VARIANT CJD (vCJD) or nvCJD

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Location: BACLIFF, Texas, United States

My mother was murdered by what I call corporate and political homicide i.e. FOR PROFIT! she died from a rare phenotype of CJD i.e. the Heidenhain Variant of Creutzfeldt Jakob Disease i.e. sporadic, simply meaning from unknown route and source. I have simply been trying to validate her death DOD 12/14/97 with the truth. There is a route, and there is a source. There are many here in the USA. WE must make CJD and all human TSE, of all age groups 'reportable' Nationally and Internationally, with a written CJD questionnaire asking real questions pertaining to route and source of this agent. Friendly fire has the potential to play a huge role in the continued transmission of this agent via the medical, dental, and surgical arena. We must not flounder any longer. ...TSS

Thursday, March 26, 2015

Variant CJD and blood transfusion: are there additional cases?

Vox Sanguinis (2014) 107, 220–225 ORIGINAL PAPER © 2014 International Society of Blood Transfusion DOI: 10.1111/vox.12161
 
Variant CJD and blood transfusion: are there additional cases?
 
L. R. R. Davidson, 1 C. A. Llewelyn, 2 J. M. Mackenzie, 1 P. E. Hewitt 3 & R. G. Will 1 1 National CJD Research & Surveillance Unit, Western General Hospital, Edinburgh, UK 2 NHS Blood and Transplant, Cambridge Centre, Cambridge, UK 3 NHS Blood and Transplant, Colindale Centre, London, UK Received: 12 February 2014, revised 29 April 2014, accepted 30 April 2014, published online 11 June 2014
 
Background and Objectives In this study, we compare variant Creutzfeldt-Jakob disease (vCJD) cases definitely linked to blood transfusion, those with a history of blood transfusion in which no donor has developed vCJD and primary cases with no history of blood transfusion. The aim is to determine whether there are any differences in the demographics or clinical phenotype in these groups that might suggest additional cases of transfusion transmission of vCJD.
 
Materials and Methods All cases of vCJD who are old enough to donate blood (i.e. >17 years old) are notified to the UKBTS at diagnosis, regardless of whether they are known to have a blood donation history. A search is then made for donor records and, if found, all components produced and issued to hospitals are identified and their fate determined. Recipient details are then checked against the NCJDRSU register to establish whether there is a match between these individuals and patients who have been diagnosed with vCJD. In the reverse study, attempts are made to trace the donors to all cases reported to have received a blood transfusion and donors’ details are checked against the register to determine if any have developed vCJD.
 
Results Of the 177 cases of vCJD diagnosed in the UK as of 1 February 2014, the TMER study identified 15 cases reported to have received a blood transfusion. Transfusion records were unavailable for 4 of these cases, all pre-1980, and in one other case there was no transfusion recorded in the medical notes. Transfu- sion records were found for 10 cases. One case transfused at symptom onset was excluded from this analysis. The mean age at onset of symptoms of the remain- ing nine transfusion recipients (four female and five male) was 42Á9 years; 57Á6 years in the three known transfusion-transmitted cases and 35Á5 years in the six not linked cases. In one of these cases, details of components transfused were unavailable, and the remaining five cases received a total of 116 donor exposures with 112 donors identified, none of whom is known to have developed clinical vCJD. To date, five of the 112 identified donors have died and none was certified as dying of vCJD or any other neurological disorder. Two of the transfu- sion-transmitted cases did not fulfil diagnostic criteria for probable vCJD during life but were confirmed at post-mortem. Both cases were in the older age range (68 and 74 years, respectively), and neither had a positive MRI brain scan. The remaining cases all fulfilled the criteria for the diagnosis of vCJD in life, but two of these had atypical features and were older than the expected age at onset for vCJD.
 
Conclusion In conclusion, it is possible that one or more of the vCJD cases that received a blood transfusion derived from an individual not known to have vCJD were infected by the blood transfusion. However, the evidence for this is weak, and the absence of a past history of transfusion in most cases of vCJD excludes a large number of unrecognised transfusion-transmitted cases.
 
