ArticleBMJ (Clinical research ed.)2013
Prevalent abnormal prion protein in human appendixes after bovine spongiform encephalopathy epizootic: large scale survey.
Article in BMJ (Clinical research ed.), 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 117 papers, 1 of them a synthesis that pooled it.
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117 citing papers in PubMed, 1 synthesis or guideline pooled it, 300 citations in OpenAlex.
- Interventions to reduce the risk of surgically transmitted Creutzfeldt-Jakob disease: a cost-effective modelling review.Health technology assessment (Winchester, England) · 2020Pooled it
- Population screening for variant Creutzfeldt-Jakob disease using a novel blood test: diagnostic accuracy and feasibility study.JAMA neurology · 2014Trial
- Variant Creutzfeldt-Jakob disease surveillance in Spain, 1993-2021.Frontiers in public health · 2026Article
- Estimating future variant Creutzfeldt-Jakob disease cases in the UK: a cohort-based probabilistic model.The Lancet regional health. Europe · 2025Article
- Human PrP E219K: a new and promising substrate for robust RT-QuIC amplification of human prions with potential for strain discrimination.Microbiology spectrum · 2025Article
- No evidence of subclinical infection in sheep surviving oral challenge with prions.The Journal of general virology · 2025Article
- Isolation of a novel human prion strain from a PRNP codon 129 heterozygous vCJD patient.PLoS pathogens · 2025Article
- Prion Disease Diagnostic Biomarker Utility in Pre-symptomatic Disease.Sub-cellular biochemistry · 2025Review
- Cases of Creutzfeldt-Jakob disease in young individuals: open questions regarding aetiology.Frontiers in cellular neuroscience · 2025Article
- BSE risk posed by ruminant collagen and gelatine derived from bones.EFSA journal. European Food Safety Authority · 2024Article
- Lack of Transmission of Chronic Wasting Disease Prions to Human Cerebral Organoids.Emerging infectious diseases · 2024Article
- Enhanced Creutzfeldt-Jakob disease surveillance in the older population: Assessment of a protocol for screening brain tissue donations for prion disease.Brain pathology (Zurich, Switzerland) · 2024Article
- A review of the enhanced CJD surveillance feasibility study in the elderly in Scotland, UK.BMC geriatrics · 2024Review
- Conservation of vCJD Strain Properties After Extraction and In Vitro Propagation of PrPMolecular neurobiology · 2023Article
- Non-human primates in prion diseases.Cell and tissue research · 2023Review
- Recombinant ovine prion protein can be mutated at position 136 to improve its efficacy as an inhibitor of prion propagation.Scientific reports · 2023Article
- Development of a sensitive real-time quaking-induced conversion (RT-QuIC) assay for application in prion-infected blood.PloS one · 2023Article
- Unexpected decrease of full-length prion protein in macaques inoculated with prion-contaminated blood products.Frontiers in molecular biosciences · 2023Article
- Risk of variant Creutzfeldt-Jakob disease transmission by blood transfusion in Australia.Vox sanguinis · 2022Article
- A clinicopathological study of selected cognitive impairment cases in Lothian, Scotland: enhanced CJD surveillance in the 65 + population group.BMC geriatrics · 2022Article
57 more citing papers are in PubMed but not listed here.
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17 authors at 6 institutions in 1 country.
Funding
Abstract
objectivesTo carry out a further survey of archived appendix samples to understand better the differences between existing estimates of the prevalence of subclinical infection with prions after the bovine spongiform encephalopathy epizootic and to see whether a broader birth cohort was affected, and to understand better the implications for the management of blood and blood products and for the handling of surgical instruments.
designIrreversibly unlinked and anonymised large scale survey of archived appendix samples.
settingArchived appendix samples from the pathology departments of 41 UK hospitals participating in the earlier survey, and additional hospitals in regions with lower levels of participation in that survey. SAMPLE: 32,441 archived appendix samples fixed in formalin and embedded in paraffin and tested for the presence of abnormal prion protein (PrP).
resultsOf the 32,441 appendix samples 16 were positive for abnormal PrP, indicating an overall prevalence of 493 per million population (95% confidence interval 282 to 801 per million). The prevalence in those born in 1941-60 (733 per million, 269 to 1596 per million) did not differ significantly from those born between 1961 and 1985 (412 per million, 198 to 758 per million) and was similar in both sexes and across the three broad geographical areas sampled. Genetic testing of the positive specimens for the genotype at PRNP codon 129 revealed a high proportion that were valine homozygous compared with the frequency in the normal population, and in stark contrast with confirmed clinical cases of vCJD, all of which were methionine homozygous at PRNP codon 129.
conclusionsThis study corroborates previous studies and suggests a high prevalence of infection with abnormal PrP, indicating vCJD carrier status in the population compared with the 177 vCJD cases to date. These findings have important implications for the management of blood and blood products and for the handling of surgical instruments.
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