ライフサイエンスコーパス: vCJD

1 vCJD appendix and blood (Buffy coat fraction) were negat

2 vCJD incidence was higher in the north of Great Britain

3 vCJD patients were correctly identified based on bilater

4 vCJD prion strain identity was retained after passage in

5 etect PrP(Sc) in the equivalent of 50 nL 10% vCJD brain homogenate, with a maximum limit of detection

6 hniques identified muscle PrP(Sc) in 8 of 17 vCJD, 7 of 26 sCJD, and 2 of 5 iCJD patients.

7 Eighty-eight of 106 (83%) vCJD cases were retrospectively classified as probable i

8 transgenic mice, in addition to producing a vCJD-like phenotype, can also result in a distinct molec

9 isease from mice infected with mouse-adapted vCJD and confirm the presence of the exosomal marker Hsp

10 isolates of human TSE agents, mouse-adapted vCJD, and Fukuoka 1.

11 se (vCJD) in the United Kingdom, because all vCJD patients tested thus far have been M/M carriers.

12 Although vCJD cases are now rare, evidence from appendix surveys

13 imary and secondary transmissions of BSE and vCJD in guinea pigs result in long incubation periods of

14 ce supports the rapid propagation of BSE and vCJD prions and suggest that Tg(GPPrP) mice may serve as

15 termine if the incubation periods of BSE and vCJD prions could be shortened, we generated transgenic

16 Inoculation of Tg(GPPrP) mice with BSE and vCJD prions resulted in mean incubation periods of 210 a

17 and neuropathological properties of BSE and vCJD prions, including the presence of type 2 protease-r

18 might mediate its susceptibility to BSE and vCJD prions.

19 human prion protein for propagating BSE and vCJD prions.

20 ame agent strain is involved in both BSE and vCJD.

21 hat the causative agent of BSE in cattle and vCJD in humans share a common origin.

22 results of transmissions of sporadic CJD and vCJD to mice.

23 Therefore, PrP species in sCJD and vCJD have dissimilar lectin immunoreactivity, which refl

24 ermore, the RCA binding activity in sCJD and vCJD samples is mostly associated with proteinase K-resi

25 inus communis agglutinin I (RCA) to sCJD and vCJD samples was significantly increased.

26 ount for the distinct phenotypes of sCJD and vCJD.

27 of PrP(C) with two human agents (sCJDMM2 and vCJD) and one hamster strain (263K).

28 This case of transfusion-associated vCJD infection, identified ante-mortem, is the third ins

29 ate whether there were transplant-associated vCJD cases in the United Kingdom (UK).

30 rovides no evidence of transplant-associated vCJD in the UK.

31 large-scale screening tests for asymptomatic vCJD prion infection.

32 y are suitable for detection of asymptomatic vCJD infection in the human population.

33 t from a total of 15 donors who later became vCJD cases and appeared on the surveillance unit's regis

34 undergone an organ (liver) transplant before vCJD onset, from a donor who had died of causes unrelate

35 here were no significant differences between vCJD and control populations in frequencies of any MHC t

36 unced its first case of probable blood-borne vCJD transmission in December, 2003, and first detected

37 relevant model for assessing the blood-borne vCJD transmission risk.

38 zfeldt-Jakob disease (sCJD), or variant CJD (vCJD) brains.

39 In 2002, 17 people died from variant CJD (vCJD) in the UK, compared with 20 in 2001 and 28 in 2000

40 ntrols, sporadic CJD (sCJD) and variant CJD (vCJD) patients has identified three polymorphisms, all o

41 n in the urine of patients with variant CJD (vCJD) using protein misfolding by cyclic amplification,

42 emergence, in the mid-1990s, of variant CJD (vCJD), a disease linked to bovine spongiform encephalopa

43 ed GWAS of sporadic CJD (sCJD), variant CJD (vCJD), iatrogenic CJD, inherited prion disease, kuru and

44 ental exposure of astrocytes to variant CJD (vCJD), the kinetics of prion replication occur in a prio

45 ase (CJD), referred to as 'new variant' CJD (vCJD), have been recognized in unusually young people in

46 just over 100 people have developed clinical vCJD, millions have probably been exposed to the infecti

47 Although cases of clinical vCJD are rare, there is evidence there may be tens of th

48 enotype-dependent susceptibility to clinical vCJD found in patients.

