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一名患有与神经纤维瘤病相关的进行性肾动脉血栓形成患者的外科治疗。

Surgical treatment of a patient with progressive thrombosis of the renal artery associated with neurofibromatosis.

作者信息

Kimura H, Sato O, Deguchi J-O, Miyata T

机构信息

Division of Vascular Surgery, Department of Surgery, Graduate School of Medicine, University of Tokyo, Tokyo, Japan.

出版信息

Int Angiol. 2009 Dec;28(6):503-6.

Abstract

Pediatric renovascular hypertension (RVH) associated with neurofibromatosis 1 (NF1) is a rare entity that is often resistant to endovascular treatment. A 12-year-old girl with NF1 and hypertension presented with severe stenosis of the right main renal artery and its posterior segment, the latter of which thrombosed spontaneously, and total occlusion of the celiac artery with rare branching of the accessory middle colic artery. She underwent successful reconstruction of the anterior segment of the right renal artery, and has been free of medication for ten years. A review of the recent literature suggests that renal artery bypass remains the best treatment in pediatric RVH with NF1.

摘要

与1型神经纤维瘤病(NF1)相关的小儿肾血管性高血压(RVH)是一种罕见疾病,通常对血管内治疗耐药。一名患有NF1和高血压的12岁女孩,表现为右肾主动脉及其后段严重狭窄,后者自发形成血栓,腹腔干动脉完全闭塞,副中结肠动脉分支罕见。她接受了右肾动脉前段的成功重建,并且已经停药十年。近期文献综述表明,肾动脉搭桥术仍然是患有NF1的小儿RVH的最佳治疗方法。

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