 
 
 
Letter Variant CJD and blood transfusion J. P. Wallis* Article first published online: 6 MAR 2015
 
DOI: 10.1111/vox.12241
 
© 2015 International Society of Blood Transfusion
 
 
 
 
Dear Editor,

The recently published histological study of surgically removed appendix tissue raised the possibility of silent carriers of the disease affecting 1 in 2000 UK donors [1]. Davidson et al. [2] report on the characteristics of patients with clinical vCJD who had previously received blood transfusion but where the donors have not themselves gone on to develop clinical vCJD. On the basis of a higher-than-expected mean age of these cases, they suggest an association between transfusion and vCJD. However, they do not adjust for factors affecting the likelihood of prior transfusion. From our study in 1994 of point incidence of transfusion and subsequent survival, I have estimated the prevalence of prior transfusion by age (Table 1) [3]. To allow for the effect of repeated transfusion and the observed 80% 5-year survival, I have applied a correction factor of 0·5 to the data. My figures are similar to other surveys of prior transfusion prevalence [4]. I have excluded neonatal transfusion as practice markedly changed during the previous 2 decades.

Table 1. Estimates of expected prevalence of prior transfusion in patients with clinical vCJD
Age group
Transfusion prevalence per 100 in 1994
vCJD clinical cases
Estimated cases with prior transfusion
10–14
0·27
0
0
15–19
0·94
25
0·24
20–24
1·79
44
0·79
25–29
3·00
44
1·32
30–34
4·04
32
1·27
35–39
5·18
19
0·98
40–44
6·59
6
0·42
45–49
8·59
2
0·16
50–54
10·88
4
0·48
55–60
14·67
1
0·09
Total
 
177
5·75

The Table 1 shows the estimated prevalence of prior transfusion in quintiles from 10 to 60 years. This increases because the cumulative likelihood of having received transfusion increases with time at risk and because transfusion point incidence rises with age. Using the known age distribution of the 177 vCJD cases, I have estimated that 5·75 cases would be expected to have had prior transfusion. Davidson et al. find 14 cases who had definitely been transfused. Of these, three were from donors who subsequently developed vCJD and are excluded, one was transfused immediately prior to diagnosis, leaving 10 cases. One was transfused as a neonate, a period I have excluded leaving 9. Four were from a period before records of donors were available, and not knowing their age at transfusion, I cannot know whether they should be included in an analysis of transfusion after the age of 10 years. A figure of between 5 and 9 remaining cases having received transfusion in the last 10–20 years prior to diagnosis is close to 5·75 as estimated above. Older patients with clinical vCJD are more likely to have been transfused, and the mean age will be higher than the whole cohort. Based on the age-adjusted transfusion prevalence, the mean age of cases that might have received an unlinked prior transfusion is 33·4 years. This compares with the observed figure given by Davidson et al. of 35·5 years. The prevalence of transfusion in females is almost twice that in males between the ages of 30 and 60. Four of six vCJD patients with prior transfusion were female.

The observations of Davidson et al. given the analysis above would suggest no significant excess of prior transfusion amongst vCJD cases during the recent epidemic, and no unexpected age or sex distribution in vCJD cases with prior transfusion.



References

1.     Top of page

·                     1

Gill ON, Spencer Y, Richard-Loendt A, et al.: Prevalent abnormal prion protein in human appendices after bovine spongiform encephalopathy epizootic: large scale survey. BMJ 2013; 347:f5675

·                     2

Davidson LR, Llewelyn CA, Mackensie JM, et al.: Variant CJD and blood transfusion: are there additional cases? Vox Sang 2014; 107:220–225

·                     3

Wallis JP, Wells AW, Matthews JN, et al.: Long-term survival after blood transfusion: a population based study in the North of England. Transfusion 2004; 44:1025–1032

·                     4

Kamper-Jørgensen M, Edgren G, Rostgaard K, et al.: Blood transfusion exposure in Denmark and Sweden. Transfusion 2009; 49:888–894
 
 
 
 
Friday, February 06, 2015
 
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Blood reference materials from macaques infected with variant Creutzfeldt-Jakob disease agent
 
 
EMBARGOED ADVANCE COPY Not to be published in full, or in part, in any form before 00.01am on Thursday 24 July 2014 HC 327
 
House of Commons Science and Technology Committee
 
After the storm?
 
UK blood safety and the risk of variant Creutzfeldt-Jakob Disease
 
Second Report of Session 2014–15
 
EMBARGOED ADVANCE COPY Not to be published in full, or in part, in any form before 00.01am on Thursday 24 July 2014
 
Summary
 
snip...see full text ;
 
Wednesday, July 23, 2014
 
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Thursday, July 24, 2014
 
Government must do more to reduce risk of vCJD infection
 
 
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Tuesday, December 30, 2014
 
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Wednesday, January 28, 2015
 
Another new prion disease: relationship with central and peripheral amyloidoses
 
here we go again...
 
