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

Clinical Characteristics of Aldosterone- and Cortisol-Coproducing Adrenal Adenoma in Primary Aldosteronism.
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原发性醛固酮增多症中醛固酮和皮质醇同时产生的肾上腺腺瘤的临床特征。

DOI:
10.1155/2018/4920841
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发表时间:
2018
影响因子:
2.8
通讯作者:
Zhang X
Zhang X
中科院分区:
医学4区
文献类型:
--
作者:
Tang L;Li X;Wang B;Ma X;Li H;Gao Y;Gu L;Nie W;Zhang X

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在原发性醛固酮增多症(PA)患者中已观察到醛固酮和皮质醇共同分泌的肾上腺腺瘤(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年间在中国人民解放军总医院就诊的555例PA患者中确定了22例A/CPA患者。对临床参数的分析显示,A/CPA患者的肿瘤比单纯醛固酮分泌腺瘤(APA)患者的更大(P < 0.05)。此外,与单纯APA患者相比,他们心血管并发症、葡萄糖耐受不良/糖尿病以及骨质减少/骨质疏松的比例更高(P < 0.001)。在分子生物学发现方面,实时定量PCR分析显示,在切除的A/CPA标本和单纯APA标本中,CYP11B1和CYP17A1 mRNA的表达相似。蛋白质印迹和免疫化学分析显示,CYP11B1、CYP11B2和CYP17A1在A/CPAs和单纯APAs中均有表达。在22例A/CPA的DNA样本中检测到17例存在KCNJ5突变,但未观察到PRKACA或其他致病性突变。每位患者在肾上腺切除术后均有改善。总之,A/CPAs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。对PA患者进行皮质醇分泌过多的筛查是必要的。
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 (P < 0.05). Moreover, they had higher proportions of cardiovascular complications, glucose intolerance/diabetes, and osteopenia/osteoporosis compared to the pure APA patients (P < 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.
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