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Latest results of HPA study on vCJD-related abnormal prion proteins in extracted tonsils

Posted May 24 2009 11:25pm
Latest results of HPA study on vCJD-related abnormal prion proteins in extracted tonsils

In 2004, the Health Protection Agency launched the National Anonymous Tissue Archive (NATA) to determine prevalence of asymptomatic vCJD in the population by looking for the prion protein associated with vCJD in extracted tonsils. The tonsils are one of the sites in the body where, once infected, vCJD prions can accumulate (other sites include the spleen, appendix, lymph nodes, spinal cord and brain).

Awareness of the prevalence of vCJD in the population is important to determine the level of public health risk and to limit the impact of infection or plan healthcare interventions for people who may develop the disease.

Newly published results from the study [1, 2] suggest there may be fewer undetected asymptomatic cases of vCJD in the population than were previously expected.

The survey will eventually collect and analyse 100,000 samples of discarded tonsil tissue but no evidence of the abnormal prion protein has been found in any of the 63,000 tonsil samples analysed to date.

When the archive was established it was estimated that up to 50 of the 100,000 samples could contain the abnormal prion protein.

References

1. Prevalence of disease related prion protein in anonymous tonsil specimens in Britain: a cross-sectional opportunistic survey, J Clewley et al, BMJ 2009; 338: b1442.

2. “Latest research into prevalence of vCJD consistent with findings of existing studies” (HPA press release, 22 May 2009). HPA website: National Press Releases.


http://www.hpa.org.uk/hpr/archives/2009/news2009.htm


Latest research into prevalence of vCJD consistent with findings of existing studies 22 May 2009

Latest estimates of the number of people asymptomatic for variant Creutzfeldt-Jakob disease (vCJD) in the population remain very low, according to results from a large scale study of tonsil tissue by the Health Protection Agency, published in today's BMJ (Friday 22nd May 2009).

No evidence of the abnormal prion protein associated with vCJD was found in any of the 63,000 samples analysed.

In 2004, the Health Protection Agency launched the National Anonymous Tissue Archive (NATA) to determine prevalence of asymptomatic vCJD in the population, by looking for the prion protein associated with vCJD in extracted tonsils. The tonsils are one of the sites in the body where, once infected, vCJD prions can accumulate (other sites include the spleen, appendix, lymph nodes, spinal cord and brain).

Awareness of the prevalence of vCJD in the population is important to determine the level of risk to the population and to limit the impact of infection or plan healthcare interventions for people who may develop the disease.

The survey has already involved collection and analysis of 63,000 discarded tonsils, and will continue on until a total of 100,000 samples of leftover tonsil tissue have been examined.

When the archive was established it was estimated that up to 50 of the 100,000 samples could contain the abnormal prion protein, however so far none of the samples are positive.

The findings suggest there may be fewer undetected asymptomatic cases of vCJD in the population than were previously expected. However, only by testing a larger number of tonsils and continuing and expanding on the current survey, will scientists be confident that the prevalence is lower than earlier estimates.

Dr Jonathan Clewley, an expert on vCJD at the Health Protection Agency, said: "It may be that we have seen the worst of vCJD already, although we need to keep vigilant and implement appropriate public health measures to prevent any possible secondary spread of disease.

"Estimating the prevalence of people who are carrying vCJD unknowingly is important in guiding our public health response to this disease and ensuring all necessary precautions are taken to reduce this risk of further transmission of the agent through surgical operations and other healthcare settings.

"Further studies are planned to strengthen prevalence estimates, these will involve large scale anonymous tissue surveys, and continuation with the testing of tonsil specimens especially in the older age groups."

Ends

Notes to editors

1. The National Anonymous Tissue Archive (NATA) is managed by the CJD Team at the Health Protection Agency and the Transmissible Spongiform Encephalopathies Unit for the Department of Health.

2. The findings are published in the BMJ paper; Prevalence of disease related prion protein in anonymous tonsil specimens in Britain: a cross-sectional opportunistic survey, J Clewley et al. BMJ 2009; 338: b1442.

3. 63,007 samples were taken, of which 12,763 were from the birth cohort where most cases had arisen (1961-1985), 19,908 were in the 1985-1995 cohort who would have also been exposed to BSE from infected meat or meat products. None of the samples that were investigated by immunohistochemistry or immunoblotting were positive for the presence of PrPCJD.