 
Wednesday, November 12, 2014
 
Molecular Modeling of Prion Transmission to Humans
 
snip...
 
Brains from humans or transgenic mice expressing a human PrP with methionine at codon 129 of PRNP provided the best substrates to amplify vCJD and BSE PrPsc [6,7], suggesting that PMCA may reproduce faithfully the genotypic transmission barrier. It was thus proposed as a means to evaluate the zoonotic risk associated with emerging prion strains (Nor98 in sheep, L-type BSE in cattle). Indeed, while classical BSE strain has been recognized to be at the origin of vCJD in humans, L-BSE is considered to be a sporadic form of prion disease in cattle, differing in many aspects (epidemiology, neuropathology, biochemical features) from the C-BSE strain.
 
***Moreover, L-BSE has been transmitted more easily to transgenic mice overexpressing a human PrP [13,14] or to primates [15,16] than C-BSE.
 
***It has been suggested that some sporadic CJD subtypes in humans may result from an exposure to the L-BSE agent.
 
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snip...
 
 
Tuesday, November 04, 2014
 
Towards an Age-Dependent Transmission Model of Acquired and Sporadic Creutzfeldt-Jakob Disease
 
 
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Development of Dose-Response Models of Creutzfeldt-Jakob Disease Infection in Nonhuman Primates for Assessing the Risk of Transfusion-Transmitted variant Creutzfeldt-Jakob Disease
 
 
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Confirmed Variant Creutzfeldt-Jakob Disease (variant CJD) Case in Texas
 
Updated: October 7, 2014
 
CDC and the Texas Department of State Health Services (DSHS) have completed the investigation of the recently reported fourth vCJD case in the United States. It confirmed that the case was in a US citizen born outside the Americas and indicated that the patient's exposure to the BSE/vCJD agent most likely occurred before he moved to the United States; the patient had resided in Kuwait, Russia and Lebanon. The completed investigation did not support the patient's having had extended travel to European countries, including the United Kingdom, or travel to Saudi Arabia. The specific overseas country where this patient’s infection occurred is less clear largely because the investigation did not definitely link him to a country where other known vCJD cases likely had been infected.
 
 
>>>the patient had resided in Kuwait, Russia and Lebanon.
 
>>>The completed investigation did not support the patient's having had extended travel to European countries, including the United Kingdom, or travel to Saudi Arabia.
 
Monday, June 02, 2014
 
Confirmed Variant CJD Case in Texas
 
 
Terry S. Singeltary Sr.
 
Sunday, December 14, 2014
 
ALERT new variant Creutzfeldt Jakob Disease nvCJD or vCJD, sporadic CJD strains, TSE prion aka Mad Cow Disease United States of America Update December 14, 2014 Report
 
 
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Prion-like transmission and spreading of tau pathology
 
 
Greetings again Friends, Neighbors, and Colleagues,
 
I would kindly like to follow up on ‘vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type prion disease, what it ???’ ran across an old paper from 1984, that some might find interest in, and I will update the link with this old science paper from 1984, a 2010 paper from Japan, and some information on scrapie transmission. The paper from Japan first, then the 1984 paper, and then the scrapie transmission studies.
 
***The occurrence of contact cases raises the possibility that transmission in families may be effected by an unusually virulent strain of the agent.
 
 
From: Terry S. Singeltary Sr.
 
Sent: Saturday, November 15, 2014 9:29 PM
 
To: Terry S. Singeltary Sr.
 
Subject: THE EPIDEMIOLOGY OF CREUTZFELDT-JAKOB DISEASE R. G. WILL 1984
 
THE EPIDEMIOLOGY OF CREUTZFELDT-JAKOB DISEASE
 
R. G. WILL
 
1984
 
snip...
 
 
Self-Propagative Replication of Ab Oligomers Suggests Potential Transmissibility in Alzheimer Disease
 
Received July 24, 2014; Accepted September 16, 2014; Published November 3, 2014
 
 
Singeltary comment ;
 
 
 
 
>>> Variant CJD and blood transfusion: are there additional cases?
 
 
TSE Prion and blood transfusion: will there be additional cases?  this should be the concern. ...TSS