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KMID : 1141320180330010105
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Kosin Medical Journal
2018 Volume.33 No. 1 p.105 ~ p.109
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Increased arterial stiffness causing resistant hypertension in an adolescent with Neurofibromatosis type 1
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Cho Min-Jung
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Abstract
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Neurofibromatosis type 1 is an autosomal dominant genetic disorder characterized by the presence of cafe 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.
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KEYWORD
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Arterial stiffness, Hypertension, Neurofibromatosis type 1, Vasculopathy
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