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Case Report
Increased arterial stiffness causing resistant hypertension in an adolescent with Neurofibromatosis type 1
Min Jung Cho
Kosin Medical Journal 2018;33(1):105-109.
DOI: https://doi.org/10.7180/kmj.2018.33.1.105
Published online: January 21, 2018

Department of Pediatrics, Pusan National University Hospital, Busan, Korea

Corresponding Author: Min Jung Cho, Department of Pediatrics, Pusan National University Hospital, 179, Gudeok-ro, Seo-gu, Busan 49241, Korea Tel: +82-51-240-7800 Fax: +82-51-248-6205 E-mail: mjchomd@gmail.com
• Received: August 23, 2017   • Accepted: September 27, 2017

Copyright © 2018 Kosin University School of Medicine Proceedings

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) 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 is an autosomal dominant genetic disorder characterized by the presence of café au lait spots, axillary and inguinal freckling, Lisch nodules, and neurofibromas. Hypertension is a relatively frequent complication, usually caused by renal artery stenosis or pheochromocytomas. We describe the case of a 15-year-old boy with neurofibromatosis type 1 who was also diagnosed with resistant hypertension. Despite an extensive evaluation, the etiology of his hypertension remained indeterminate. Estimation of the brachial-ankle pulse wave velocity and ambulatory arterial stiffness index could validate the existence of arterial stiffness. Further, a combination of carvedilol and angiotension receptor blockers was administered, which successfully controlled his resistant hypertension. We propose that the estimation of the brachial-ankle pulse wave velocity measure and ambulatory arterial stiffness index is a noninvasive method, and these two parameters are relatively simple tools that can be used for the detection of arterial stiffness due to neurofibromatosis type 1-related vasculopathy.
Fig. 1.
Brachial-ankle pulse wave velocities. Markedly elevated for his age; 1305 cm/s for right and 1390 cm/s for left
kmj-33-105f1.jpg
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      Increased arterial stiffness causing resistant hypertension in an adolescent with Neurofibromatosis type 1
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      Fig. 1. Brachial-ankle pulse wave velocities. Markedly elevated for his age; 1305 cm/s for right and 1390 cm/s for left
      Increased arterial stiffness causing resistant hypertension in an adolescent with Neurofibromatosis type 1

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