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原发性醛固酮增多症中因双侧醛固酮明显抑制而误诊为异位醛固酮瘤的重复肾上腺静脉。

Duplicated adrenal veins in primary aldosteronism misdiagnosed with ectopic aldosteronoma due to apparent bilateral aldosterone suppression.

机构信息

Department of Endocrinology, Hainan General Hospital, Hainan Affiliated Hospital of Hainan Medical University, Haikou, China.

出版信息

Blood Press. 2023 Dec;32(1):2209664. doi: 10.1080/08037051.2023.2209664.

DOI:10.1080/08037051.2023.2209664
PMID:37183447
Abstract

BACKGROUND

Primary aldosteronism (PA) is considered the number one aetiology for secondary hypertension. Apart from confirmatory tests and localisation of PA determined by computed tomography (CT), adrenal venous sampling (AVS) is used to define whether aldosterone hypersecretion occurs inside one or both adrenal glands. However, even correctly-performed AVS may lead to undiagnostic results such as apparent bilateral adrenal suppression (apparent bilateral aldosterone suppression), in which the adrenal aldosterone-to-cortisol ratios (AC ratios) are decreased bilaterally compared to the peripheral blood sample, with several causes contributing to it.

CASE DESCRIPTION

Here, we describe the case of a 48-year-old man who was referred to our department for further investigation with a history of refractory hypertension, hypokalaemia, and aortic dissection. His hypertension and hypokalaemia were initially attributed to ectopic aldosteronoma due to his adrenal CT scan and AVS results. However, the correct diagnosis of an adenoma with duplicated right adrenal veins (duplicated adrenal veins) due to apparent bilateral aldosterone suppression was confirmed during surgery.

CONCLUSION

AVS is the gold standard accepted for PA subtyping, but sometimes when apparent bilateral aldosterone suppression is present, it can give ambiguous results. Duplicated right adrenal veins, may impact results, thus, AVS may not accurately provide evidence of unilateral hypersecretion for all PA patients. Repeat AVS or adrenal surgery can provide worthwhile diagnostic conclusions.

摘要

背景

原发性醛固酮增多症(PA)被认为是继发性高血压的首要病因。除了通过计算机断层扫描(CT)确定的确认性检查和 PA 定位外,还使用肾上腺静脉采样(AVS)来确定醛固酮分泌过多是发生在一个或两个肾上腺内。然而,即使正确进行 AVS,也可能导致诊断结果不明确,例如明显的双侧肾上腺抑制(双侧醛固酮抑制),双侧肾上腺的醛固酮与皮质醇比值(AC 比值)与外周血样本相比均降低,有几个原因会导致这种情况。

病例描述

这里,我们描述了一位 48 岁男性的病例,他因难治性高血压、低血钾和主动脉夹层而被转至我们科室进一步检查。他的高血压和低血钾最初归因于异位醛固酮瘤,因为他的肾上腺 CT 扫描和 AVS 结果。然而,在手术中,由于明显的双侧醛固酮抑制,正确诊断为右侧肾上腺静脉重复(重复肾上腺静脉)伴腺瘤。

结论

AVS 是 PA 亚型分型的金标准,但有时当出现明显的双侧醛固酮抑制时,它会给出模棱两可的结果。右侧肾上腺静脉重复可能会影响结果,因此,AVS 可能无法为所有 PA 患者的单侧过度分泌提供准确证据。重复 AVS 或肾上腺手术可以提供有价值的诊断结论。

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Duplicated adrenal veins in primary aldosteronism misdiagnosed with ectopic aldosteronoma due to apparent bilateral aldosterone suppression.原发性醛固酮增多症中因双侧醛固酮明显抑制而误诊为异位醛固酮瘤的重复肾上腺静脉。
Blood Press. 2023 Dec;32(1):2209664. doi: 10.1080/08037051.2023.2209664.
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引用本文的文献

1
Utility of Repeat Sampling in Bilateral Aldosterone Suppression During Adrenal Vein Sampling for Primary Aldosteronism.重复采样在原发性醛固酮增多症肾上腺静脉采样双侧醛固酮抑制中的效用
JCEM Case Rep. 2024 Apr 10;2(4):luae051. doi: 10.1210/jcemcr/luae051. eCollection 2024 Apr.