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Clinical Risk Factors for Mortality in Patients With Neurofibromatosis 1

2003; American Medical Association; Volume: 139; Issue: 2 Linguagem: Inglês

10.1001/archderm.139.2.187

1538-3652

Kiarash Khosrotehrani, Sylvie Bastuji‐Garin, J Zeller, J. Revuz, P. Wolkenstein,

Soft tissue tumors and treatment

To identify the main clinical features associated with mortality in patients with neurofibromatosis 1.Cohort study.Referral center for neurofibromatosis.Three hundred seventy-eight patients with neurofibromatosis 1 who had more than 1 year of follow-up in the center.Mortality. Clinical features, especially dermatological, were evaluated as potential factors associated with mortality.Factors associated independently with mortality were the presence of subcutaneous neurofibromas (odds ratio, 10.8; 95% confidence interval, 2.1-56.7; P<.001), the absence of cutaneous neurofibromas (odds ratio, 5.3; 95% confidence interval, 1.2-25.0; P =.03), and facial asymmetry (odds ratio, 11.4; 95% confidence interval, 2.6-50.2; P<.01).Some features that can be found by a routine clinical examination are associated with mortality in patients with neurofibromatosis 1. Clinical follow-up should be focused on patients with subcutaneous neurofibromas and/or the absence of cutaneous neurofibromas and/or facial asymmetry.