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病例系列:尽管正常筛查调查,但仍诊断为原发性醛固酮增多症:三例报告。

Case series: Primary aldosteronism diagnosed despite normal screening investigations: A report of three cases.

机构信息

Department of Ultrasonography, The Second Affiliated Hospital Zhejiang University School of Medicine, Hangzhou, China.

Clinical Research Center, The Second Affiliated Hospital Zhejiang University School of Medicine, Hangzhou, China.

出版信息

Medicine (Baltimore). 2023 May 19;102(20):e33724. doi: 10.1097/MD.0000000000033724.

DOI:10.1097/MD.0000000000033724
PMID:37335717
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10194677/
Abstract

RATIONALE

Since the introduction of the aldosterone-to-renin ratio (ARR) as a screening tool for primary aldosteronism (PA), there has been a marked increase in the reported prevalence of this condition among hypertensive, even normotensive, subjects.

PATIENT CONCERNS

But ARR as a spot blood draw for estimating a patient's aldosterone secretory status is influenced by many factors.

DIAGNOSES

Here, we describe a series of patients with biochemically confirmed PA, whose diagnosis was delayed by the initial ARR assessment with non-suppressed renin.

INTERVENTIONS

Patient 1 had a history of resistant hypertension for many years and had a negative initial screening for secondary hypertension (including ARR). At the reevaluation, ARR was close to cutoff still with normal renin after strict and extended drug washout, and the further workup for PA demonstrated a unilateral aldosterone producing adenoma that was surgically removed, with subsequent complete biochemical remission and partial clinical success. Patient 2 was diagnosed with idiopathic hyperaldosteronism combined with obstructive sleep apnea syndrome, which could increase renin resulting in a negative ARR, and finally got a better treatment effect with PA-specific spironolactone, as well as continuous positive airway pressure. Patient 3 with hypokalemia as the main presentation was finally diagnosed with PA after excluding other diseases, and proceeded to laparoscopic adrenalectomy and histologically confirmed an aldosterone producing adenoma. Postoperatively, patient 3 achieved complete biochemical success without any medicine.

OUTCOMES

The clinical status of all three patients was effectively managed, resulting in either complete resolution or notable improvement of their respective conditions.

LESSONS

After rigorous standardized diagnostic evaluation, there are still many reasons for ARR negative in PA, but they all basically occur in the background of normal or normal-high renin without suppression. A negative screening test result should be repeated and analyzed carefully if this is not consistent with the clinical picture. If, despite a repeatedly negative ARR, clinical suspicion remains high, we recommend consideration of further evaluation, including confirmatory tests and adrenal venous blood sampling (AVS) or even 68Ga-pentixafor PET/CT to better confirm the diagnosis and improve patient outcomes.

摘要

背景

自从醛固酮与肾素比值(ARR)作为原发性醛固酮增多症(PA)的筛查工具引入以来,高血压甚至血压正常的人群中这种疾病的报告患病率显著增加。

患者关注点

但是,作为评估患者醛固酮分泌状态的即时采血,ARR 受到许多因素的影响。

诊断

在这里,我们描述了一系列生化确诊的 PA 患者,他们的诊断因初始 ARR 评估中肾素未被抑制而被延迟。

干预措施

患者 1 患有多年的难治性高血压,且最初的继发性高血压筛查(包括 ARR)均为阴性。在重新评估时,ARR 仍然接近截止值,且在严格和延长的药物洗脱后肾素仍正常,进一步检查 PA 发现单侧醛固酮分泌腺瘤,经手术切除后,随后生化完全缓解和部分临床疗效改善。患者 2 被诊断为特发性醛固酮增多症合并阻塞性睡眠呼吸暂停综合征,这会导致肾素增加,ARR 呈阴性,最终使用特定的醛固酮拮抗剂螺内酯以及持续气道正压通气治疗取得了更好的效果。以低钾血症为主要表现的患者 3 最终在排除其他疾病后被诊断为 PA,并进行了腹腔镜肾上腺切除术,组织学证实为醛固酮分泌腺瘤。术后,患者 3 无需任何药物即可达到完全生化缓解。

结果

所有三名患者的临床状况均得到有效管理,症状得到完全缓解或明显改善。

经验教训

经过严格的标准化诊断评估,PA 中仍有许多 ARR 阴性的原因,但这些原因基本上都发生在肾素正常或正常偏高且未被抑制的背景下。如果与临床情况不符,应重复并仔细分析阴性筛查检测结果。如果尽管 ARR 反复为阴性,但临床怀疑仍然很高,我们建议考虑进一步评估,包括确认性检查和肾上腺静脉采血(AVS),甚至 68Ga- 戊替昔福 PET/CT,以更好地确认诊断并改善患者结局。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a708/10194677/e57c90a3e2ba/medi-102-e33724-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a708/10194677/d1c71e03cd31/medi-102-e33724-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a708/10194677/4fc53c104332/medi-102-e33724-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a708/10194677/2d1acabf56c9/medi-102-e33724-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a708/10194677/e57c90a3e2ba/medi-102-e33724-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a708/10194677/d1c71e03cd31/medi-102-e33724-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a708/10194677/4fc53c104332/medi-102-e33724-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a708/10194677/2d1acabf56c9/medi-102-e33724-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a708/10194677/e57c90a3e2ba/medi-102-e33724-g004.jpg

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