Expert judgement and re-elicitation for prion disease risk uncertainties

Much uncertainty surrounds transmission of transmissible spongiform encephalopathies (TSEs) through blood and blood derived products. A first expert elicitation with 14 experts was conducted in March 2008, and a second re-elicitation involving 11 experts was held a year later in March 2009. Both expert groups were calibrated using a series of seed questions for which values are known, and then were asked to provide their individual judgements on a set of seven target questions for which values are not known or have not been determined through conventional research. Questions dealing with uncertainty of TSE prevalence, genotype effects, susceptibility, and infectivity were answered by the experts. Elicitation can be used to obtain quantitative values for parameters affecting prion uncertainty gaps. We show that the method is amenable to re-elicitation over time allowing refinement of expert opinion as new knowledge becomes available for improved risk assessments where data gaps continue to exist.

[1]  J. Childs,et al.  Ecological Factors Associated with West Nile Virus Transmission, Northeastern United States , 2013 .

[2]  Susie ElSaadany,et al.  Expert Elicitation for the Judgment of Prion Disease Risk Uncertainties , 2011, Journal of toxicology and environmental health. Part A.

[3]  Azra C. Ghani,et al.  Uncertainty in the Tail of the Variant Creutzfeldt-Jakob Disease Epidemic in the UK , 2010, PloS one.

[4]  S. Love,et al.  Variant CJD infection in the spleen of a neurologically asymptomatic UK adult patient with haemophilia , 2010, Haemophilia : the official journal of the World Federation of Hemophilia.

[5]  W. Aspinall A route to more tractable expert advice , 2010, Nature.

[6]  J. Ironside,et al.  Prevalence of disease related prion protein in anonymous tonsil specimens in Britain: cross sectional opportunistic survey , 2009, BMJ : British Medical Journal.

[7]  A. Farrugia Globalisation and blood safety. , 2009, Blood reviews.

[8]  M. Turner,et al.  An update on the assessment and management of the risk of transmission of variant Creutzfeldt‐Jakob disease by blood and plasma products , 2009, British journal of haematology.

[9]  Claudio J. Verzilli,et al.  Genetic risk factors for variant Creutzfeldt–Jakob disease: a genome-wide association study , 2009, The Lancet Neurology.

[10]  M. Alpers,et al.  A clinical study of kuru patients with long incubation periods at the end of the epidemic in Papua New Guinea , 2008, Philosophical Transactions of the Royal Society B: Biological Sciences.

[11]  F. Chianini,et al.  Experimental transmission of bovine spongiform encephalopathy to European red deer (Cervus elaphus elaphus) , 2008, BMC veterinary research.

[12]  Roger M. Cooke,et al.  TU Delft expert judgment data base , 2008, Reliab. Eng. Syst. Saf..

[13]  P. Gambetti,et al.  Evaluation of the Human Transmission Risk of an Atypical Bovine Spongiform Encephalopathy Prion Strain , 2008, Journal of Virology.

[14]  K. Wilson The Krever Commission — 10 years later , 2007, Canadian Medical Association Journal.

[15]  M. Bishop,et al.  The role of host PrP in Transmissible Spongiform Encephalopathies. , 2007, Biochimica et biophysica acta.

[16]  W. Schulz-Schaeffer,et al.  Accumulation of Pathological Prion Protein PrPSc in the Skin of Animals with Experimental and Natural Scrapie , 2007, PLoS pathogens.

[17]  J. Wilesmith,et al.  Bovine spongiform encephalopathy: the effect of oral exposure dose on attack rate and incubation period in cattle. , 2007, The Journal of general virology.

[18]  L. Manuelidis A 25 nm virion is the likely cause of transmissible spongiform encephalopathies , 2007, Journal of cellular biochemistry.

[19]  J. McGeehan,et al.  Insoluble Aggregates and Protease-resistant Conformers of Prion Protein in Uninfected Human Brains* , 2006, Journal of Biological Chemistry.

[20]  P. Bennett,et al.  vCJD and blood transfusion: risk assessment in the United Kingdom. , 2006, Transfusion clinique et biologique : journal de la Societe francaise de transfusion sanguine.