4. The archive is completely anonymous; after tonsils are removed, they are separated from any identifiable patient information before going into the archive. Therefore if abnormal prion proteins are found in a tonsil sample, the results cannot be passed back to the patient.

This anonymous procedure is used because the significance for an otherwise well person of finding abnormal prion protein in their tonsil tissue is unknown at present. The Research Ethics Committee that reviewed the study supported the view that the tonsils should be tested anonymously.

5. Since 1995 there have been 168 definite or probable cases of vCJD in Britain, resulting in 115 deaths from vCJD and 49 deaths thought likely to be due to vCJD. Back calculation based on these cases would suggest between 10 and 190 further clinical cases over the next ten years.

6. The NATA study is able to detect presence of the prion protein regardless of the genotype of the prion protein gene.

7. For further information on this press release please contact the Health Protection Agency's Centre for Infections press office on:

Kate Swan 020 8327 7097 Georgina Fletcher 020 8327 6690 Louise Brown 020 8327 7080 Alex Baker 020 8327 7098 David Daley 020 8327 664

Last reviewed: 21 May 2009


http://www.hpa.org.uk/webw/HPAweb&HPAwebStandard/HPAweb_C/1242914502235?p=123125



WHAT ABOUT AWARENESS OF THE SPORADIC CJD's ???



* Creutzfeldt's first patient in 1920 was aged 23

snip...


http://www.bseinquiry.gov.uk/files/yb/1995/10/26005001.pdf


Sunday, May 10, 2009

Identification and characterization of bovine spongiform encephalopathy cases diagnosed and not diagnosed in the United States

snip...

Saturday, February 28, 2009

NEW RESULTS ON IDIOPATHIC BRAINSTEM NEURONAL CHROMATOLYSIS "All of the 15 cattle tested showed that the brains had abnormally accumulated PrP" 2009

SEAC 102/2


http://bse-atypical.blogspot.com/2009/02/new-results-on-idiopathic-brainstem.htm


''THE LINE TO TAKE'' ON IBNC $$$ 1995 $$$

1995

page 9 of 14 ;

30. The Committee noted that the results were unusual. the questioned whether there could be coincidental BSE infection or contamination with scrapie. Dr. Tyrell noted that the feeling of the committee was that this did not represent a new agent but it was important to be prepared to say something publicly about these findings. A suggested line to take was that these were scientifically unpublishable results but in line with the policy of openness they would be made publicly available and further work done to test their validity. Since the BSE precautions were applied to IBNC cases, human health was protected. Further investigations should be carried out on isolations from brains of IBNC cases with removal of the brain and subsequent handling under strict conditions to avoid the risk of any contamination.

31. Mr. Bradley informed the Committee that the CVO had informed the CMO about the IBNC results and the transmission from retina and he, like the Committee was satisfied that the controls already in place or proposed were adequate. ...

snip... see full text


http://www.bseinquiry.gov.uk/files/yb/1995/06/21005001.pdf


LINE TO TAKE

18. There is nothing to add to CMO's statement in January 1994, in relation to both the safety of meat and to the diagnosis in the 16 year old girl.

snip...


http://www.bseinquiry.gov.uk/files/yb/1995/08/01006001.pdf


IMPORTANT - CONFIDENTIAL

LINE TO TAKE


http://www.bseinquiry.gov.uk/files/yb/1995/08/17006001.pdf


CJD FOURTH FARMER LINE TO TAKE, preparing for media storm ;


http://www.bseinquiry.gov.uk/files/yb/1995/09/29009001.pdf


http://www.bseinquiry.gov.uk/files/yb/1995/09/29013001.pdf


LINE TO TAKE ;


http://www.bseinquiry.gov.uk/files/yb/1995/10/23010001.pdf


IN CONFIDENCE

CJD IN YOUNG PEOPLE

* in the USA, a 16 year old in 1978

* in France, a 19 year old in 1982

* in Canada, a 14 year old of UK origin in 1988

* in Poland, cases in people aged 19, 23 and 27 were identified in a retrospective study (published 1991), having been originally misdiagnosed with a viral encephalitis;

* Creutzfeldt's first patient in 1920 was aged 23

snip...


http://www.bseinquiry.gov.uk/files/yb/1995/10/26005001.pdf


snip... see full text ;


http://bse-atypical.blogspot.com/2009/05/identification-and-characterization-of.


