NR AWQL
AU Will,R.G.
TI Variant CJD: 10 years after
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-01
PT Konferenz-Vortrag
AB
The identification of variant Creutzfeldt-Jakob disease (vCJD) depended on the systematic characterisation of suspected cases of human prion disease throughout the UK and comparison of data with other European countries in which systematic surveillance of CJD was also being undertaken. The collaborative study of CJD has been funded by the EU since 1993 and this has allowed accurate international data on vCJD to be provided to the scientific community, policy makers and the general public.
This has been important because vCJD is a fatal zoonosis caused by infection with the agent of bovine spongiform encephalopathy. The implications for public health have been significant because of the long interval between exposure and disease expression, the probable transmission route through the human food chain and the probability of transfusion transmission of infection. Concerns have lessened in the past few years because of the decline in BSE epidemics in most countries and the relatively limited and declining outbreak of vCJD in the UK. An important question is whether or not it is appropriate that public health concerns regarding vCJD may also be in decline.
There are many remaining scientific uncertainties. Why is there a mismatch between the extensive human exposure to the BSE agent, the estimated prevalence of infection and the actual observed number of cases of vCJD? Will there be further outbreaks related to variation in human PRNP genetic background? Will other mechanisms of transmission be identified, for example via contaminated surgical instruments or via a maternal route? Will further countries be affected and have cases caused be exposure to non-UK indigenous BSE yet been observed? Is there a possibility that newly identified forms of animal disease such as BASE or atypical scrapie have implications for human health? These important questions should be addressed before there is complacency about the potential public health implications of prion diseases and continuing international collaboration may still have important contributions to make in the study of natural diseases.
AD National CJD Surveillance Unit, Edinburgh, UK
SP englisch
PO Italien