[21]  J. Castilla,et al.  Presymptomatic Detection of Prions in Blood , 2006, Science.

[22]  C. Sigurdson,et al.  Prions in Skeletal Muscles of Deer with Chronic Wasting Disease , 2006, Science.

[23]  J. Ironside,et al.  Predicting susceptibility and incubation time of human-to-human transmission of vCJD , 2005, The Lancet Neurology.

[24]  K. Abid,et al.  Protein misfolding cyclic amplification for diagnosis and prion propagation studies. , 2006, Methods in enzymology.

[25]  Organización Mundial de la Salud WHO guidelines on tissue infectivity distribution in transmissible spongiform encephalopathies , 2006 .

[26]  C. Herzog,et al.  PrPTSE Distribution in a Primate Model of Variant, Sporadic, and Iatrogenic Creutzfeldt-Jakob Disease , 2005, Journal of Virology.

[27]  F. Bastian,et al.  Slow virus disease: Deciphering conflicting data on the transmissible spongiform encephalopathies (TSE) also called prion diseases , 2005, Microscopy research and technique.

[28]  A. Aguzzi,et al.  PrPSc in mammary glands of sheep affected by scrapie and mastitis , 2005, Nature Medicine.

[29]  A. Kincaid,et al.  Interspecies Transmission of Chronic Wasting Disease Prions to Squirrel Monkeys (Saimiri sciureus) , 2005, Journal of Virology.

[30]  Ken Chen,et al.  Chronic Wasting Disease of Elk: Transmissibility to Humans Examined by Transgenic Mouse Models , 2005, The Journal of Neuroscience.

[31]  Paul Clarke,et al.  Projections of the future course of the primary vCJD epidemic in the UK: inclusion of subclinical infection and the possibility of wider genetic susceptibility , 2005, Journal of The Royal Society Interface.

[32]  F. Cohen,et al.  Strain-specified characteristics of mouse synthetic prions. , 2005, Proceedings of the National Academy of Sciences of the United States of America.

[33]  P. Sneath Estimation of the size of the vCJD epidemic , 2004, Antonie van Leeuwenhoek.

[34]  Mark Penney,et al.  Prevalence of lymphoreticular prion protein accumulation in UK tissue samples , 2004, The Journal of pathology.

[35]  E. Vamvakas,et al.  Proceedings of a consensus conference: the screening of blood donors for variant CJD. , 2004, Transfusion medicine reviews.

[36]  A. Buschmann,et al.  Atypical scrapie cases in Germany and France are identified by discrepant reaction patterns in BSE rapid tests. , 2004, Journal of virological methods.

[37]  S. Supattapone Prion protein conversion in vitro , 2004, Journal of Molecular Medicine.

[38]  F. Tagliavini,et al.  Identification of a second bovine amyloidotic spongiform encephalopathy: molecular similarities with sporadic Creutzfeldt-Jakob disease. , 2004, Proceedings of the National Academy of Sciences of the United States of America.

[39]  J Mackenzie,et al.  Possible transmission of variant Creutzfeldt-Jakob disease by blood transfusion , 2004, The Lancet.

[40]  M. Porta,et al.  Why aren't We More Ahead? The Risk of Variant Creutzfeldt–Jakob Disease from Eating Bovine Spongiform Encephalopathy-Infected Foods: Still Undetermined , 2003, European Journal of Epidemiology.

[41]  T. Sata,et al.  Atypical proteinase K-resistant prion protein (PrPres) observed in an apparently healthy 23-month-old Holstein steer. , 2003, Japanese journal of infectious diseases.

[42]  P. Sarradin,et al.  Cases of scrapie with unusual features in Norway and designation of a new type, Nor98 , 2003, Veterinary Record.

[43]  Y. Agid,et al.  Distribution of codon 129 genotype in human growth hormone-treated CJD patients in France and the UK , 2003, The Lancet.

[44]  J. Collinge,et al.  Pathogenesis: HLA-DQ7 antigen and resistance to variant CJD , 2001, Nature.