49 ecificity to justify a large study comparing vCJD prevalence in the United Kingdom with a bovine spon

50 ds to posttest probabilities and can confirm vCJD infection.

51 patients with neuropathologically confirmed vCJD and from individuals without neurological disease.

52 ikely (n = 105), and patients with confirmed vCJD (n = 10).

53 By contrast, vCJD was transmitted to all three human lines with diffe

54 Among them were two fatal cases of definite vCJD and one case of probable vCJD; all were reported in

55 t reported all cases of probable or definite vCJD to the UK blood services, which searched for donati

56 ad occurred in people who went on to develop vCJD.

57 usion from donors who subsequently developed vCJD were identified post-mortem and reported in 2004.

58 usion from donors who subsequently developed vCJD.

59 sion from a donor who subsequently developed vCJD.

60 Variant Creutzfeldt-Jakob disease (vCJD) and bovine spongiform encephalopathy (BSE) prions

61 s, namely varient Creutzfeldt-Jakob disease (vCJD) and classical bovine spongiform encephalopathy (BS

62 condition variant Creutzfeldt-Jakob disease (vCJD) and, based on recent human prevalence studies, sig

63 ood-based variant Creutzfeldt-Jakob disease (vCJD) assay has sufficient sensitivity and specificity t

64 arrier of variant Creutzfeldt-Jakob disease (vCJD) can be followed up to quantify transmission risks.

65 number of variant Creutzfeldt-Jakob disease (vCJD) cases.

66 Variant Creutzfeldt-Jakob disease (vCJD) differs from other human prion diseases in that th

67 Variant Creutzfeldt-Jakob disease (vCJD) has a pathogenesis distinct from other forms of hu

68 Variant Creutzfeldt-Jakob disease (vCJD) has been recognized to date only in individuals ho

69 ission of variant Creutzfeldt-Jakob disease (vCJD) has occurred through blood transfusion and could a

70 ions with variant Creutzfeldt-Jakob disease (vCJD) have almost exclusively occurred in young patients

71 ents with variant Creutzfeldt-Jakob disease (vCJD) have been treated with intraventicular pentosan po

72 eclinical variant Creutzfeldt-Jakob disease (vCJD) in a patient who died from a non-neurological diso

73 , causing variant Creutzfeldt-Jakob disease (vCJD) in humans and having guided protective measures fo

74 tbreak of variant Creutzfeldt-Jakob disease (vCJD) in the United Kingdom, because all vCJD patients t

75 opment of variant Creutzfeldt-Jakob disease (vCJD) in three recipients of non-leukoreduced red blood

76 evels and variant Creutzfeldt-Jakob disease (vCJD) incidence.

77 Variant Creutzfeldt-Jakob disease (vCJD) is a devastating disease caused by transmission of

78 Variant Creutzfeldt-Jakob disease (vCJD) is a fatal neurodegenerative disorder characterise

79 Variant Creutzfeldt-Jakob disease (vCJD) is a fatal neurodegenerative disorder originating

80 Variant Creutzfeldt-Jakob disease (vCJD) is a novel human prion disease caused by infection

81 Variant Creutzfeldt-Jakob disease (vCJD) is a unique and highly distinctive clinicopatholog

82 ssion of, variant Creutzfeldt-Jakob disease (vCJD) is essential for future management of the disease.

83 ised that variant Creutzfeldt-Jakob disease (vCJD) might be transmissible by blood transfusion.

84 bution of variant Creutzfeldt-Jakob disease (vCJD) might indicate the transmission route of the infec

85 patients (variant Creutzfeldt-Jakob disease (vCJD) n = 20, iatrogenic CJD (iCJD) n = 11, sporadic CJD

86 (BSE) and variant Creutzfeldt-Jakob disease (vCJD) prions are faithfully maintained upon transmission

87 ission of variant Creutzfeldt-Jakob disease (vCJD) through blood transfusions has created new concern

88 ission of variant Creutzfeldt-Jakob disease (vCJD) to humans.

