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原发性醛固酮增多症中醛固酮和皮质醇共分泌肾上腺腺瘤的临床特征

Clinical Characteristics of Aldosterone- and Cortisol-Coproducing Adrenal Adenoma in Primary Aldosteronism.

作者信息

Tang Lu, Li Xintao, Wang Baojun, Ma Xin, Li Hongzhao, Gao Yu, Gu Liangyou, Nie Wenyuan, Zhang Xu

机构信息

State Key Laboratory of Kidney Disease, Department of Urology, Chinese PLA Medical Academy, Chinese People's Liberation Army General Hospital, Beijing, China.

Department of Urology, Air Force General Hospital, Beijing, China.

出版信息

Int J Endocrinol. 2018 Mar 25;2018:4920841. doi: 10.1155/2018/4920841. eCollection 2018.

Abstract

Aldosterone- and cortisol-coproducing adrenal adenoma (A/CPA) cases have been observed in patients with primary aldosteronism (PA). This study investigated the incidence, clinical characteristics, and molecular biological features of patients with A/CPAs. We retrospectively identified 22 A/CPA patients from 555 PA patients who visited the Chinese People's Liberation Army General Hospital between 2004 and 2015. Analysis of clinical parameters revealed that patients with A/CPAs had larger tumors than those with pure APAs ( < 0.05). Moreover, they had higher proportions of cardiovascular complications, glucose intolerance/diabetes, and osteopenia/osteoporosis compared to the pure APA patients ( < 0.001). In the molecular biological findings, quantitative real-time PCR analysis revealed similar CYP11B1 and CYP17A1 mRNA expressions in resected A/CPA specimens and in pure APA specimens. Western blot and immunochemical analyses showed CYP11B1, CYP11B2, and CYP17A1 expressions in both A/CPAs and pure APAs. Seventeen cases with KCNJ5 mutations were detected among the 22 A/CPA DNA samples, but no PRKACA or other causative mutations were observed. Each patient improved following adrenalectomy. In conclusion, A/CPAs were not rare among PA patients. These patients associated with high incidences of cardiovascular events and metabolic disorders. Screening for excess cortisol secretion is necessary for PA patients.

摘要

原发性醛固酮增多症(PA)患者中已观察到产生醛固酮和皮质醇的肾上腺腺瘤(A/CPA)病例。本研究调查了A/CPA患者的发病率、临床特征和分子生物学特征。我们回顾性地从2004年至2015年期间就诊于中国人民解放军总医院的555例PA患者中识别出22例A/CPA患者。临床参数分析显示,A/CPA患者的肿瘤比单纯醛固酮瘤(APA)患者的肿瘤更大(<0.05)。此外,与单纯APA患者相比,他们发生心血管并发症、糖耐量异常/糖尿病和骨质减少/骨质疏松的比例更高(<0.001)。在分子生物学研究结果中,定量实时PCR分析显示,切除的A/CPA标本和单纯APA标本中CYP11B1和CYP17A1 mRNA表达相似。蛋白质印迹和免疫化学分析显示A/CPA和单纯APA中均有CYP11B1、CYP11B2和CYP17A1表达。在22份A/CPA DNA样本中检测到17例KCNJ5突变,但未观察到PRKACA或其他致病突变。每位患者肾上腺切除术后均有改善。总之,A/CPA在PA患者中并不罕见。这些患者心血管事件和代谢紊乱的发生率较高。PA患者有必要筛查皮质醇分泌过多情况。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9fc6/5889857/c7082d339155/IJE2018-4920841.001.jpg

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