{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T07:09:44Z","timestamp":1791011384948,"version":"4.1.0"},"reference-count":90,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2005,12,17]],"date-time":"2005-12-17T00:00:00Z","timestamp":1134777600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["pathsocjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Journal of Pathology"],"published-print":{"date-parts":[[2006,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>In the late 1980s and early 1990s, there was widespread exposure of the UK population to bovine spongiform encephalopathy (BSE)\u2010contaminated food products, which has led to over 150 deaths from variant Creutzfeldt\u2013Jakob disease (vCJD). Although the pathogenesis in humans is not fully understood, data from animal models and, to a lesser extent, patients with vCJD suggest that oral exposure to BSE is rapidly followed by accumulation of PrP<jats:sup>res<\/jats:sup> in gut\u2010associated lymphoid tissue, then, after haematogenous spread, throughout the lymphoreticular system. Spread to the central nervous system may not occur for several years, but blood from individuals in the pre\u2010clinical phase appears to be able to transmit disease. The incidence of vCJD has remained low and is in decline, but it is known from iatrogenic CJD and kuru that human prion disease can have incubation periods of up to 40 years. Cases of vCJD are therefore likely to occur for many more years and alternative phenotypes may develop in individuals with different <jats:italic>PRNP<\/jats:italic> genotypes to those seen to date. Studies in transgenic mice have shown that sub\u2010clinical infection is frequent following oral exposure to BSE and a study looking at the accumulation of PrP in anonymized human lymphoid tissue samples found positive cases. There are likely to be a number of asymptomatic \u2018carriers\u2019 of disease within the UK and although it is unclear whether these individuals will develop clinical disease, there is a potential for iatrogenic spread to others. These uncertainties highlight the importance of developing a reliable blood test for vCJD and the continued need for surveillance. Copyright \u00a9 2006 Pathological Society of Great Britain and Ireland. Published by John Wiley &amp; Sons, Ltd.<\/jats:p>","DOI":"10.1002\/path.1880","type":"journal-article","created":{"date-parts":[[2005,12,17]],"date-time":"2005-12-17T12:50:57Z","timestamp":1134823857000},"page":"134-141","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":67,"title":["Pathogenesis and prevalence of variant Creutzfeldt\u2013Jakob disease"],"prefix":"10.1002","volume":"208","author":[{"given":"David 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