Increased arterial stiffness causing resistant hypertension in an adolescent with Neurofibromatosis type 1.
10.7180/kmj.2018.33.1.105
- Author:
Min Jung CHO
1
Author Information
1. Department of Pediatrics, Pusan National University Hospital, Busan, Korea. mjchomd@gmail.com
- Publication Type:Case Report
- Keywords:
Arterial stiffness;
Hypertension;
Neurofibromatosis type 1;
Vasculopathy
- MeSH:
Adolescent*;
Humans;
Hypertension*;
Male;
Methods;
Neurofibroma;
Neurofibromatoses*;
Neurofibromatosis 1*;
Pheochromocytoma;
Pulse Wave Analysis;
Renal Artery Obstruction;
Vascular Stiffness*
- From:Kosin Medical Journal
2018;33(1):105-109
- CountryRepublic of Korea
- Language:English
-
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.
