology and histopathology of primary aldosteronism.

作者信息

Williams Tracy Ann, Reincke Martin

机构信息

Medizinische Klinik und Poliklinik IV, Klinikum der Universität München, LMU München, München, Germany; Division of Internal Medicine and Hypertension, Department of Medical Sciences, University of Turin, Turin, Italy.

Medizinische Klinik und Poliklinik IV, Klinikum der Universität München, LMU München, München, Germany.

出版信息

Trends Endocrinol Metab. 2022 Jan;33(1):36-49. doi: 10.1016/j.tem.2021.10.002. Epub 2021 Nov 4.

Abstract

Primary aldosteronism (PA) can be sporadic or familial and classified into unilateral and bilateral forms. Sporadic PA predominates with excessive aldosterone production usually arising from a unilateral aldosterone-producing adenoma (APA) or bilateral adrenocortical hyperplasia. Familial PA is rare and caused by germline variants, that partly correspond to somatic alterations in APAs. Classification into unilateral and bilateral PA determines the treatment approach but does not accurately mirror disease pathology. Some evidence indicates a disease continuum ranging from balanced aldosterone production from each adrenal to extreme asymmetrical bilateral aldosterone production. Nonetheless, surgical removal of the overactive adrenal in unilateral PA achieves highly successful outcomes and almost all patients are biochemically cured of their aldosteronism.

摘要

原发性醛固酮增多症(PA)可呈散发性或家族性,分为单侧和双侧形式。散发性PA占主导,醛固酮过度分泌通常源于单侧醛固酮瘤(APA)或双侧肾上腺皮质增生。家族性PA较为罕见,由种系变异引起,部分与APA中的体细胞改变相对应。将PA分为单侧和双侧决定了治疗方法,但不能准确反映疾病病理。一些证据表明,存在一个疾病连续谱,从每个肾上腺醛固酮分泌平衡到极度不对称的双侧醛固酮分泌。尽管如此,单侧PA中手术切除功能亢进的肾上腺可取得非常成功的结果,几乎所有患者的醛固酮增多症在生化指标上都可治愈。

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