89 ndividual variant Creutzfeldt-Jakob disease (vCJD) transmission through blood and blood-derived produ

90 eports of variant Creutzfeldt-Jakob disease (vCJD) transmission through blood transfusion from humans

91 ission of variant Creutzfeldt-Jakob disease (vCJD) via blood transfusion has caused concern over spre

92 nsmitting variant Creutzfeldt-Jakob disease (vCJD) via blood transfusion have relied largely on data

93 Variant Creutzfeldt-Jakob disease (vCJD) was first described in the United Kingdom in 1996

94 Variant Creutzfeldt-Jakob Disease (vCJD) was first reported in 1996; the youngest patient d

95 Variant Creutzfeldt-Jakob disease (vCJD), a novel form of human prion disease, was recogniz

96 cted with variant Creutzfeldt-Jakob disease (vCJD), a prion disease typically acquired from consumpti

97 a variant form of Creutzfeldt-Jakob disease (vCJD), also mostly in the United Kingdom, that occurs in

98 onset of variant Creutzfeldt-Jakob disease (vCJD), and the unknown prevalence of infection after the

99 uivalent, variant Creutzfeldt-Jakob disease (vCJD), are caused by the same strain of infectious agent

100 of human variant Creutzfeldt-Jakob disease (vCJD), experimental ovine bovine spongiform encephalopat

101 thy (BSE)/variant Creutzfeldt-Jakob disease (vCJD), Nipah virus, several viral hemorrhagic fevers and

102 ents with variant Creutzfeldt-Jakob disease (vCJD), sheep with natural scrapie or rodents following e

103 ated with variant Creutzfeldt-Jakob disease (vCJD).

104 test for variant Creutzfeldt-Jakob disease (vCJD).

105 rriers of variant Creutzfeldt-Jakob disease (vCJD).

106 agent of variant Creutzfeldt-Jakob disease (vCJD).

107 a case of variant Creutzfeldt-Jakob disease(vCJD) in a 74-year old man in whom diagnosis was made at

108 ity of the assay for detection of endogenous vCJD, we analysed a masked panel of 190 whole blood samp

109 JD diagnosis by adaptation of an established vCJD diagnostic blood test to urine.

110 tinguish a 10(-)(1)(0) dilution of exogenous vCJD prion-infected brain from a 10(-)(6) dilution of no

111 nescent signal, 1.3x10(5) [SD 1.1x10(4)] for vCJD vs 9.9x10(4) [4.5x10(3)] for normal brain; p<0.0001

112 est for vCJD, the Direct Detection Assay for vCJD, was negative.

113 e have previously reported a blood assay for vCJD.

114 dy confirms that the diagnostic criteria for vCJD are sensitive and specific and provide a useful sta

115 of PRNP was the main genetic risk factor for vCJD; however, additional candidate loci have been ident

116 tic use and found it to be PMCA-negative for vCJD and BSE prions.

117 ice with intermediate incubation periods for vCJD prions had a mixture of the 2 strains.

118 pies, was associated with increased risk for vCJD, as was reported frequent chicken consumption.

119 icity supports using the assay to screen for vCJD infection in prion-exposed populations.

120 (PIND) in UK children, and have searched for vCJD among the children who were reported.

121 with vCJD, showing an assay sensitivity for vCJD of 71.4% (95% CI 47.8-88.7) and a specificity of 10

122 ay, developed originally as a blood test for vCJD, for the detection of disease-associated prion prot

123 The result of a blood test for vCJD, the Direct Detection Assay for vCJD, was negative.

124 Four vCJD cases linked to transfusion of vCJD-contaminated bl

125 We analysed data for deaths from vCJD since 1995 and estimated the underlying trend in mo

126 Scans from vCJD cases were reassessed to reach a consensus on all a

127 imate the 95% confidence interval for future vCJD mortality to be 50 to 50,000 human deaths consideri

128 ry of a patient recently diagnosed as having vCJD in the United Kingdom.

129 lly attributable to interrupt human-to-human vCJD transmission or treat it.

130 a screening tool for the presence of human (vCJD) and bovine (BSE) prions in a human cell therapy pr

131 carriers could present a risk of iatrogenic vCJD transmission through medical procedures or blood/or

132 gth PrP species, the RCA binding activity in vCJD is associated with truncated and full-length PrP sp

133 80% of the assessments in vCJD cases were graded as moderate or substantial.

134 .5 x 10(-17); best haplotypic association in vCJD p=1 x 10(-24)).

135 ngle nucleotide polymorphism] association in vCJD p=2.5 x 10(-17); best haplotypic association in vCJ

136 the extremely low concentration of PrP(D) in vCJD urine.

