Cardiovascular disease in neurofibromatosis 1: Report of the NF1 cardiovascular task force

被引:271
作者
Friedman, JM
Arbiser, J
Epstein, JA
Gutmann, DH
Huot, SJ
Lin, AE
McManus, B
Korf, BR
机构
[1] Univ British Columbia, Dept Med Genet, Vancouver, BC V6T 1Z3, Canada
[2] Univ British Columbia, Dept Pathol & Lab Med, Vancouver, BC V6T 1Z3, Canada
[3] Emory Univ, Dept Dermatol, Sch Med, Atlanta, GA 30322 USA
[4] Univ Penn, Dept Med, Div Cardiovasc, Philadelphia, PA 19104 USA
[5] Washington Univ, Sch Med, Dept Neurol, St Louis, MO 63110 USA
[6] Yale Univ, Sch Med, Dept Internal Med, New Haven, CT 06510 USA
[7] Massachusetts Gen Hosp, Teratol Unit, Boston, MA 02114 USA
[8] Harvard Univ, Sch Med, Partners Ctr Human Genet, Boston, MA USA
关键词
neurofibromatosis; 1; vasculopathy; hypertension; congenital heart defects;
D O I
10.1097/00125817-200205000-00002
中图分类号
Q3 [遗传学];
学科分类号
071007 ; 090102 ;
摘要
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 NF1. Recommendations are made regarding routine surveillance for cardiovascular disease and diagnostic evaluation and management of cardiovascular disorders in individuals with NF1. Conclusion: Our understanding of the natural history and pathogenesis of cardiovascular disease in NF1 has improved substantially in the past few years, but many clinically important questions remain unanswered.
引用
收藏
页码:105 / 111
页数:7
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