Managing the risk of transmission of variant Creutzfeldt Jakob disease by blood products

British Journal of Haematology

Volumn 132, Issue 1, 2006, Pages 13-24

  Ludlam, Christopher A ^a   Turner, Marc L ^a  

^a ROYAL INFIRMARY OF EDINBURGH   (United Kingdom)

Author keywords

Epidemiology; Haemophilia; Safety; Transfusion; Variant Creutzfeld Jakob disease

Indexed keywords

METHIONINE; PRION PROTEIN; VALINE;

BETA SHEET; BLOOD DONOR; BLOOD TRANSFUSION; BOVINE SPONGIFORM ENCEPHALOPATHY; CARBOXY TERMINAL SEQUENCE; CLINICAL FEATURE; COLON BIOPSY; CREUTZFELDT JAKOB DISEASE; DISEASE TRANSMISSION; FOLLICULAR DENDRITIC CELL; GENETIC PREDISPOSITION; GENETIC VARIABILITY; GERSTMANN STRAUSSLER SCHEINKER SYNDROME; HUMAN; INFECTION RATE; INFECTION RISK; INTESTINE BIOPSY; MEDICAL ASSESSMENT; NEUROPATHOLOGY; NON INVASIVE MEASUREMENT; NONHUMAN; NUCLEAR MAGNETIC RESONANCE IMAGING; PATHOPHYSIOLOGY; PEYER PATCH; PLASMA; PRION DISEASE; PRIORITY JOURNAL; PROTEIN FUNCTION; PROTEIN GLYCOSYLATION; PROTEIN SECONDARY STRUCTURE; REVIEW; SCRAPIE; SCREENING TEST; SINGLE NUCLEOTIDE POLYMORPHISM; VIRUS INFECTIVITY; VIRUS REPLICATION; VIRUS STRAIN; VIRUS TRANSMISSION; ANIMAL; BLOOD; METHODOLOGY; MICROBIOLOGY;

ANIMALS; BLOOD; BLOOD DONORS; BLOOD TRANSFUSION; CREUTZFELDT-JAKOB SYNDROME; DISEASE TRANSMISSION, HORIZONTAL; HUMANS; PRION DISEASES;

EID: 30844460196     PISSN: 00071048     EISSN: 13652141     Source Type: Journal    
DOI: 10.1111/j.1365-2141.2005.05796.x     Document Type: Review

References (124)