137 PrP(Sc) in muscle could also be detected in vCJD, sCJD, and iatrogenic (iCJD) patients from other po

138 Regional differences in vCJD incidence are unlikely to be due to ascertainment b

139 ain characteristics of the PrP(D) present in vCJD brains, such as infectivity and phenotype determina

140 mmunoblot method for detection of PrP(Sc) in vCJD tissues that can be used to provide an upper limit

141 The mean survival in vCJD is 17 months, with 40 months the maximum survival i

142 Data on survival in vCJD is available from information held at the National

143 ation with acquired prion disease, including vCJD (p=5.6 x 10(-5)), kuru incubation time (p=0.017), a

144 g longer incubation times, of the inoculated vCJD strain; the second strain produced a phenotype rese

145 of the patient was well beyond that of most vCJD cases, and the chance of observing a case of vCJD i

146 ilution series of 10(-)(7) to 10(-)(1)(0) of vCJD prion-infected brain homogenate into whole human bl

147 ivity, has been demonstrated in the blood of vCJD patients.

148 cases, and the chance of observing a case of vCJD in a recipient in the absence of transfusion transm

149 the neuropathological findings in a case of vCJD treated with PPS.

150 12 years at onset--the youngest ever case of vCJD.

151 er the 10-year period, 106 definite cases of vCJD and 45 pathologically confirmed "noncases" were ide

152 e have raised concerns that further cases of vCJD could emerge as a result of prolonged incubation an

153 are autopsy/cerebral biopsy-proven cases of vCJD referred to the National CJD Surveillance Unit (NCJ

154 Since one probable and two definite cases of vCJD were reported to the study in 1999, there is concer

155 udes the duration of illness in 176 cases of vCJD, five of which were treated with iPPS.

156 ients or donors and the database of cases of vCJD.

157 findings show that strain characteristics of vCJD brain PrP(D), including infectivity, are preserved

158 Conversion of vCJD-like to sCJD-like strains was favored in Tg1014 mic

159 city of a blood-based assay for detection of vCJD prion infection.

160 examination which confirmed the diagnosis of vCJD and showed severe, but typical, changes, including

161 ifficulties in the differential diagnosis of vCJD and the more rare sCJD subtypes based on molecular

162 vide a prototype blood test for diagnosis of vCJD in symptomatic individuals, which could allow devel

163 Health Protection Agency; after diagnosis of vCJD, the patient was enrolled into the MRC PRION-1 tria

164 resonance imaging (MRI) for the diagnosis of vCJD.

165 seful non-invasive test for the diagnosis of vCJD.

166 y in the UK), altered tissue distribution of vCJD, and the failure of decontamination processes to ad

167 o represent the geographical distribution of vCJD.

168 een MHC type and age of onset or duration of vCJD disease.

169 a comparison of transmission efficiencies of vCJD and bovine spongiform encephalopathy (BSE) and an a

170 ay represent one of the earliest features of vCJD and it is possible that, at least in some cases, ne

171 other children with the clinical features of vCJD were identified.

172 Other common MRI features of vCJD were medial thalamic and periaqueductal grey matter

173 ongiform encephalopathy and the incidence of vCJD has declined, leading to questions about the requir

174 Cumulative incidences of vCJD by standard region were calculated.

175 investigated whether regional incidences of vCJD were correlated with regional dietary data.

176 roducts being the main route of infection of vCJD, but recall bias cannot be excluded.

177 s study, we used a well-established model of vCJD (sheep experimentally infected with bovine spongifo

178 rom a well-established large animal model of vCJD.

179 ed in the retina and proximal optic nerve of vCJD eye at levels of 2.5 and 25%, respectively of those

180 The occurrence of vCJD in an individual in this age group is unlikely to b

181 HC phenotypes in relation to age of onset of vCJD and its duration from presentation to death.

182 formation regarding the incubation period of vCJD and the number of people who may have been exposed,

183 that a change in the pathologic phenotype of vCJD would not be expected as a result of exposure to in

184 neuropathological and molecular phenotype of vCJD, consistent with these diseases being caused by the

185 Presence of vCJD infection determined by a prototype test (now in cl

186 grammes that target the younger age range of vCJD cases.

187 te the distribution of PrP(Sc) in a range of vCJD tissues.

188 Regional rates of vCJD were correlated with consumption of other meat or m

189 genome-wide association study of the risk of vCJD and tested for replication of our findings in sampl

190 identify individuals who might be at risk of vCJD by this route so that appropriate public health mea

191 other nearby SNP conferred increased risk of vCJD.

192 ons for future estimates and surveillance of vCJD in the UK.

