Persistent hypertension despite dilation of renal artery stenosis in a boy with neurofibromatosis type 1
Persistent hypertension despite dilation of renal artery stenosis in a boy with neurofibromatosis type 1
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患有 1 型神经纤维瘤病的男孩尽管肾动脉狭窄扩张，但持续高血压
DOI：
10.1002/ajmg.a.35829
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发表时间：
2013
期刊：
影响因子：
--
通讯作者：
Takahashi. T
中科院分区：
文献类型：
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作者：
Ueda;K.;Awazu;M.;Konishi;Y.;Takenouchi;T.;Shimozato;S.;Kosaki;K.;Takahashi. T
高血压是1型神经纤维瘤病（NF1）的主要并发症之一。已知是由肾动脉狭窄或嗜铬细胞瘤引起的。然而，超过一半的NF1高血压患者没有这两种疾病。我们在此报告一例13岁男性NF1患者，他患有高血压和右肾动脉狭窄，并伴有病变侧肾静脉肾素升高。他接受了经皮腔内肾血管成形术。尽管动脉扩张成功，肾素水平正常化，但高血压持续超过6个月，需要抗高血压药物。宽脉压提示因NF1血管病变引起的动脉僵硬。我们认为，该患者高血压的原因被认为是NF1血管病变引起的动脉僵硬，而不是肾动脉狭窄。脉压升高支持了这一假设。这种动脉僵硬的标志可以无创地评估，并应在NF1中进行常规评估。©2013 Wiley期刊公司
Hypertension is one of the major complications in neurofibromatosis type 1 (NF1). It is known to be caused by renal artery stenosis or pheochromocytoma. However, more than half of hypertensive patients with NF1 do not have either disorder. We report here on a 13‐year‐old male with NF1 who had hypertension and a stenosis of the right renal artery associated with elevated renal vein renin on the diseased side. He underwent percutaneous transluminal renal angioplasty. In spite of successful dilation of the artery and normalized renin level, high blood pressure persisted beyond 6 months requiring antihypertensive medication. His wide pulse pressure suggested arterial stiffness due to NF1 vasculopathy. We posit that the cause of hypertension in this patient was considered to be arterial stiffness ascribed to NF1 vasculopathy rather than renal artery stenosis. Increased pulse pressure supports the hypothesis. This marker of arterial stiffness can be assessed non‐invasively and should be evaluated routinely in NF1. © 2013 Wiley Periodicals, Inc.