Manuscript Draft Manuscript Number: Title: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory Article Type: Personal View Corresponding Author: Mr. Terry S. Singeltary, Corresponding Author's Institution: na First Author: Terry S Singeltary, none Order of Authors: Terry S Singeltary, none; Terry S. Singeltary Abstract: TSEs have been rampant in the USA for decades in many species, and they all have been rendered and fed back to animals for human/animal consumption. I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2007.


http://www.regulations.gov/fdmspublic/ContentViewer?objectId=090000648027c28e&di


Thursday, July 10, 2008 A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008


http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnorm


Thursday, July 10, 2008 A New Prionopathy update July 10, 2008


http://cjdmadcowbaseoct2007.blogspot.com/2008/07/new-prionopathy-update-july-10-


Sunday, August 10, 2008 A New Prionopathy OR more of the same old BSe and sporadic CJD


http://creutzfeldt-jakob-disease.blogspot.com/2008/08/new-prionopathy-or-more-of


Sunday, March 16, 2008 MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE


http://bse-atypical.blogspot.com/2008/03/mad-cow-disease-terminology-uk-c-bse.ht


P26

TRANSMISSION OF ATYPICAL BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN HUMANIZED MOUSE MODELS

Liuting Qing1, Fusong Chen1, Michael Payne1, Wenquan Zou1, Cristina Casalone2, Martin Groschup3, Miroslaw Polak4, Maria Caramelli2, Pierluigi Gambetti1, Juergen Richt5*, and Qingzhong Kong1 1Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA; 2CEA, Istituto Zooprofilattico Sperimentale, Italy; 3Friedrich-Loeffler-Institut, Germany; 4National Veterinary Research Institute, Poland; 5Kansas State University, Diagnostic Medicine/Pathobiology Department, Manhattan, KS 66506, USA. *Previous address: USDA National Animal Disease Center, Ames, IA 50010, USA

Classical BSE is a world-wide prion disease in cattle, and the classical BSE strain (BSE-C) has led to over 200 cases of clinical human infection (variant CJD). Two atypical BSE strains, BSE-L (also named BASE) and BSE-H, have been discovered in three continents since 2004. The first case of naturally occurring BSE with mutated bovine PrP gene (termed BSE-M) was also found in 2006 in the USA. The transmissibility and phenotypes of these atypical BSE strains/isolates in humans were unknown. We have inoculated humanized transgenic mice with classical and atypical BSE strains (BSE-C, BSE-L, BSE-H) and the BSE-M isolate. We have found that the atypical BSE-L strain is much more virulent than the classical BSE-C. The atypical BSE-H strain is also transmissible in the humanized transgenic mice with distinct phenotype, but no transmission has been observed for the BSE-M isolate so far.

III International Symposium on THE NEW PRION BIOLOGY: BASIC SCIENCE, DIAGNOSIS AND THERAPY 2 - 4 APRIL 2009, VENEZIA (ITALY)


http://www.istitutoveneto.it/prion_09/Abstracts_09.pdf


Research Project: GENETIC AND BIOLOGICAL DETERMINANTS OF RESPIRATORY DISEASE SUSCEPTIBILITY Location: Animal Health Systems Research

Title: Association of a bovine prion gene haplotype with atypical BSE

Author

Clawson, Michael

Submitted to: Meeting Abstract Publication Type: Abstract Publication Acceptance Date: December 2, 2008 Publication Date: January 1, 2009 Citation: Clawson, M.L. 2009. Association of a bovine prion gene haplotype with atypical BSE [abstract]. Plant and Animal Genomes XVII Conference. Abstract No. W091. Available: http://www.intl-pag.org/17/abstracts/

Technical Abstract: Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, are a class of fatal neurodegenerative disorders that occur in humans, ruminants, cats, and mink. Three distinct TSEs afflict cattle: classical bovine spongiform encephalopathy (BSE), atypical H-type BSE, and atypical L-type BSE. Classical BSE was identified in the 1980s and is acquired by cattle through the consumption of feed contaminated with the infectious prion agent. Atypical BSEs have only recently been recognized as distinct cattle prion diseases and are extremely rare. The full extent of genetic susceptibilities to atypical BSEs is unknown; however, one atypical H-type case identified in the United States (2006) was most likely caused by a genetic mutation in the prion gene, E211K. We have identified an association of a bovine prion DNA haplotype with atypical BSE that is independent of E211K. The haplotype spans a portion of the prion gene that includes part of intron 2, the entire coding region of exon 3, and part of the three prime untranslated region of exon 3 (13 kb). Despite the low frequency of this haplotype among general cattle populations, it was present in a majority of H- and L-type atypical BSE cases from Canada, France, and the United States. This result indicates that there is a genetic component to atypical BSE susceptibility in addition to E211K.


http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=234699


I ask Professor Kong ;

Thursday, December 04, 2008 3:37 PM Subject: RE: re--Chronic Wating Disease (CWD) and Bovine Spongiform Encephalopathies (BSE): Public Health Risk Assessment

''IS the h-BSE more virulent than typical BSE as well, or the same as cBSE, or less virulent than cBSE? just curious.....''

