vCJD transfusion-associated Fourth Case UK

Prionemia and Leukocyte-Platelet-Associated Infectivity in Sheep Transmissible Spongiform Encephalopathy Models

2012-01-26T11:13:00.000-08:00

Prionemia and Leukocyte-Platelet-Associated Infectivity in Sheep Transmissible Spongiform Encephalopathy Models

Caroline Lacrouxa, Didier Vilettea, Natalia Fernández-Borgesb, Claire Litaisea, Séverine Lugana, Nathalie Moreld, Fabien Corbièrea, Stéphanie Simond, Hugh Simmonse, Pierrette Costesa, Jean-Louis Weisbeckerf, Isabelle Lantierg, Frederic Lantierg, François Schelchera, Jacques Grassid, Joaquin Castillab,c and Olivier Andréolettia

+ Author Affiliations

aUMR INRA ENVT 1225, Interactions Hôtes Agents Pathogènes, Ecole Nationale Vétérinaire de Toulouse, Toulouse, France

bCIC bioGUNE, Parque Tecnológico de Bizkaia, Derio, Spain

cIKERBASQUE, Basque Foundation for Science, Bilbao, Spain

dCEA, Service de Pharmacologie et d'Immunoanalyse, IBiTec-S, DSV, CEA/Saclay, Gif sur Yvette, France

eVLA Weybridge, ASU, New Haw, Addlestone, Surrey, United Kingdom

fINRA Domaine de Langlade, Pompertuzat, France

gINRA IASP, Centre INRA de Tours, Nouzilly, France

ABSTRACT

The dynamics of the circulation and distribution of transmissible spongiform encephalopathy (TSE) agents in the blood of infected individuals remain largely unknown. This clearly limits the understanding of the role of blood in TSE pathogenesis and the development of a reliable TSE blood detection assay. Using two distinct sheep scrapie models and blood transfusion, this work demonstrates the occurrence of a very early and persistent prionemia. This ability to transmit disease by blood transfusion was correlated with the presence of infectivity in white blood cells (WBC) and peripheral blood mononucleated cells (PBMC) as detected by bioassay in mice overexpressing the ovine prion protein PrP (tg338 mice) and with the identification of abnormal PrP in WBC after using protein misfolding cyclic amplification (PMCA). Platelets and a large variety of leukocyte subpopulations also were shown to be infectious. The use of endpoint titration in tg338 mice indicated that the infectivity in WBC (per ml of blood) was 106.5-fold lower than that in 1 g of posterior brainstem sample. In both WBC and brainstem, infectivity displayed similar resistance to PK digestion. The data strongly support the concept that WBC are an accurate target for reliable TSE detection by PMCA. The presence of infectivity in short-life-span blood cellular elements raises the question of the origin of prionemia.

FOOTNOTES Received 11 October 2011. Accepted 18 November 2011. Address correspondence to O. Andreoletti, o.andreoletti@envt.fr.

Published ahead of print 7 December 2011

http://jvi.asm.org/content/86/4/2056.abstract?sid=8698b701-1122-4a5c-bcec-f959a6dda499

Wednesday, August 24, 2011

All Clinically-Relevant Blood Components Transmit Prion Disease following a Single Blood Transfusion: A Sheep Model of vCJD
http://transmissiblespongiformencephalopathy.blogspot.com/2011/08/all-clinically-relevant-blood.html

Wednesday, August 24, 2011

There Is No Safe Dose of Prions
http://transmissiblespongiformencephalopathy.blogspot.com/2011/08/there-is-no-safe-dose-of-prions.html

Tuesday, September 14, 2010

Transmissible Spongiform Encephalopathies Advisory Committee; Notice of Meeting October 28 and 29, 2010 (COMMENT SUBMISSION)
http://tseac.blogspot.com/2010/09/transmissible-spongiform_14.html

Monday, February 7, 2011

FDA’s Currently-Recommended Policies to Reduce the Possible Risk of Transmission of CJD and vCJD by Blood and Blood Products 2011 ???
http://tseac.blogspot.com/2011/02/fdas-currently-recommended-policies-to.html

Thursday, December 29, 2011

Aerosols An underestimated vehicle for transmission of prion diseases?

PRION www.landesbioscience.com

please see more on Aerosols and TSE prion disease here ;
http://transmissiblespongiformencephalopathy.blogspot.com/2011/12/aerosols-underestimated-vehicle-for.html

Tuesday, January 17, 2012

American Red Cross Fined $9.6 Million for Blood-Safety Lapses AGAIN

http://transmissiblespongiformencephalopathy.blogspot.com/2012/01/american-red-cross-fined-96-million-for.html

TSS

A blood test for variant Creutzfeldt‐Jakob disease: briefing note for patients, carers and health professionals

2011-12-18T09:54:00.000-08:00

A blood test for variant Creutzfeldt‐Jakob disease: briefing note for patients, carers and health professionals

A blood test for variant Creutzfeldt‐Jakob disease (vCJD) has been an important goal of medical research laboratories and companies around the world for many years. It has been very difficult to achieve because the infectious agent (germ) causing vCJD, known as a prion, has unique features that mean that the sensitive methods doctors normally use to detect the presence of a germ (detecting the body’s antibody response to the germ or the germ’s own genetic material) do not work.

