NR AWMB
AU Pocchiari,M.; Puopulo,M.
TI Epidemiology of human transmissible spongiform encephalopathies
QU International Conference - Prion 2006: Strategies, advances and trends towards protection of society - 3.10.-6.10.2006, Torino, Italy, Lingotto Conference Centre - Oral sessions ORAL-05
PT Konferenz-Vortrag
AB
An international study of the epidemiologic characteristics of human transmissible spongiform encephalopathy (TSE) diseases was established in 1993 and included national registries in France, Germany, Italy, the Netherlands, Slovakia, and the United Kingdom. In 1997, the study was extended to Australia, Austria, Canada, Spain, and Switzerland. Data were pooled from all participating countries for the years 1993 to 2004 and included deaths from definite or probable TSE diseases of all etiologic subtypes. Five thousand six hundred fifteen cases were available for analysis and included 4,727 cases of sporadic Creutzfeldt-Jakob disease (CJD), 560 genetic cases, 170 iatrogenic cases, and 158 variant cases. The overall annual mortality rate between 1999 and 2004 was 1.68 per million for all cases and 1.42 per million for sporadic CJD. There was heterogeneity in the distribution of TSE cases by etiologic subtype.
Data for the analyses of predictors of survival were available in sporadic (n = 4618), iatrogenic (n = 163) and variant Creutzfeldt-Jakob disease (n = 158), and in cases associated with mutations of the prion protein gene (n=530). Overall, survival for each disease type was assessed by the Kaplan- Meier method and the multivariate analyses by the Cox proportional hazards model.
AD EUROCJD surveillance and Department of Cell Biology and Neurosciences, Istituto Superiore di Sanità, Rome, Italy. E-mail: pocchia@iss.it
SP englisch
PO Italien