临床儿科杂志 ›› 2019, Vol. 37 ›› Issue (1): 51-54.doi: 10.3969/j.issn.1000-3606.2019.01.013

• 罕见病 疑难病 • 上一篇    下一篇

儿童肾动脉纤维肌发育不良致难治性高血压1 例报告

陈乃清,肖慧捷,王芳   

  1. 1. 北京大学第一医院(北京 100034);2. 广州中医药大学第二附属医院(广东广州 510120 )
  • 出版日期:2019-01-15 发布日期:2019-02-01
  • 通讯作者: 肖慧捷 电子信箱:huijiexiao2@hotmail.com

Refractory hypertension caused by renal artery fibromuscular dysplasia in one child

CHEN Naiqing, XIAO Huijie, WANG Fang   

  1. 1. Department of Pediatrics, Peking University First Hospital, Beijing 100034, China; 2. The Second Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou 510120, Guangdong, China
  • Online:2019-01-15 Published:2019-02-01

摘要: 目的 探讨儿童肾动脉纤维肌发育不良(FMD)致难治性高血压的临床特点、诊断和治疗。方法 回顾分析1 例FMD致难治性高血压患儿的临床资料。结果 患儿,男, 4岁起病,以血压明显升高为突出表现,同时伴血钾降低,肾素、 血管紧张素明显升高,经血管彩色超声和胸腹增强CT加血管重建,诊断为肾血管性高血压。经口服多种降压药效果欠佳, 行肾血管造影后诊断为FMD所致难治性高血压。因不宜手术,调整降压方案,血压趋于稳定。连续随访8年,每年调整降 压方案并动态评估血压波动及靶器官受损情况。结论 儿童FMD所致肾血管性高血压初期可无特异性表现,首选药物治 疗,并可考虑外科治疗。

关键词: 肾血管性高血压; 肾素-血管紧张素-醛固酮; 肾动脉纤维肌层发育不良; 儿童

Abstract:  Objective To explore the clinical characteristics, diagnosis and treatment of refractory hypertension caused by renal artery fibromuscular dysplasia (FMD) in children. Method The clinical data of a child with refractory hypertension caused by FMD were retrospectively analyzed. Results A boy, with onset at age of 4 years, had significantly increased blood pressure and decreased serum potassium. His renin and angiotensin levels were significantly elevated. The boy was diagnosed with renovascular hypertension by color Doppler ultrasonography, enhanced thoracoabdominal CT and vascular reconstruction. Oral administration of a variety of antihypertensive drugs is not effective. The child was finally diagnosed of refractory hypertension caused by FMD using renal angiography. The child was not suitable for surgery. After adjusting the antihypertensive drugs, the blood pressure of the child tended to be stable. The boy received continuous follow-up for 8 years. The antihypertensive regimen was adjusted annually and blood pressure fluctuations and target organ damage were dynamically assessed. Conclusion Children with FMD caused renal vascular hypertension may have no specific manifestations in the early stage. The drug treatment is preferred, and surgical treatment may be considered.

Key words: renal vascular hypertension; renin-angiotensin-aldosterone; renal artery fibromuscular dysplasia; child