1. Aguzzi A. Glatzel M. 2004 vCJD tissue distribution and transmission by transfusion - a worst case scenario coming true Lancet 363 411 412
2. Aguzzi A. Polymenidou M. 2004 Mammalian prion biology: one century of evolving concepts Cell 116 313 327
3. Aguzzi A. Weissmann C. 1997 Prion research: the next frontiers Nature 389 795 798
4. Anderson R.M. Donnelly C.A. Ferguson N.M. Woolhouse M.E. Wattl C.J. Udy H.J. MaWhinney S. Dunstan S.P. Southwood T.R. Wilesmith J.W. Ryan J.B. Hoinville L.J. Hillerton J.E. Austin A.R. Wells G.A. 1996 Transmission dynamics and epidemiology of BSE in British cattle Nature 382 779 788
5. Bird S.M. 2004 Recipients of blood or blood products and vCJD risk British Medical Journal 328 118 119
6. Blajchman M.A. Goldman M. Webert K.E. Vamvakas E.C. Hannon J. Delage G. 2004 Proceedings of a consensus conference: the screening of blood donors for variant CJD Transfusion Medicine Reviews 18 73 92
7. Boelle P.Y. Cesbron J.Y. Valleron A.J. 2004 Epidemiological evidence of higher susceptibility to vCJD in the young BMC Infectious Diseases 4 26
8. Brown P. 1995 Can Creutzfeldt-Jakob disease be transmitted by transfusion Current Opinion in Haematology 2 472 477
9. Brown P. 1998 On the origins of BSE Lancet 352 252 253
10. Brown P. Preece M.A. Will R.G. 1992 'Friendly fire' in medicine: hormones, homografts and Creutzfeldt-Jakob disease Lancet 340 24 27
11. Brown P. Gibbs C.J. Rodgers-Johnson P. Asher D.M. Sulima M.P. Bacote A. Goldfarb L.G. Gajduesk D.C. 1994 Human spongiform encephalopathy: the National Institutes of Health series of 300 experimentally transmitted cases Annals of Neurology 35 513 529
12. Brown D.R. Qin K. Herms J.W. Madlung A. Manson J. Strome R. Fraser P.E. Kruck T. von Bohlen A. Schulz-Schaeffer W. Giese A. Westaway D. Kretzchmar H. 1997a q10 The cellular prion protein binds copper in vivo Nature 390 684 687
13. Brown K.L. Stewart K. Fraser H. Bruce M.E. 1997b q11 Severely combined immunodeficient (SCID) mice resist infection with bovine spongiform encephalopathy Journal of General Virology 78 2707 2710
14. Brown P. Rohwer R.G. Dunstan B.C. MacAuley C. Gajdusek D.C. Drolan W.N. 1998 The distribution of infectivity in blood components and plasma derivatives in experimental models of transmissible spongiform encephalopathy Transfusion 38 810 816
15. Brown P. Cervenakova L. McShane L.M. Barber P. Rubenstein R. Drohan W.N. 1999 Further studies of blood infectivity in an experimental model of transmissible spongiform encephalopathy, with an explanation of why blood components do not transmit Creutzfeldt-Jakob disease in humans Transfusion 39 1169 1178
16. Brown P. Preece M. Brandel J.P. Sato T. McShane L. Zerr J. Fletcher A. Will R.G. Pocchairi M. Cashman N.R. d'Aignaux J.H. Cervenakova L. Frandklin J. Schonberger L.B. Collins S.J. 2000 latrogenic Creutzfeldt-Jakob disease at the millennium Neurology 55 1075 1081
17. Bruce M.E. Will R.G. Ironside J.W. McConnell L. Drummond D. Suttie A. McCardle L. Chree A. Hope J. Birkett C. Cousens S. Fraser H. Bostock C. 1997 Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent Nature 389 498 501
18. Buchanan C.R. Procco M.A. Milner R.D.G. 1991 Mortality, neoplasia and Creutzfeldt-Jakob disease in patients treated with human pituitary growth hormone in the United Kingdom British Medical Journal 302 824 828
19. Bueler H. Aguzzi A. Saller A. Greiner R.A. Autenried P. Aguet M. Weissmann C. 1993 Mice devoid of PrP are resistant to scrapie Cell 73 1339 1347
20. Burwinkel M. Holtkamp H. Baier M. 2004 Biology of infectious proteins: lessons from yeast prions Lancet 364 1471 1472
