Renovascular disease and hypertension in children with neurofibromatosis

E. Fossali, E. Signorini, R. C. Intermite, E. Casalini, A. Lovaria, M. M. Maninetti, L. N. Rossi

Research output: Contribution to journalArticle

Abstract

Neurofibromatosis type 1 (NF1) is associated with vascular lesions, such as renal artery stenosis, and secondary hypertension. The real prevalence is largely unknown, particularly in children. We observed 27 patients with NF1, mean age 12.8 years (range 4.2-24 years), for 2-10 years to assess the association of NF1 with vascular abnormalities and secondary hypertension. Patients were studied with angiography, 24-h blood pressure monitoring, a captopril test, and Doppler ultrasonography of aorta and renal arteries. The prevalence of hypertension was 18.5%; 61.5% of patients studied with angiography had vascular lesions, half of whom were apparently normotensive. However, they had abnormal 24-h blood pressure monitoring, which was a first sign of poor blood pressure control. Those patients with severe hypertension (11.1%) were successfully treated with percutaneous transluminal angioplasty (PTA); stenosis recurred in 2 of 3 patients after a 2-year follow-up period, and was responsive to drugs. We conclude that hypertension is a frequent complication of NF1 in pediatric patients, it is usually secondary to typical vascular lesions, and requires careful followup. Ambulatory blood pressure monitoring (24-h) is a sensitive method for detecting initial alterations of the blood pressure pattern. PTA may be an effective treatment in this condition.

Original languageEnglish
Pages (from-to)806-810
Number of pages5
JournalPediatric Nephrology
Volume14
Issue number8-9
DOIs
Publication statusPublished - Aug 2000

Keywords

  • Ambulatory blood pressure monitoring
  • Hypertension
  • Neurofibromatosis type 1
  • Percutaneous transluminal angioplasty
  • Renal artery stenosis

ASJC Scopus subject areas

  • Nephrology
  • Pediatrics, Perinatology, and Child Health

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  • Cite this

    Fossali, E., Signorini, E., Intermite, R. C., Casalini, E., Lovaria, A., Maninetti, M. M., & Rossi, L. N. (2000). Renovascular disease and hypertension in children with neurofibromatosis. Pediatric Nephrology, 14(8-9), 806-810. https://doi.org/10.1007/s004679900260