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Journal of the Korean Society of Pediatric Nephrology 2013;17(2): 143-148. doi: https://doi.org/10.3339/jkspn.2013.17.2.143
제 1형 신경섬유종증에 합병된 모야모야병에서 신장동맥 협착을 동반한 고혈압
서영호, 임형은, 유기환
고려대학교 의과대학 소아과학교실
A Case of Moyamoya Disease Associated with Neurofibromatosis Type 1 in Patients with Renal Artery Stenosis and Hypertension
Young Ho Seo, Hyung Eun Yim, Kee Hwan Yoo
Department of Pediatrics, College of Medicine, Korea University
Corresponding Author: Hyung Eun Yim ,Tel: 031-412-5096, Fax: 031-405-8591, Email: he-yim@hanmail.net
Received: October 8, 2013;  Accepted: October 11, 2013.
This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http:// creativecommons. org/licenses/bync/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
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Neurofibromatosis type 1 (NF-1) is an autosomal dominant neurocutaneous disorder, which can affect different organs or systems of the body, including the cardiovascular system. One of the more serious aspects of the disease relates to arterial involvement. In particular, renal artery stenosis is one of the most common vascular abnormalities in patients with NF-1, and the manifestations vary, ranging from no symptoms to end-stage renal failure. Treatment usually consists of antihypertensive drugs, percutaneous transluminal angioplasty, or surgery. Other causes of hypertension should be ruled out and the patient followed up for close monitoring and proper management. We report a case of bilateral renal artery stenosis and hypertension in a patient with moyamoya disease associated with neurofibromatosis type 1. This report discusses the literature available on the current subject, its clinical features, diagnosis, and treatment.
Key words: Renal artery stenosis | Hypertension | Nneurofibromatosis 1 | Moyamoya disease
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