title: Persistent hypertension despite successful dilation of a stenotic renal artery in a boy with neurofibromatosis type 1.
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.
authors.affiliation: Division of Neurology, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan.
authors.forename: Keisuke
authors.initials: K
authors.lastname: Ueda
authors.forename: Midori
authors.initials: M
authors.lastname: Awazu
authors.forename: Yoriko
authors.initials: Y
authors.lastname: Konishi
authors.forename: Toshiki
authors.initials: T
authors.lastname: Takenouchi
authors.forename: Sachiko
authors.initials: S
authors.lastname: Shimozato
authors.forename: Kenjiro
authors.initials: K
authors.lastname: Kosaki
authors.forename: Takao
authors.initials: T
authors.lastname: Takahashi
mesh_terms: D000293:Adolescent; D017130:Angioplasty / Q000379:methods*; D000959:Antihypertensive Agents / Q000627:therapeutic use; D001794:Blood Pressure; D003937:Diagnosis, Differential; D006801:Humans; D006978:Hypertension, Renovascular / Q000188:drug therapy / Q000209:etiology* / Q000628:therapy; D008297:Male; D009456:Neurofibromatosis 1 / Q000150:complications*; D012077:Renal Artery / Q000473:pathology*; D012078:Renal Artery Obstruction / Q000150:complications* / Q000188:drug therapy / Q000628:therapy
chemical_list: D000959:Antihypertensive Agents