Clinical risk factors for mortality in patients with neurofibromatosis 1: a cohort study of 378 patients.
OBJECTIVE
To identify the main clinical features associated with mortality in patients with neurofibromatosis 1.


METHODS
Cohort study.


METHODS
Referral center for neurofibromatosis.


METHODS
Three hundred seventy-eight patients with neurofibromatosis 1 who had more than 1 year of follow-up in the center.


METHODS
Mortality. Clinical features, especially dermatological, were evaluated as potential factors associated with mortality.


RESULTS
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).


CONCLUSIONS
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.
Khosrotehrani
Kiarash
K
Department of Dermatology, Hôpital Henri-Mondor, Assistance Publique-Hôpitaux de Paris, Créteil, France.
Bastuji-Garin
Sylvie
S
Zeller
Jacques
J
Revuz
Jean
J
Wolkenstein
Pierre
P
D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000368:Aged; D001707:Biopsy, Needle; D002423:Cause of Death; D002648:Child; D015331:Cohort Studies; D016001:Confidence Intervals; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D009367:Neoplasm Staging; D009456:Neurofibromatosis 1 / Q000401:mortality* / Q000473:pathology* / Q000628:therapy; D016017:Odds Ratio; D011336:Probability; D012042:Registries; D012307:Risk Factors; D017678:Sex Distribution; D012878:Skin Neoplasms / Q000401:mortality* / Q000473:pathology* / Q000628:therapy; D016019:Survival Analysis