193 nts was identified as developing symptoms of vCJD 6.5 years after receiving a transfusion of red cell

194 years before the donor developed symptoms of vCJD.

195 Four vCJD cases linked to transfusion of vCJD-contaminated blood or blood products have been desc

196 inty surrounding the risk of transmission of vCJD by blood products, blood transfusion services in a

197 Transmission of vCJD prions to Tg1014 mice resulted in 2 different strai

198 be susceptible to secondary transmission of vCJD through routes such as blood transfusion.

199 d barrier for human-to-human transmission of vCJD.

200 otential risk for iatrogenic transmission of vCJD.

201 and died after a clinical course typical of vCJD.

202 either susceptibility to, or expression of, vCJD.

203 ly truncated PrP species were compared, only vCJD samples had more RCA binding activity.

204 Mice were inoculated with BSE or vCJD and assessed for clinical and pathological signs of

205 se the danger of additional spread of BSE or vCJD infection by contaminated blood, surgical instrumen

206 tissues to assess prevalence of preclinical vCJD infection within the UK and other populations.

207 ssues, the unknown prevalence of preclinical vCJD, and a transmission barrier which limits the sensit

208 Definite and probable vCJD cases (n = 136) were residing in Great Britain at d

209 A clinical diagnosis of probable vCJD was made; tonsil biopsy was not done.

210 es of definite vCJD and one case of probable vCJD; all were reported in 1999.

211 Inoculation of raw vCJD urine caused no disease, confirming the extremely l

212 o other people, UK patients who had received vCJD-implicated plasma products are being contacted.

213 ose (amount of infected blood) and response (vCJD infection) has never been well quantified.

214 rP(res) accumulation in the body of a simian vCJD model.

215 d a case of probable blood-borne subclinical vCJD in July, 2004.

216 In Switzerland, surveillance for subclinical vCJD includes unconsented testing in autopsies: consente

217 the UK has found few children with suspected vCJD is relatively reassuring.

218 isolated event.Doctors need to be aware that vCJD can arise in elderly patients so that appropriate i

219 lationship for a wide range of doses for the vCJD agent in transfused blood and plasma derivatives.

220 CJD-like or novel phenotypes in addition to vCJD, depending on the genotype of the prion source and

221 nsfusion, but this evidence may not apply to vCJD.

222 celerated loss of survival after exposure to vCJD, classical BSE, or CWD prions at the larval stage.

223 hich used a different method, sensitivity to vCJD infection was low (7.7%; 95% CI, 0.2%-36%), with on

224 9 of PRNP, suggesting that susceptibility to vCJD infection is not confined to the methionine homozyg

225 a pig prion protein are fully susceptible to vCJD and BSE prions but not to sporadic CJD prions.

226 a donor who had died of causes unrelated to vCJD.

227 te the likelihood of transfusion-transmitted vCJD (TTvCJD) in humans.

228 Medical histories were reviewed for 177 UK vCJD cases to identify situations where the transplantat

229 We aimed to identify whether vCJD is transmissible through blood transfusion.

230 A SNP in the CHN2 gene was associated with vCJD [P = 1.5 x 10(-7); odds ratio (OR), 2.36], but not

231 were analysed for regional correlations with vCJD incidence.

232 blished MHC genotypes of 76 individuals with vCJD and 131 controls, and analysed MHC phenotypes in re

233 The number of asymptomatic individuals with vCJD prion infection is unknown, posing risk to others v

234 gland, and thymus from a single patient with vCJD.

235 rom unaffected individuals and patients with vCJD (70% sensitivity; 95% CI, 34.8%-93.3%).

236 MRI from patients with vCJD and controls (patients with suspected CJD) were ana

237 ese findings suggest that some patients with vCJD have very low peripheral prion colonization and the

238 90 whole blood samples from 21 patients with vCJD, 27 with sporadic CJD, 42 with other neurological d

239 All 15 samples were from patients with vCJD, showing an assay sensitivity for vCJD of 71.4% (95

240 ly detection and management of patients with vCJD.

241 logical reports prepared on 24 patients with vCJD.

242 Five people with vCJD in Leicestershire formed a cluster (p=0.004).

243 Unit prospectively identified 84 people with vCJD up to Nov 10, 2000, in Great Britain.

244 score for areas of residence of people with vCJD was -0.09 (-0.73 to 0.55), which is close to the na

245 us of the places of residence of people with vCJD was compared with that of the general population.