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

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

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

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

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

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

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

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

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

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

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

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

16 human prion protein for propagating BSE and vCJD prions.

17 might mediate its susceptibility to BSE and vCJD prions.

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

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

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

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

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

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

24 ount for the distinct phenotypes of sCJD and vCJD.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

46 ds to posttest probabilities and can confirm vCJD infection.

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

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

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

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

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

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

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

54 sion from a donor who subsequently developed vCJD.

55 usion from donors who subsequently developed vCJD.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

106 e have previously reported a blood assay for vCJD.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

123 80% of the assessments in vCJD cases were graded as moderate or substantial.

124 .5 x 10(-17); best haplotypic association in vCJD p=1 x 10(-24)).

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

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

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

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

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

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

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

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

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

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

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

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

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

138 12 years at onset--the youngest ever case of vCJD.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

153 o represent the geographical distribution of vCJD.

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

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

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

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

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

159 Cumulative incidences of vCJD by standard region were calculated.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

174 other nearby SNP conferred increased risk of vCJD.

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

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

177 years before the donor developed symptoms of vCJD.

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

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

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

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

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

183 otential risk for iatrogenic transmission of vCJD.

184 and died after a clinical course typical of vCJD.

185 either susceptibility to, or expression of, vCJD.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

205 9 of PRNP, suggesting that susceptibility to vCJD infection is not confined to the methionine homozyg

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

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

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

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

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

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

212 were analysed for regional correlations with vCJD incidence.

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

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

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

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

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

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

219 90 whole blood samples from 21 patients with vCJD, 27 with sporadic CJD, 42 with other neurological d

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

221 ly detection and management of patients with vCJD.

222 logical reports prepared on 24 patients with vCJD.

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

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

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

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