Increased arterial stiffness causing resistant hypertension in an adolescent with Neurofibromatosis type 1

Article information

Kosin Med J. 2018;33(1):105-109

Publication date (electronic) : 2018 January 21

doi : https://doi.org/10.7180/kmj.2018.33.1.105

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 2017 August 23; 2017 September 19; Accepted 2017 September 27.

Abstract

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.

Keywords: Arterial stiffness; Hypertension; Neurofibromatosis type 1; Vasculopathy

Fig. 1.

Brachial-ankle pulse wave velocities. Markedly elevated for his age; 1305 cm/s for right and 1390 cm/s for left

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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