21. Casaccia P. Ladogana L. Xi Y.G. Pocchairi M. 1989 Levels of infectivity in the blood throughout the incubation period of hamster peripherally injected with scrapie Archives of Virology 108 145 149
22. Clarke P. Ghani A.C. 2005 Projections of the future course of the primary variant CJD epidemic in the UK: incidence of subclinical infection and the possibility of wider genetic susceptibility Journal of the Royal Society Interface 17 1 13
23. Collinge J. Sidle K.C.L. Meads J. Ironside J. Hill A.F. 1996 Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD Nature 383 685 690
24. Collins S. Law M.G. Fletcher A. Boyd A. Kaldor J. Masters C.L. 1999 Surgical treatment and risk of sporadic Creutzfeldt-Jakob disease: a case-control study Lancet 353 693 696
25. Collins S.J. Lawson V.A. Masters C.L. 2004 Transmissible spongiform encephalopathies Lancet 363 51 61
26. Comer P. Spouge J. 1999 Assessment of the risk of exposure to vCJD infectivity in blood and blood products Det Norske Veritus Final Report, Job No. 8288
27. Committee for Propriety Medicinal Products 1998 Position statement on new variant CJD and plasma-derived medicinal products. CPMP/201/98 European Agency for the Evaluation of Medicinal Products London
28. Cousens S.N. Vynnycky E. Zeidler M. Will R.G. Smith P.G. 1997 Predicting the CJD epidemic in humans Nature 385 197 198
29. Creange A. Gray F. Cesaro P. Adle-Biasette H. Duvoux C. Cherqui D. Bell J. Parchi P. Gambetti P. Degos J.D. 1995 Creutzfeldt-Jakob disease and liver transplantation Annals of Neurology 38 269 272
30. Creange A. Gray H. Cesaro P. Degos J.D. 1996 Pooled plasma derivatives and Creutzfeldt-Jakob disease Lancet 347 482
31. Davanipour Z. Alter M. Sobel E. Asher D.M. Gajdusek D.C. 1985 A case control study of Creutzfeldt-Jakob disease: dietary risk factors American Journal of Epidemiology 122 443 451
32. Department of Health 1998a Further precautionary measures on blood products announced Press Release 98/076 Department of Health London
33. Department of Health 1998b Government accepts advice on leucodepletion from Spongiform Encephalopathy Advisory Committee Press Release 98/295 Department of Health London
34. Deslys J.P. Jaegly A. Hullard d'Aignaux J. Mouthon F. de Villemeour T.B. Dormont D. 1998 Genotype at codon 129 and susceptibility to Creutzfeldt-Jakob disease Lancet 351 1251
35. Diringer H. 1984 Sustained viraemia in experimental hamster scrapie Archives of Virology 82 105 109
36. Esmonde T.F.G. Will R.G. Slattery J.M. Knight R. Harries-Jones R. de Silva R. Mathew W.B. 1993 Creutzfeldt Jakob disease and blood transfusion Lancet 341 205 207
37. Evatt B.L. 1998 Prions and haemophilia: assessment of risk Haemophilia 4 628 631
38. Evatt B.L. Austin H. Banhart E. Schonberger L. Sharer L. Jones R. De Armond S. 1998 Surveillance for Creutzfeldt-Jakob disease among persons with haemophilia Transfusion 38 817 820
39. Farquhar C.F. Dornan J. Somerville R.A. Tunstall A.M. Hope J. 1994 Effect of Sinc genotype, agent isolate and route of infection on the accumulation of protease-resistant PrP in non-central nervous system issues during the development of murine scrapie Journal of General Virology 75 495 504
40. Favereaux A. Quadrio I. Vital C. Perret-Liaudet A. Anne O. Laplanche J.L. Petry K.G. Vital A. 2004 Pathological prion protein spreading in the peripheral nervous system of a patient with sporadic Creutzfeldt Jakob disease Archives of Neurology 61 747 750
41. Flanagan P. Barbara J.A.J. 1996 Prion disease and blood transfusion Transfusion Medicine 6 213 215
42. Foster P. 1999 Assessment of the potential plasma fractionation processes to remove causative agents of transmissible spongiform encephalopathy Transfusion Medicine 9 3 14
