Wu Hong-hua, Wang Guang-ya, Ma Xiao-wei, Guo Xiao-hui
Department of Endocrinology, Peking University First Hospital, Beijing, China.
Zhonghua Nei Ke Za Zhi. 2012 Jan;51(1):24-7.
Reninoma is a rare benign tumor of the renal juxtaglomerular cell apparatus that causes hypertension and hypokalemia via hypersecretion of renin, while it is extremely rare that reninoma induced hypertensive crisis with reversible posterior encephalopathy syndrome (RPES). To improve the clinical understanding for this disease, we conducted a case-analysis.
To analyze the clinical and pathological data of a case of reninoma-induced hypertensive crisis with reversible posterior encephalopathy syndrome, who was admitted to Peking University First Hospital in November, 2007 and follow-up.
This was a 16-year old female patient, onset with suddenly spasm with loss of consciousness, while blood pressure stepped up to 210/140 mm Hg (1 mm Hg = 0.133 kPa), and the head magnetic resonance imaging (MRI) revealed "multiple long-T(2) signal", and hypokalemia (2.8 - 3.2 mmol/L), urine protein positive, ultrasound cardiogram revealed left ventricular hypertrophy, laboratory study revealed hyperreninism (38.23 ng·ml(-1)×h(-1), normal range 0.07 - 1.15 ng·ml(-1)×h(-1)) and hyperaldosteronism (660.9 ng/L, normal range 60 - 174 ng/L), abdominal CT-Scan revealed a mass at right kidney, blood pressure achieved safety range and the head MRI was rechecked and revealed "the abnormal long-T(2) signal disappeared". The clinical diagnosis was reninoma induced hypertensive crisis with RPES. The tumor was resected and the pathologic diagnosis was reninoma. The patient remained normotensive in the postoperative period without any medication.
Reninoma represents a rare but surgically curable cause of hypertension, thus the clinical suspicion of it is very important in young patients. If the diagnosis is confirmed, positive treatment must be done immediately to improve the prognosis. The most common cause of RPES is hypertension, and the diagnosis depends on the distinctive head MRI. There is always a good prognosis with the decline of blood pressure rapidly.
肾素瘤是一种罕见的起源于肾球旁器的良性肿瘤，可通过肾素分泌过多导致高血压和低钾血症，而肾素瘤诱发伴有可逆性后部脑病综合征（RPES）的高血压危象极为罕见。为提高对该疾病的临床认识，我们进行了病例分析。
分析2007年11月入住北京大学第一医院的1例肾素瘤诱发伴有可逆性后部脑病综合征的高血压危象患者的临床及病理资料，并进行随访。
该患者为16岁女性，突发抽搐伴意识丧失，血压升至210/140 mmHg（1 mmHg = 0.133 kPa），头颅磁共振成像（MRI）显示“多发长T2信号”，低钾血症（2.8 - 3.2 mmol/L），尿蛋白阳性，超声心动图显示左心室肥厚，实验室检查显示高肾素血症（38.23 ng·ml-1×h-1，正常范围0.07 - 1.15 ng·ml-1×h-1）和高醛固酮血症（660.9 ng/L，正常范围60 - 174 ng/L），腹部CT扫描显示右肾有一肿块，血压降至安全范围后复查头颅MRI显示“异常长T2信号消失”。临床诊断为肾素瘤诱发伴有RPES的高血压危象。肿瘤切除后病理诊断为肾素瘤。术后患者未服用任何药物，血压维持正常。
肾素瘤是一种罕见但可通过手术治愈的高血压病因，因此临床上对年轻患者怀疑此病非常重要。确诊后必须立即积极治疗以改善预后。RPES最常见的病因是高血压，其诊断依赖于特征性的头颅MRI表现。血压迅速下降通常预后良好。
