Scholl U I
Medizinische Klinik mit Schwerpunkt Nephrologie und Internistische Intensivmedizin, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Charitéplatz 1, 10117, Berlin, Deutschland.
Berlin Institute of Health (BIH), Berlin, 10178, Deutschland.
Internist (Berl). 2021 Mar;62(3):245-251. doi: 10.1007/s00108-021-00972-8. Epub 2021 Feb 18.
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.
醛固酮由肾上腺皮质分泌,调控血容量及电解质平衡。醛固酮增多症可分为原发性醛固酮增多症(不依赖肾素)和继发性醛固酮增多症(依赖肾素)。作为继发性高血压最常见的病因,原发性醛固酮增多症与心血管疾病风险增加相关。其最常见的亚型是醛固酮瘤,这是最常见的单侧形式,以及双侧醛固酮增多症。单侧增生、家族性醛固酮增多症和醛固酮分泌癌较为罕见。醛固酮/肾素比值可作为原发性醛固酮增多症的筛查指标。如果该比值升高,则需进行确诊检查及肾上腺成像。肾上腺静脉采血被认为是区分单侧与双侧病变的金标准。单侧病变可通过肾上腺切除术治愈,而不适合手术或患有双侧病变的患者则用盐皮质激素受体拮抗剂治疗。在过去10年中,离子通道或转运体的体细胞突变已被确定为醛固酮瘤以及所谓的醛固酮分泌细胞团(腺瘤的潜在前体以及双侧增生的相关因素,也是亚临床醛固酮增多症的相关因素)的病因。此外,重叠基因中的种系突变可导致家族性醛固酮增多症。继发性醛固酮增多症可发生于使用利尿剂治疗的高血压患者或肾动脉狭窄患者中。