43. Foster P.R. 2004 Removal of TSE agents from blood products Vox Sanguinis 87 Suppl. 2 7 10
44. Foster P.R. Welch A.G. McLean C. Griffin B.D. Hardy J.C. Bartley A. MacDonald S. Bailey A.C. 2000 Studies on the removal of abnormal prion protein by processes used in the manufacture of human plasma products Vox Sanguinis 78 86 95
45. Foster P.R. Griffin B.D. Bienek C. McIntosh R.V. Mac Gregor I.R. Somerville R.A. Steele P.J. Reichl H.E. 2004 Distribution of a bovine spongiform encephalopathy-derived agent over ion exchange chromatography used in the preparation of concentrates of fibrinogen and factor VIII Vox Sanguinis 86 92 99
46. Fraser H. Brown K.L. Stewart K. McConnell I. McBride P. Williams A. 1996 Replication of scrapie in spleens of SCID mice follows reconstitution with wild type mouse bone marrow Journal of General Virology 77 1935 1940
47. Gajdusek D.C. Zigas V. 1957 Degenerative disease of the central nervous system in New Guinea: epidemic of 'Kuru' in the native population New England Journal of Medicine 257 974 978
48. Ghani A.C. Ferguson N.M. Donnelly C.A. Hagenaars T.J. Anderson R.M. 1998a Estimation of the number of people incubating variant CJD Lancet 352 1353 1354
49. Ghani A.C. Ferguson N.M. Donnelly C.A. Hagenaars T.J. Anderson R.M. 1998b Epidemiological determinants of the pattern and magnitude of the vCJD epidemic in Great Britain Proceedings of the Royal Society 265 2443 2452
50. Glatzel M. Abela E. Maissen M. Aguzzi A. 2003 Extraneural pathologic prion protein in sporadic Creutzfeldt-Jakob disease New England Journal of Medicine 349 1812 1820
51. Glatzel M. Giger O. Seeger H. Aguzzi A. 2004 Variant Creutzfeldt Jakob disease: between lymphoid organs and brain Trends in Microbiology 12 51 53
52. Gregori L. McCombie N. Palmer D. Birch P. Sowemimo-Coker S.O. Giulivi A. Rohwer R.G. 2004 Effectiveness of leucoreduction for removal of infectivity of transmissible spongiform encephalopathies from blood Lancet 364 527 531
53. Harries-Jones R. Knight R. Will R.G. Cousens S. Smith P.G. Mathews W.B. 1988 Creutzfeldt-Jakob disease in England & Wales, 1980-1984: a case control study of potential risk factors Journal of Neurology, Neurosurgery and Psychiatry 51 1113 1119
54. Hart J. Leier B. Nahirniak S. 2004 Informed consent for blood transfusion: should the possibility of prion risk be included Transfusion Medicine Reviews 18 177 183
55. Herzog C. Sales N. Etchegaray N. Charbonnier A. Freire S. Dormont D. Deslys J.P. Lasmezas C.I. 2004 Tissue distribution of bovine spongiform encephalopathy agent in primates after intravenous or oral infection Lancet 363 422 428
56. Hewitt P.E. 2004 Implications of notifying donors and recipients Vox Sanguinis 87 Suppl. 2 1 2
57. Heye N. Hensen S. Muller N. 1994 Creutzfeldt-Jakob disease and blood transfusion Lancet 343 298 299
58. Hill A.F. Desbruslais M. Joiner S. Sidle K.C.L. Gowland I. Collinge J. Doey L.J. Lantos P. 1997a The same prion strain causes vCJD and BSE Nature 389 448 450
59. Hill A.F. Zeidler M. Ironside J. Collinge J. 1997b Diagnosis of new variant Creutzfeldt-Jakob disease by tonsil biopsy Lancet 349 99 100
60. Hill A.F. Butterworth R.J. Joiner S. Jackson G. Rossor M.N. Thomas D.J. Frosh A. Tolley N. Bell J.E. Spencer M. King A. Al-Sarraj S. Ironside J.W. Lantos P.L. Collinge J. 1999 Investigations of variant Creutzfeldt-Jakob disease and other human prion diseases with tonsil biopsy samples Lancet 353 183 189
61. Hilton D.A. Fathers E. Edwards P. Ironside J.W. Zajicek J. 1998 Prion immunoreactivity in appendix before clinical onset of variant Creutzfeldt disease Lancet 352 703 704
