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因右侧肾上腺静脉重复导致原发性醛固酮增多症伴假双侧肾上腺静脉采样的多维诊断策略:一例报告

Multidimensional diagnostic strategies for managing primary aldosteronism with false bilateral AVS due to a duplicated right adrenal vein: A case report.

作者信息

Ma Meng, Shen Sikui, Abudukerimu Buatikamu, Xie Wei, Zou Mingxi, Chen Ying, Tian Haoming, Ren Yan, Chen Tao

机构信息

Department of Endocrinology and Metabolism, Adrenal Center, West China Hospital of Sichuan University, Chengdu, Sichuan Province, PR China.

Department of Radiology, West China Hospital of Sichuan University, Chengdu, Sichuan Province, PR China.

出版信息

Medicine (Baltimore). 2025 Jul 25;104(30):e43520. doi: 10.1097/MD.0000000000043520.

DOI:10.1097/MD.0000000000043520
PMID:40725906
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12303502/
Abstract

RATIONALE

Primary aldosteronism can be categorized into 2 main subtypes: aldosterone-producing adenoma (APA) and idiopathic hyperaldosteronism. The treatment strategies for these 2 subtypes differ markedly: APA requires surgical removal of the adenoma, whereas idiopathic hyperaldosteronism is managed with pharmacological therapy. Adrenal venous sampling (AVS) is the most widely used technique for diagnosing APA, but its results can be influenced by abnormal adrenal or tumor venous drainage.

PATIENT CONCERNS

A 58-year-old female was admitted due to elevated blood pressure for over 20 years and persistent hypokalemia for 3 months, accompanied by reduced physical strength, nocturia (2-3 times per night, 200-300 mL each time), and poor sleep quality.

DIAGNOSES

The AVS results of the patient did not demonstrate lateralized aldosterone secretion. However, the patient's Kupers and Kobayashi predictive scores, as well as elevated levels of 18-hydroxycortisol and 11-deoxycorticosterone, all supported the presence of a unilateral APA. Retrospective analysis of contrast-enhanced adrenal computed tomography revealed a right adrenal adenoma with a solitary vein draining directly into the inferior vena cava. In addition, 68Ga-pentixafor positron emission tomography/magnetic resonance imaging supported the diagnosis of a right-sided APA.

INTERVENTIONS

Right adrenalectomy was performed.

OUTCOMES

At 1 and 3 months postoperatively, the patient exhibited complete clinical and biochemical remission.

LESSONS

This case highlights that when there is discordance between AVS findings and clinical assessments (e.g., predictive models), a multifaceted approach incorporating predictive scores, 18-hydroxycortisol, 11-deoxycorticosterone levels, and 68Ga-pentixafor positron emission tomography/magnetic resonance imaging can facilitate accurate diagnosis and guide management decisions.

摘要

理论依据

原发性醛固酮增多症可分为2种主要亚型:醛固酮瘤(APA)和特发性醛固酮增多症。这2种亚型的治疗策略显著不同:APA需要手术切除腺瘤,而特发性醛固酮增多症则采用药物治疗。肾上腺静脉采样(AVS)是诊断APA最广泛使用的技术,但其结果可能受到肾上腺或肿瘤静脉引流异常的影响。

患者情况

一名58岁女性因血压升高20多年、持续性低钾血症3个月入院,伴有体力下降、夜尿(每晚2 - 3次,每次200 - 300毫升)和睡眠质量差。

诊断

患者的AVS结果未显示醛固酮分泌的侧别差异。然而,患者的库珀斯和小林预测评分以及18 - 羟皮质醇和11 - 脱氧皮质酮水平升高,均支持存在单侧APA。对增强肾上腺计算机断层扫描的回顾性分析显示右肾上腺腺瘤有一条单独的静脉直接汇入下腔静脉。此外,68Ga - 喷替酸正电子发射断层扫描/磁共振成像支持右侧APA的诊断。

干预措施

进行了右肾上腺切除术。

结果

术后第1个月和第3个月,患者临床和生化指标完全缓解。

经验教训

该病例表明,当AVS结果与临床评估(如预测模型)不一致时,采用包括预测评分、18 - 羟皮质醇、11 - 脱氧皮质酮水平以及68Ga - 喷替酸正电子发射断层扫描/磁共振成像在内的多方面方法,有助于准确诊断并指导管理决策。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3e65/12303502/7e6edc7cd3e3/medi-104-e43520-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3e65/12303502/7e6edc7cd3e3/medi-104-e43520-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3e65/12303502/7e6edc7cd3e3/medi-104-e43520-g001.jpg

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本文引用的文献

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J Korean Soc Radiol. 2024 Mar;85(2):456-462. doi: 10.3348/jksr.2023.0057. Epub 2024 Mar 5.
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Accuracy of Gallium-68 Pentixafor Positron Emission Tomography-Computed Tomography for Subtyping Diagnosis of Primary Aldosteronism.镓-68 吡咯烷酮[¹¹C]戊烷多靶素正电子发射断层扫描-计算机断层扫描用于原发性醛固酮增多症的亚型诊断的准确性。
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Radiological characteristics and diagnostic impact of duplicated right adrenal veins on adrenal venous sampling in primary aldosteronism.
原发性醛固酮增多症中右肾上腺静脉重复对肾上腺静脉采样的放射学特征和诊断影响。
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Duplicate inferior vena cava complicating the evaluation of primary aldosteronism.下腔静脉重复导致原发性醛固酮增多症的评估复杂化。
BMJ Case Rep. 2021 Jan 18;14(1):e240093. doi: 10.1136/bcr-2020-240093.
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Use of Steroid Profiling Combined With Machine Learning for Identification and Subtype Classification in Primary Aldosteronism.应用类固醇分析联合机器学习对原发性醛固酮增多症进行鉴定和亚型分类。
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J Hypertens. 2018 Nov;36(11):2269-2276. doi: 10.1097/HJH.0000000000001855.
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Targeting CXCR4 (CXC Chemokine Receptor Type 4) for Molecular Imaging of Aldosterone-Producing Adenoma.靶向 CXCR4(CXC 趋化因子受体型 4)用于醛固酮分泌腺瘤的分子成像。
Hypertension. 2018 Feb;71(2):317-325. doi: 10.1161/HYPERTENSIONAHA.117.09975. Epub 2017 Dec 26.
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