Persistent Hypertension Despite Successful Dilation of a Stenotic Renal Artery in a Boy With Neurofibromatosis Type 1

Keisuke Ueda, Midori Awazu, Yoriko Konishi, Toshiki Takenouchi, Sachiko Shimozato, Kenjiro Kosaki, Takao Takahashi

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)1154-1157
Number of pages4
JournalAmerican Journal of Medical Genetics, Part A
Volume161
Issue number5
DOIs
Publication statusPublished - 2013 May 1

Keywords

  • Arterial stiffness
  • Neurofibromatosis type 1
  • Percutaneous transluminal renal angioplasty
  • Pulse pressure
  • Pulse wave velocity
  • Renal artery stenosis
  • Vasculopathy

ASJC Scopus subject areas

  • Genetics
  • Genetics(clinical)

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