62. Hilton D.A. Ghani A.C. Conyers L. Edwards P. McCardle L. Ritchie D. Penney M. Hegazy D. Ironside J.W. 2004 Prevalence of lymphoreticular prion protein accumulation in UK tissue samples Journal of Pathology 203 733 739
63. Hunter N. Foster J. Chong A. McCutcheon S. Parnham D. Eaton S. MacKenzie C. Houston F. 2002 Transmission of prion diseases by blood transfusion Journal of General Virology 83 267 271
64. Ironside J.W. Head M.W. 2003 Variant Creutzfeldt-Jakob disease and its transmission by blood Journal of Thrombosis and Haemostasis 1 1479 1486
65. Kawashima T. Furukawa H. Doh-ura K. Iwaki T. 1997 Diagnosis of new variant Creutzfeldt-Jakob disease by tonsil biopsy Lancet 349 69 79
66. Kirkwood J.K. Cunningham A. 1994 Epidemiological observations on spongiform encephalopathies in captive wild animals in the British Isles Veterinary Record 135 296 303
67. Klein R. Dumble I.L.J. 1993 Transmission of Creutzfeldt-Jakob disease by blood transfusion Lancet 341 768
68. Klein M.A. Frigg R. Flechsig E. Reaber A.J. Kalinke U. Bluethmann H. Bootz F. Suter M. Zinkernagel R.M. Aguzzi A. 1997 A crucial role for B cells in neuroinvasive scraple Nature 390 687 690
69. Klein M.A. Frigg R. Raeber A.J. Flechsig E. Heggi I. Zinkernagel R.M. Wissman C. Aguzzi A. 1998 PrP expression in B lymphocytes is not required for prion neuroinvasion Nature Medicine 4 1429 1433
70. Kondo K. Kuroiwa Y. 1982 A case control study of Creutzfeldt-Jakob disease: association with physical injuries Annals of Neurology 11 377 381
71. Lee C.A. Ironside J.W. Bell J.E. Giangrande P. Ludlam C. Esiri M.M. McLaughlin J.E. 1998 Retrospective neuropathological review of prion disease in UK haemophilia patients Thrombosis and Haemostasis 80 909 911
72. Llewelyn C.A. Hewitt P.E. Knight R.S. Amar K. Cousens S. MacKenzie J. Will R.G. 2004 Possible transmission of variant Creutzfeldt-Jakob disease by blood transfusion Lancet 363 417 421
73. Ludlam C.A. 1997 New-variant Creutzfeldt-Jakob disease and treatment of haemophilia. Executive Committee of the UKHCDO. United Kingdom Haemophilia Centre Directors' Organisation Lancet 350 1704
74. Mabbott N. Turner M.L. 2005 Prions and the blood and immune systems Haematologica 90 540 547
75. Mabbott N.A. Farquhar C.F. Brown K.L. Bruce M.E. 1998 Involvement of the immune system in TSE pathogenesis Immunology Today 19 201 203
76. Manuelidis E.A. Kim J.H. Mericangas J.R. Manuelidis L. 1985 Transmission to animals of Creutzfeldt-Jakob disease from human blood Lancet 2 896 897
77. Martins V.R. Graner E. Garcia-Abreu J. de Souza S.J. Mercadante A.F. Veiga S.S. Zanta S.M. Neto V.M. Brentani R.R. 1997 Complementary hydropathy identifies a cellular prion protein receptor Nature Medicine 3 1376 1381
78. McBride P.A. Eiklenboom P. Kraal G. Fraser H. Bruce M.E. 1992 PrP protein is associated with follicular dendritic cells of spleens and lymph nodes in uninfected and scrapie-infected mice Journal of Pathology 164 413 418
79. McClelland B. Contreras M. 2005 Appropriateness and safety of blood transfusion British Medical Journal 330 104 105
80. McCullough J. Anderson D. Brookie D. Bouchard J.P. Fergusson D. Joly J. Kenny N. Lee D. Megann H. Page D. Reinharz D. Williams J.R. Wilson K. 2004 Consensus conference on vCJD screening of blood donors: report of the panel Transfusion 44 675 683
81. Miele G. Manson J. Clinton M. 2001 A novel erythroid-specific marker of transmissible spongiform encephalopathies Nature Medicine 7 361 364
82. Minor P.D. 2004 Technical aspects of the development and validation of tests for variant Creutzfeldt-Jakob disease in blood transfusion Vox Sanguinis 86 164 170
