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高血压伴低钾血症的罕见病因:一例肾素瘤病例

[A rare cause of hypertension with hypokalemia: A case of reninoma].

作者信息

Zimmermann Yaël, Tawadros Cécile, Andrejevic-Blant Snezana, Vogel Gérard

机构信息

Service de néphrologie, hôpital Riviera-Chablais site de Rennaz, route du Vieux Sequoia 20, 1847 Rennaz, Suisse.

Service d'urologie, hôpital Riviera-Chablais site de Rennaz, 1847 Rennaz, Suisse.

出版信息

Nephrol Ther. 2021 Dec;17(7):538-542. doi: 10.1016/j.nephro.2021.05.005. Epub 2021 Jul 30.

Abstract

Reninoma or juxtaglomerular cell tumor is a rare usually benign renal renin secreting tumor. We report the case of an 18-year old woman, without any medical history, investigated in our hospital's emergency department for a bilateral papilledema. Ambulatory ophthalmological investigations were performed because of a newly occurring blurry vision, associated with diffuse headaches. Cerebral mRI and lumbar puncture recommended by the ophthalmologist and neurologist excluded intra-cranial hypertension. The patient presented with severe hypertension. Laboratory values showed hypokalemia, compensated metabolic alkalosis and microalbuminuria. During the hospital stay, she developed AKIN 1 acute renal injury. Ultrasound revealed a tissular cystic lesion of the superior pole of the right kidney. Abdominal mRI confirmed the lesion and raised suspicion for a renal cell carcinoma without calicial or vascular invasion. Plasma renin value was >500 mUI/L with normal values for plasma aldosterone. Renal biopsy diagnosed a juxtaglomerular cell tumor. After an aggressive initial treatment, hypertension remained well controlled with spironolactone only, finally allowing for withdrawal of all antihypertensive medications. Robot-assisted laparoscopic partial nephrectomy was performed. Studies of the operative specimen confirmed the diagnosis of benign reninoma. Clinical follow-up showed complete resolution of clinical and biological parameters.

摘要

肾素瘤或球旁细胞瘤是一种罕见的通常为良性的肾素分泌性肿瘤。我们报告了一例18岁女性病例,该患者无任何病史,因双侧视乳头水肿在我院急诊科接受检查。由于新出现的视力模糊并伴有弥漫性头痛,进行了门诊眼科检查。眼科医生和神经科医生建议的脑部磁共振成像(MRI)和腰椎穿刺排除了颅内高压。患者出现严重高血压。实验室检查结果显示低钾血症、代偿性代谢性碱中毒和微量白蛋白尿。住院期间,她发生了急性肾损伤网络(AKIN)1级急性肾损伤。超声检查发现右肾上极有一个组织性囊性病变。腹部MRI证实了该病变,并怀疑为无钙化或血管侵犯的肾细胞癌。血浆肾素值>500 mUI/L,而血浆醛固酮值正常。肾活检诊断为球旁细胞瘤。经过积极的初始治疗后,仅使用螺内酯就能很好地控制高血压,最终停用了所有抗高血压药物。实施了机器人辅助腹腔镜部分肾切除术。对手术标本的研究证实了良性肾素瘤的诊断。临床随访显示临床和生物学参数完全恢复正常。

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