Department of Medicine-DIMED, University of Padua, Padua PD, Italy.
Department of Pharmacology and Toxicology, G.V. (Sonny) Montgomery VA Medical Center, Jackson, Mississippi.
Endocr Rev. 2018 Dec 1;39(6):1029-1056. doi: 10.1210/er.2018-00060.
The identification of several germline and somatic ion channel mutations in aldosterone-producing adenomas (APAs) and detection of cell clusters that can be responsible for excess aldosterone production, as well as the isolation of autoantibodies activating the angiotensin II type 1 receptor, have rapidly advanced the understanding of the biology of primary aldosteronism (PA), particularly that of APA. Hence, the main purpose of this review is to discuss how discoveries of the last decade could affect histopathology analysis and clinical practice. The structural remodeling through development and aging of the human adrenal cortex, particularly of the zona glomerulosa, and the complex regulation of aldosterone, with emphasis on the concepts of zonation and channelopathies, will be addressed. Finally, the diagnostic workup for PA and its subtyping to optimize treatment are reviewed.
醛固酮瘤(APA)中几种种系和体细胞离子通道突变的鉴定,以及能够导致醛固酮过度产生的细胞簇的检测,以及血管紧张素 II 型 1 受体激活的自身抗体的分离,这些都迅速推动了对原发性醛固酮增多症(PA)生物学的理解,特别是 APA 的理解。因此,本篇综述的主要目的是讨论过去十年的发现如何影响组织病理学分析和临床实践。本文将讨论人类肾上腺皮质,特别是肾小球带的发育和衰老过程中的结构重塑,以及醛固酮的复杂调节,重点是分区和通道病的概念。最后,回顾了 PA 的诊断评估及其亚型分类以优化治疗。