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Pathophysiology and histopathology of primary aldosteronism
Trends in Endocrinology & Metabolism ( IF 11.4 ) Pub Date : 2021-11-04 , DOI: 10.1016/j.tem.2021.10.002
Tracy Ann Williams 1 , Martin Reincke 2
Affiliation  

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.



中文翻译:

原发性醛固酮增多症的病理生理学和组织病理学

原发性醛固酮增多症 (PA) 可以是散发性或家族性的,分为单侧和双侧形式。散发性 PA 主要表现为醛固酮分泌过多,通常由单侧醛固酮分泌腺瘤 (APA) 或双侧肾上腺皮质增生引起。家族性 PA 很少见,由种系变异引起,部分对应于 APA 的体细胞改变。单侧和双侧 PA 的分类决定了治疗方法,但不能准确反映疾病病理。一些证据表明,从每个肾上腺的平衡醛固酮产生到极端不对称的双侧醛固酮产生,疾病的连续性不一而足。尽管如此,

更新日期:2021-12-15
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