Percutaneous transluminal renal angioplasty in neurofibromatosis

Fossali, Emilio F.; Minoja, M; Intermite, R.; Spreafico, Carlo; Casalini, Eli D.; Sereni, F

doi:10.1007/bf00860959

Cited by 14 publications

(13 citation statements)

References 9 publications

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“…It is generally accepted that NF lesions respond poorly to PTA, presumably because of the tough fibrotic tissue involved that is refractory to dilatation [13,19,22,23]; poor results are sometimes obtained by PTA in middle-aortic syndrome and in ostial lesions [22,32]. Nonetheless, some authors have used this method in NF1 [3,5,9,15,16], and encouraging results (67% success rate) or at least temporary benefits were obtained. Our results, in accordance with other reports, suggest the use of this technique in patients with NF1 and vascular lesions.…”

Section: Discussionmentioning

confidence: 95%

Renovascular disease and hypertension in children with neurofibromatosis

Fossali

Signorini

Intermite

et al. 2000

Pediatric Nephrology

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114

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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 follow-up. 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.

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Section: Discussionmentioning

confidence: 95%

Renovascular disease and hypertension in children with neurofibromatosis

Fossali

Signorini

Intermite

et al. 2000

Pediatric Nephrology

Self Cite

114

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“…37 Renal artery angioplasty for stenotic lesions appears to have a lower success rate in NF1 patients than in non-NF1 patients. 38 Surgical revascularization or nephrectomy frequently produces improvement in blood pressure in such patients, but nephrectomy should be reserved for those with parenchymal lesions or severe disease of the renal artery that prevents grafting. The high incidence of essential hypertension among NF1 patients probably contributes to surgical treatment failures because essential hypertension cannot be diagnosed with certainty on a prospective basis.…”

Section: Managementmentioning

confidence: 99%

Cardiovascular disease in neurofibromatosis 1: Report of the NF1 Cardiovascular Task Force

Friedman

Arbiser

Epstein

et al. 2002

Genetics in Medicine

351

357

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Purpose: Patients with neurofibromatosis 1 (NF1) are at increased risk for a variety of cardiovascular disorders, but the natural history and pathogenesis of these abnormalities are poorly understood. Methods: The National Neurofibromatosis Foundation convened an expert task force to review current knowledge about cardiovascular manifestations of NF1 and to make recommendations regarding clinical management and research priorities related to these features of the disease. Results: This report summarizes the NF1 Cardiovascular Task Force's current understanding of vasculopathy, hypertension, and congenital heart defects that occur in association with Key Words: neurofibromatosis 1, vasculopathy, hypertension, congenital heart defects Neurofibromatosis 1 (NF1), an autosomal dominant disease, is one of the most common mendelian disorders. 1 It is characterized by extremely variable expressivity, but most patients have café-au-lait spots, intertriginous freckling, dermal and plexiform neurofibromas, and learning disabilities. 2 People with NF1 may also develop cardiac and vascular disease, but the frequency, natural history, and pathogenesis of these abnormalities are uncertain. The National Neurofibromatosis Foundation convened an expert panel, the NF1 Cardiovascular Task Force, to review current knowledge about the cardiovascular manifestations of NF1 and to make recommendations regarding clinical management and research priorities. This is the report of that Task Force.Neurofibromas, the characteristic tumors of NF1, can develop within the heart, obstruct blood flow in the heart or major vessels by compression or invasion, or erode a vessel and cause hemorrhage. Fortunately, these are rare complications. This report will concentrate on the three most common cardiovascular manifestations of NF1, viz., vasculopathy, hypertension, and congenital heart defects.People with NF1 constitute a substantial fraction of all patients with dysplastic renal artery stenosis, 3 early-onset cerebrovascular disease, 4 or pheochromocytomas, 5 and cardiovascular disease is a frequent cause of premature death in individuals with NF1. Sørensen and associates 6 found that myocardial infarction and cerebrovascular accidents often occurred at a younger than expected age among NF1 patients. Zöller et al. 7 reported that cardiovascular disease, hemorrhage, and embolism were frequent causes of death in 70 adult NF1 patients who were followed for 12 years. Hypertension was significantly associated with mortality, and the mean age at death among the NF1 patients was approximately 14 years younger than expected. The median age of death reported on death certificates of 3,253 individuals with probable NF1 was approximately 15 years less than expected in another study. 8 Diagnoses suggestive of NF1 vasculopathy were listed 7.2 times more often than expected among NF1 patients Ͻ 30 years old and 2.2 times more often than expected among those who were 30 to 40 years old at the time of death. NF1 VASCULOPATHY Epidemiology and clinical featuresNF1 c...

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“…It is generally felt that stenoses involving the renal ostium respond poorly to PTA. There have been mixed reports of the effectiveness of PTA in NF1 with success rates of approximately 30% [3][4][5][6][7][8][9][10][11][12][13][14][15][16]. Reasons cited for the poor outcome included position of the stenosis and the variable extent of fibrotic tissue involved [9].…”

Section: Introductionmentioning

confidence: 99%