Professor Kong reply ;

.....snip

''As to the H-BSE, we do not have sufficient data to say one way or another, but we have found that H-BSE can infect humans. I hope we could publish these data once the study is complete. Thanks for your interest.''

Best regards, Qingzhong Kong, PhD Associate Professor Department of Pathology Case Western Reserve University Cleveland, OH 44106 USA

END...TSS

I look forward to further transmission studies, and a true ENHANCED BSE/atypical BSE surveillance program put forth testing all cattle for human and animal consumption for 5 years. a surveillance program that uses the most sensitive TSE testing, and has the personnel that knows how to use them, and can be trusted. I look forward to a stringent mad cow feed ban being put forth, and then strictly enforced. we need a forced, not voluntary feed ban, an enhanced feed ban at that, especially excluding blood. we need some sort of animal traceability. no more excuses about privacy. if somebody is putting out a product that is killing folks and or has the potential to kill you, then everybody needs to know who they are, and where that product came from. same with hospitals, i think medical incidents in all states should be recorded, and made public, when it comes to something like a potential accidental transmission exposure event. so if someone is out there looking at a place to go have surgery done, if you have several hospitals having these type 'accidental exposure events', than you can go some place else. it only makes sense. somewhere along the road, the consumer lost control, and just had to take whatever they were given, and then charged these astronomical prices. some where along the line the consumer just lost interest, especially on a long incubating disease such as mad cow disease i.e. Transmissible Spongiform Encephalopathy. like i said before, there is much more to the mad cow story than bovines and eating a hamburger, we must start focusing on all TSE in all species. ...TSS


http://bse-atypical.blogspot.com/2009/02/atypical-bse-north-america-update.html


Sunday, May 17, 2009

WHO WILL WATCH THE CHILDREN ? SCHOOL LUNCH PROGRAM FROM DOWNER CATTLE UPDATE


http://downercattle.blogspot.com/2009/05/who-will-watch-children.html


Sunday, May 10, 2009

Meeting of the Transmissible Spongiform Encephalopathies Committee On June 12, 2009 (Singeltary submission)


http://tseac.blogspot.com/2009/05/meeting-of-transmissible-spongiform.html


Sunday, May 10, 2009

Identification and characterization of bovine spongiform encephalopathy cases diagnosed and not diagnosed in the United States


http://bse-atypical.blogspot.com/2009/05/identification-and-characterization-of.


Sunday, April 12, 2009 BSE MAD COW TESTING USA 2009 FIGURES Month Number of Tests

Feb 2009 -- 1,891

Jan 2009 -- 4,620


http://www.aphis.usda.gov/newsroom/hot_issues/bse/surveillance/ongoing_surv_resu


see full text ;


http://madcowtesting.blogspot.com/2009/04/bse-mad-cow-testing-usa-2009-figures.h


Atypical BSE North America Update February 2009


http://bse-atypical.blogspot.com/2009/02/atypical-bse-north-america-update.html


Monday, May 11, 2009

Rare BSE mutation raises concerns over risks to public health


http://bse-atypical.blogspot.com/2009/05/rare-bse-mutation-raises-concerns-over.