83. Operskalski E.A. Mosley J.W. 1995 Pooled plasma derivatives and Creutzfeldt-Jakob disease Lancet 346 1224
84. Otto M. Wiltfang J. Schultz E. Zerr I. Otto A. Pfahlberg A. Gefeller O. Unr M. Giese A. Weber T. Kretzschmar H.A. Poser S. 1998 Diagnosis of Creutzfeldt Jakob disease by measurement of S100 protein in serum: prospective case control study British Medical Journal 316 577 582
85. Patry D. Curry B. Easton D. Mastrianni J.A. Hogan D.B. 1998 Creutzfeldt-Jakob disease (CJD) after blood product transfusion from a donor with CJD Neurology 50 1872 1873
86. Peden A.H. Ironside J.W. 2004 Review: pathology of variant Creutzfeldt-Jakob disease Folia Neuropathologica 42 Suppl. A 85 91
87. Peden A.H. Head M.W. Ritchie D.L. Bell J.E. Ironside J.W. 2004 Pre-clinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient Lancet 364 527 529
88. Prowse C.V. Bailey A. 2000 Validation of prion removal by leucocyte-depleting filters: a cautionary tale Vox Sanguinis 79 248
89. Prusiner S.B. 1998 Prions Proceedings of the National Academy of Sciences of the United States of America 95 13363 13383
90. Reichl H.E. Foster P.R. Welch A.G. Li Q. MacGregor I.R. Somerville R.A. Fernie K. Steele P.J. Taylor D.M. 2002 Studies on the removal of a bovine spongiform encephalopathy-derived agent by processes used in the manufacture of human immunoglobulin Vox Sanguinis 83 137 145
91. Ricketts M.N. 1997 Is Creutzfeldt-Jakob disease transmitted in blood? Is the absence of evidence of risk evidence of the absence of risk Canadian Medical Association Journal 157 1367 1370
92. Ricketts M.N. Cashman N.R. Stratton E.E. El Saadany S. 1997 Is Creutzfeldt-Jakob disease transmitted in blood Emerging Infectious Diseases 3 155 163
93. Rieger R. Edenhofer H. Lasmezas C.I. Weiss S. 1997 The human 37 kDa laminin receptor precursor interacts with the prion protein in eukaryotic cells Nature 382 1383 1388
94. Roddie P.H. Turner M.L. Williamson L.M. 2000 Leucocyte depletion of blood components Blood Reviews 14 145 156
95. Shyng S.L. Huber M.T. Harris D.A. 1993 A prion protein cycles between the cell surface and an endocytic compartment in cultured neuroblastoma cells Journal of Biological Chemistry 268 15922 15928
96. de Silva R. 1996a Human spongiform encephalopathy In: Prion Diseases ed. by Baker H.F. Ridley R.M. pp 15 33 Humana Press Totowa, NJ
97. de Silva R. 1996b Pooled plasma derivatives and Creutzfeldt-Jakob disease Lancet 347 967
98. de Silva R. Mathews W.B. 1993 Creutzfeldt-Jakob disease and blood transfusion Lancet 341 205 207
99. Sirchia G. Giovanetti A.M. McClelland D.B.L. Fracchia G.N. (eds) 1994 Safe and good use of blood in surgery (SANGUIS): use of blood products and artificial colloids in 43 European hospitals European Commission EUR15398EN Brussels
100. Smith P.G. Cousens S.N. Hullard Aignaux J.N. Ward H.J. Will R.G. 2004 The epidemiology of variant Creutzfeldt-Jakob disease Current Topics in Microbiology and Immunology 284 161 191
101. Sneath P.H. 2004 Estimation of the size of the vCJD epidemic Antonie Van Leeuwenhoek 86 93 103
102. St Romaine C. Hazlehurst G. Jewell A.P. 2004 Leucodepletion for transmissible spongiform encephalopathies British Journal of Biomedical Science 61 48 54
103. Stenland C.J. Lee D.C. Brown P. Petteway S.R. Rubenstein R. 2002 Partitioning of human and sheep forms of the pathogenic prion protein during the purifications of therapeutic proteins from human plasma Transfusion 42 1497 1500
104. Tahiri-Alaoui A. Gill A.C. Disterer P. James W. 2004 Methionine 129 variant of human prion protein oligomerizes more rapidly than the valine 129 variant: implications for disease susceptibility to Creutzfeldt-Jakob disease Journal of Biological Chemistry 279 31390 31397