[45]  C. Masters,et al.  Gerstmann–Sträussler–Scheinker syndrome,fatal familial insomnia, and kuru: a review ofthese less common human transmissiblespongiform encephalopathies , 2001, Journal of Clinical Neuroscience.

[46]  S. Fallows Scientific Steering Committee , 2000 .

[47]  J. Aiken,et al.  Adaptation and Selection of Prion Protein Strain Conformations following Interspecies Transmission of Transmissible Mink Encephalopathy , 2000, Journal of Virology.

[48]  A. Ghani,et al.  Assessment of the prevalence of vCJD through testing tonsils and appendices for abnormal prion protein , 2000, Proceedings of the Royal Society of London. Series B: Biological Sciences.

[49]  Steering Committee PRELIMINARY OPINION ORAL EXPOSURE OF HUMANS TO THE BSE AGENT: INFECTIVE DOSE AND SPECIES BARRIER , 2000 .

[50]  J. Hope Spongiform encephalopathies: Breech-birth prions , 1999, Nature.

[51]  A. Alpérovitch,et al.  Codon 129 prion protein genotype and sporadic Creutzfeldt-Jakob disease , 1999, The Lancet.

[52]  P. Brown,et al.  Natural and experimental oral infection of nonhuman primates by bovine spongiform encephalopathy agents. , 1999, Proceedings of the National Academy of Sciences of the United States of America.

[53]  J. Aiken,et al.  The host range of chronic wasting disease is altered on passage in ferrets. , 1998, Virology.

[54]  C. Allin Cornell,et al.  Use of Technical Expert Panels: Applications to Probabilistic Seismic Hazard Analysis * , 1998 .

[55]  S. Cousens,et al.  Transmissions to mice indicate that ‘new variant’ CJD is caused by the BSE agent , 1997, Nature.

[56]  Robert J. Budnitz,et al.  Recommendations for probabilistic seismic hazard analysis: Guidance on uncertainty and use of experts , 1997 .

[57]  A. Hofman,et al.  A new variant of Creutzfeldt-Jakob disease in the UK , 1996, The Lancet.

[58]  H. Fraser,et al.  Transmission of bovine spongiform encephalopathy and scrapie to mice. , 1992, The Journal of general virology.

[59]  P Brown,et al.  Fatal familial insomnia and familial Creutzfeldt-Jakob disease: disease phenotype determined by a DNA polymorphism. , 1992, Science.

[60]  R. Cooke Experts in Uncertainty: Opinion and Subjective Probability in Science , 1991 .

[61]  T. Gruffydd-Jones,et al.  Naturally occurring scrapie-like spongiform encephalopathy in five domestic cats , 1991, Veterinary Record.

[62]  S. Lehmann,et al.  Epidemiological and experimental studies on a new incident of transmissible mink encephalopathy. , 1991, The Journal of general virology.

[63]  G. Wells,et al.  Spongiform encephalopathy in an arabian oryx (Oryx leucoryx) and a greater kudu (Tragelaphus strepsiceros) , 1990, Veterinary Record.

[64]  Kimberlin Rh,et al.  Bovine spongiform encephalopathy. , 1992, The Veterinary record.

[65]  M. Jeffrey,et al.  Spongiform Encephalopathy in a Nyala (Tragelaphus angasi) , 1988, Veterinary pathology.

[66]  M. Jeffrey,et al.  A novel progressive spongiform encephalopathy in cattle , 1987, Veterinary Record.

[67]  S. Prusiner Novel proteinaceous infectious particles cause scrapie. , 1982, Science.

[68]  Gajdusĕk Dc,et al.  Experimental subacute spongiform virus encephalopathies in primates and other laboratory animals. , 1973 .

[69]  D. Gajdusek,et al.  Experimental Subacute Spongiform Virus Encephalopathies in Primates and Other Laboratory Animals , 1973, Science.

[70]  J. R. Greig Scrapie in sheep. , 1950, Journal of comparative pathology.

[71]  The nature and cause of BSE , 2022 .