There is no conflict of interest to disclose.
Persistent Hypertension Despite Successful Dilation of a Stenotic Renal Artery in a Boy With Neurofibromatosis Type 1
Article first published online: 5 APR 2013
Copyright © 2013 Wiley Periodicals, Inc.
American Journal of Medical Genetics Part A
Volume 161, Issue 5, pages 1154–1157, May 2013
How to Cite
2012. Persistent hypertension despite successful dilation of a stenotic renal artery in a boy with neurofibromatosis type 1. Am J Med Genet Part A 161A:1154–1157., , , , , , .
- Issue published online: 22 APR 2013
- Article first published online: 5 APR 2013
- Manuscript Accepted: 4 DEC 2012
- Manuscript Received: 27 APR 2012
- neurofibromatosis type 1;
- arterial stiffness;
- renal artery stenosis;
- percutaneous transluminal renal angioplasty;
- pulse pressure;
- pulse wave velocity
Hypertension is one of the major complications in neurofibromatosis type 1 (NF1). It is known to be caused by renal artery stenosis or pheochromocytoma. However, more than half of hypertensive patients with NF1 do not have either disorder. We report here on a 13-year-old male with NF1 who had hypertension and a stenosis of the right renal artery associated with elevated renal vein renin on the diseased side. He underwent percutaneous transluminal renal angioplasty. In spite of successful dilation of the artery and normalized renin level, high blood pressure persisted beyond 6 months requiring antihypertensive medication. His wide pulse pressure suggested arterial stiffness due to NF1 vasculopathy. We posit that the cause of hypertension in this patient was considered to be arterial stiffness ascribed to NF1 vasculopathy rather than renal artery stenosis. Increased pulse pressure supports the hypothesis. This marker of arterial stiffness can be assessed non-invasively and should be evaluated routinely in NF1. © 2013 Wiley Periodicals, Inc.