A Case of Moyamoya Disease Associated with Neurofibromatosis Type 1 in Patients with Renal Artery Stenosis and Hypertension.
- Author:
Young Ho SEO
1
;
Hyung Eun YIM
;
Kee Hwan YOO
Author Information
1. Department of Pediatrics, College of Medicine, Korea University, Korea. he-yim@hanmail.net
- Publication Type:Case Report
- Keywords:
Renal artery stenosis;
Hypertension;
Nneurofibromatosis 1;
Moyamoya disease
- MeSH:
Angioplasty;
Antihypertensive Agents;
Cardiovascular System;
Diagnosis;
Humans;
Hypertension*;
Kidney Failure, Chronic;
Moyamoya Disease*;
Neurocutaneous Syndromes;
Neurofibromatoses*;
Neurofibromatosis 1*;
Renal Artery Obstruction*;
Renal Artery*
- From:Journal of the Korean Society of Pediatric Nephrology
2013;17(2):143-148
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
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.