105. Tateishi J. 1985 Transmission of Creutzfeldt-Jakob disease from human blood and urine into mice Lancet ii 896 897
106. Tateishi J. Kitamoto T. Mohri S. Satoh S. Sato T. Shepherd A. Macnaughton M.R. 2001 Scrapie removal using Planova® virus removal filters Biologicals 29 17 25
107. Tobler I. Gaus S.E. Deboer T. Anchermann P. Fisher M. Rulicke T. Moser M. Oesch B. McBride P.A. Manson J.C. 1996 Altered circadian activity rhythms and sleep in mice devoid of prion protein Nature 380 639 642
108. Van Duijin C.M. Delasnerie-Laupretre N. Masullo C. Zerr I. de Silva R. Wientjens D.P. Brandel J.P. Weber T. Bonavita V. Zeidler M. Alperovitch A. Poser S. Granieri E. Hofman A. Will R.G. 1998 Case-control study of risk factors of Creutzfeldt-Jakob disease in Europe during 1993-1995 Lancet 351 1081 1085
109. Vey M. Baron H. Weimer T. Groner A. 2002 Purity of spiking agent affects partitioning of prions in plasma protein purification Biologicals 30 187 196
110. Wadsworth J.D. Asante E.A. Desbruslais M. Linehan J.M. Joiner S. Gowland I. Welch J. Stone L. Lloyd S.E. Hill A.F. Brandner S. Collinge J. 2004 Human prion protein with valine 129 prevents expression of variant CJD phenotype Science 306 1793 1796
111. Wells G.A.H. Scott A.C. Johnson C.T. Gunning R.E. Hancock R.D. Jeffrey M. Dawson M. Bradley R. 1987 A novel progressive spongiform encephalopathy in cattle Veterinary Record 121 419 420
112. Wientjens D.P.W.M. Davlnipour Z. Hofman A. Kondo K. Mathews W.B. Will R.G. van Duijin C.M. 1996 Risk factors for Creutfeldt-Jakob disease: a reanalysis of case-control studies Neurology 46 1287 1291
113. Wilesmith J.W. Wells G.A.H. Cranwell M.P. Ryan J.B.M. 1988 Bovine spongiform encephlopathy: epidemiological studies Veterinary Record 123 638 644
114. Will R.G. 1991 Epidemiological surveillance of Creutzfeldt-Jakob disease in the United Kingdom European Journal of Epidemiolology 7 460 465
115. Will R.G. 2003 The biology and epidemiology of variant Creutzfeldt-Jakob disease Bulletin et Memoires de l'Academie Royale de Medecine de Belgique 158 250 256
116. Will R. 2004 Variant Creutzfeldt-Jakob disease Folia Neuropathologica 42 Suppl. A 77 83
117. Will R.G. Kimberlin R.H. 1998 Creutzfeldt-Jakob disease and the risk from blood or blood products Vox Sanguinis 75 178 180
118. Will R.G. Ward H.J. 2004 Clinical features of variant Creutzfeldt-Jakob disease Current Topics in Microbiology and Immunology 284 121 132
119. Will R.G. Ironside J.W. Zeldler M. Cousens S.N. Estibeiro K. Alperovitch A. Poser S. Pocchiari M. Hofman A. Smith P.G. 1996 A new variant of Creutzfeldt-Jakob disease in the UK Lancet 347 921 925
120. Will R.G. Alperovitch A. Poser S. Pocchiari M. Hofman A. Mitrova E. de Silva R. Alessandro M. Delasnerie-Laupretre N. Zerr I. van Duijin C. 1998 Descriptive epidemiology of Creutzfeldt-Jakob disease in six European countries 1993-1995 Annals of Neurology 43 763 767
121. Wilson K. Ricketts M.N. 2004a q14 The success of precaution? Managing the risk of transfusion transmission of variant Creutzfeldt-Jakob disease Transfusion 44 1475 1478
122. Wilson K. Ricketts M.N. 2004b q15 Transfusion transmission of vCJD: a crisis avoided Lancet 364 477 479
123. Wyatt J.M. Pearson G.R. Smerdon T.N. Gruffydd-Jones T.J. Wells G.A.H. Wilesmith J.W. 1991 Naturally occurring scrapie-like spongiform encephalopathy in five domestic cats Veterinary Record 129 233 236
124. Zerr I. Bodemer M. Gefeller O. Otto M. Poser S. Wiltfaring J. Windl O. Kretzschamar H.A. Weber T. 1998 Detection of the 14-3-3 protein in the cerebrospinal fluid supports the diagnosis of Creutzfeldt-Jakob disease Annals of Neurology 43 32 40