April 20, 2009

National Prion Disease Pathology Surveillance Center Cases Examined1 (December 31, 2008)

National Prion Disease Pathology Surveillance Center Cases Examined1 (December 31, 2008)

Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD 1996 & earlier 42 32 28 4 0 0 1997 115 68 59 9 0 0 1998 93 53 45 7 1 0 1999 115 69 61 8 0 0 2000 151 103 89 14 0 0 2001 210 118 108 9 0 0 2002 258 147 123 22 2 0 2003 273 176 135 41 0 0 2004 335 184 162 21 0 13 2005 346 193 154 38 1 0 2006 380 192 159 32 0 14 2007 370 212 185 26 0 0 2008 383 228 182 23 0 0 TOTAL 30715 17756 1490 254 4 2 1 Listed based on the year of death or, if not available, on year of referral; 2 Cases with suspected prion disease for which brain tissue and/or blood (in familial cases) were submitted; 3 Disease acquired in the United Kingdom; 4 Disease acquired in Saudi Arabia; 5 Includes 20 cases in which the diagnosis is pending, and 17 inconclusive cases; 6 Includes 25 cases with type determination pending in which the diagnosis of vCJD has been excluded. Rev 2/13/09 National


http://www.cjdsurveillance.com/pdf/case-table.pdf


http://www.cjdsurveillance.com/resources-casereport.html


http://www.aan.com/news/?event=read&article_id=4397&page=72.45.45


>>> *5 Includes 20 cases in which the diagnosis is pending, and 17 inconclusive cases; *6 Includes 25 cases with type determination pending in which the diagnosis of vCJD has been excluded. < < <


Greetings,

it would be interesting to know what year these atypical cases occurred, as opposed to lumping them in with the totals only. are they accumulating ? did they occur in one year, two years, same state, same city ? location would be very interesting ? age group ? sex ? how was it determined that nvCJD was ruled out ? from 1997, the year i started dealing with this nightmare, there were 28 cases (per this report), up until 2007 where the total was 185 cases (per this report), and to date 2008 is at 182. a staggering increase in my opinion, for something that just happens spontaneously as some would have us believe. i don't believe it, not in 85%+ of all sporadic CJD cases. actually, i do not believe yet that anyone has proven that any of the sporadic CJD cases have been proven to be a spontaneous misfolding of a protein. there are many potential routes and sources for the sporadic CJD's. ...TSS


Sunday, April 12, 2009 r-calf and the USA mad cow problem, don't look, don't find, and then blame Canada


http://prionunitusaupdate2008.blogspot.com/2009/04/r-calf-and-usa-mad-cow-proble


snip...SEE FULL TEXT BELOW ! Monday, April 20, 2009 National Prion Disease Pathology Surveillance Center Cases Examined1 (December 31, 2008)


http://prionunitusaupdate2008.blogspot.com/2009/04/national-prion-disease-pathol


Friday, November 30, 2007

CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION

snip...

i am reminded of a few things deep throat told me years ago;


=================================================2001


The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people......... Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie..... why???? than the UK... then would the same mechanisms that make different strains of scrapie here make different strains of BSE... if the patterns are different in sheep and mice for scrapie..... could not the BSE be different in the cattle, in the mink, in the humans....... I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........ bse..... scrapie

Scrape the damn slide and put it into mice..... wait..... chop up the mouse brain and and spinal cord........ put into some more mice..... dammit amplify the thing and start the damned research..... This is NOT rocket science... we need to use what we know and get off our butts and move.... the whining about how long everything takes..... well it takes a whole lot longer if you whine for a year and then start the research!!!

Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde..... for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year....... it is a big fat sponge... the agent continues to eat the brain ...... you can't make slides anymore because the agent has never stopped........ and the old slides that are stained with Hemolysin and Eosin...... they get holier and holier and degenerate and continue... what you looked at 6 months ago is not there........ Gambetti better be photographing every damned thing he is looking at.....

Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........ if you want to move this thing along and shake the earth.... then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........ I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........ forget any action........ it is ALL gonna be sporadic!!! And, if there is a case....... there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats.... and this may be their biggest downfall...

Thanks as always for your help. (Recently had a very startling revelation from a rather senior person in government here.......... knocked me out of my chair........ you must keep pushing. If I was a power person.... I would be demanding that there be at least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the wood work as there is money to be made!!!

In short: "FIRE AT WILL"!!! for the very dumb.... who's "will"! "Will be the burden to bare if there is any coverup!"

again it was said years ago and it should be taken seriously.... BSE will NEVER be found in the US!

As for the BSE conference call... I think you did agreat service to freedom of information and making some people feign integrity... I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing.

You need to watch your back........ but keep picking at them....... like a buzzard to the bone... you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.)


================================================


greetings again voice,

then i remind everyone to read this;

'As implied in the Inset 25 we must not assume that transmission of BSE to other species will invariably present pathology typical of a scrapie-like disease.'


http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf


CWRU CJD QUESTIONNAIRE HISTORY


http://cjdquestionnaire.blogspot.com/


TSS
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