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血管周细胞瘤的临床特征、实验室检查结果及治疗:系统综述。

Clinical features, laboratory findings and treatment of juxtaglomerular cell tumors: a systemic review.

机构信息

Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

出版信息

Hypertens Res. 2024 May;47(5):1380-1390. doi: 10.1038/s41440-024-01606-w. Epub 2024 Mar 4.

Abstract

Juxtaglomerular cell tumors (JGCTs) or reninoma are rare kidney tumors leading to secondary hypertension, and the non-specific clinical manifestations bring about challenges to the diagnosis. This study is to summarize the clinical features, laboratory findings, and treatment of JGCTs. The PubMed, EMBASE database, and manual search were utilized to find all cases, and 158 reports containing 261 patients were identified. Data on patients' demographics, clinical features, diagnostic methods, and treatment options were collected and analyzed. JGCTs occurred predominantly in female patients (female to male ratio, 2.1:1). The median age of patients was 25 years (IQR:18-34 years). Hypertension (97.24%) was the cardinal manifestation. Hypokalemia was reported in 78.71% (159/202) of subjects, and normal serum potassium accounted for 20.79% (42/202). In cases with assessed plasma renin activity (PRA) levels, the median PRA was 7.89 times the upper limit of normal (IQR:3.58-14.41), and 3.82% (5/131) of cases in the normal range. Tumors were detected in 97.8% (175/179) computed tomography (CT), 94.7% (72/76) magnetic resonance imaging (MRI), and 81.5% (110/135) ultrasound, respectively. For 250/261 patients undergoing surgical procedures, 89.14% (197/221), 94.94% (150/158), and 100% (131/131) of patients were restored to normal blood pressure, PRA, and serum potassium, respectively. JGCTs are commonly associated with hypertension, hypokalemia, and hyperreninemia, whereas patients with normotension, normokalemia, and PRA should be systematically pursued after drug-elution lasting for 2 weeks. CT and MRI are more sensitive imaging diagnostic methods. The blood pressure and biochemical parameters of most patients returned to normal after surgery.

摘要

肾小球旁细胞瘤(JGCT)或肾素瘤是一种罕见的肾肿瘤,可导致继发性高血压,其非特异性临床表现给诊断带来挑战。本研究旨在总结 JGCT 的临床特征、实验室发现和治疗方法。我们利用 PubMed、EMBASE 数据库和手动搜索,查找了所有病例,并确定了包含 261 例患者的 158 份报告。收集并分析了患者的人口统计学、临床特征、诊断方法和治疗选择的数据。JGCT 主要发生在女性患者中(女:男比例为 2.1:1)。患者的中位年龄为 25 岁(IQR:18-34 岁)。高血压(97.24%)是主要表现。202 例中有 78.71%(159/202)存在低钾血症,而血清钾正常者占 20.79%(42/202)。在评估血浆肾素活性(PRA)水平的病例中,中位 PRA 是正常上限的 7.89 倍(IQR:3.58-14.41),而正常范围内的病例占 3.82%(5/131)。97.8%(175/179)的 CT、94.7%(72/76)的 MRI 和 81.5%(110/135)的超声分别检测到肿瘤。261 例患者中有 250 例行手术治疗,221 例(197/221)、158 例(150/158)和 131 例(131/131)的患者血压、PRA 和血清钾分别恢复正常。JGCT 通常与高血压、低钾血症和高肾素血症相关,而在药物洗脱持续 2 周后,应系统检测血压正常、血钾正常和 PRA 的患者。CT 和 MRI 是更敏感的影像学诊断方法。大多数患者术后血压和生化参数恢复正常。

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