The Medical Research Council (MRC) Prion Unit, working with the NHS National Prion Clinic at the National Hospital for Neurology and Neurosurgery (NHNN) in London, has now developed an entirely new type of test following a number of years of intensive research.

The test is at an early (prototype) stage but is able to correctly identify the large majority of patients with symptoms of vCJD and has not yet given any false results in patients with other brain diseases or in healthy individuals. We think this is an important breakthrough and it raises a number of issues which need to be carefully considered. Details of the test have been published by the leading medical journal, the Lancet, on 3rd February 2011. The full text of the paper is available here.

This brief article describes CJD and other so‐called prion diseases, why a blood test is important, how the test works and how to approach us at the National Prion Clinic to inquire further about this test. It is important to be cautious about this news, because although the results so far are very encouraging, we want to go on to look at blood samples from much larger numbers of healthy people and those with other brain diseases to get a better idea of how specific the test is in practice. This will be vital before a version of this test could be considered to routinely screen healthy blood donors.

What are prion diseases? Also known as transmissible spongiform encephalopathies, prion diseases are a group of rare fatal conditions affecting the brain. Prion diseases are caused by one of the body’s normal proteins, called the prion protein, changing its shape and forming clumps of protein in the brain. This process damages and eventually kills brain cells. In humans, there are three different ways these diseases can start. The commonest form is called sporadic CJD and this form is seen all over the world and appears to occur at random as an unlucky event when the production of prions in the brain is triggered spontaneously. Secondly, the disease can be passed down from generation to generation as a genetic condition in some families with a faulty prion protein gene. Thirdly, and most importantly from the point of view of this new test, someone can “catch” a prion infection by being exposed to infectious prions.

These illnesses affect both animals and humans. The animal prion diseases include scrapie, a common prion disease affecting sheep and goats which is not thought to pose a threat to human health and bovine spongiform encephalopathy (BSE or mad cow disease) in cattle which can jump species to infect humans. BSE prions are responsible for variant CJD which was first recognised in 1996 and which has so far affected about 200 people, most from the UK. It is thought that people become infected by BSE prions by eating food containing material from BSE‐infected cattle, although other sources of exposure are possible. Much of the UK population born before 1996 (when rigorous measures to limit exposure were enforced) have potentially been exposed to BSE‐contaminated food and the number of people who may carry the infection but remain healthy is unknown.

Why is a blood test important? vCJD (as with other forms of CJD) tends to be diagnosed only when the patient has had the disease for some time and has developed symptoms that are associated with extensive damage to the brain. There are several reasons why this is the case. The early symptoms of the disease (such as anxiety, depression and tingling pains in the legs) have many much more common causes and so doctors will understandably not attribute these symptoms to something much more serious until other features such as difficulty with movement or balance and loss of mental abilities occur. At this stage, it will be apparent there is a serious brain condition but a series of tests are required to make the diagnosis and these take time to organise and interpret. Because the disease itself typically progresses quite rapidly (over weeks and months), the patient is likely to be showing quite advanced symptoms by the time a confident diagnosis is reached. A simple blood test gives us an opportunity to make the diagnosis at a much earlier stage. While at present there is no treatment we know is effective in stopping progression of these diseases, an early diagnosis does avoid the need for other tests and gives the patient and their family a clear answer. This enables them to make the best use of their time together and spend less of this precious time in hospital. However, experimental drugs are being developed at the MRC Prion Unit and elsewhere with a view to clinical trials in the next few years and we would want to try such treatments at the earliest stage before irreversible brain damage has occurred.

It is now known that vCJD can be passed on by blood transfusion. Several vCJD patients had been blood donors before they developed symptoms of the disease. To date, three individuals who had received blood transfusions from such donors have themselves developed and died from the disease. A further individual, who had also received prion‐infected blood, died of unrelated causes but showed evidence of prion infection at autopsy examination.

Only a very small number of individuals are definitely known to have received such potentially infected blood transfusions. However, several thousand individuals have been notified by the Health Protection Agency that they have received possibly infected blood products such as plasma, clotting factors, or purified antibodies. One individual who had received a clotting factor from a donor who went on to get vCJD died of unrelated causes but showed signs of vCJD infection at autopsy. It is not known whether this individual would have gone on to develop the disease had he not died of other causes.

Prion diseases are known to exist in “carrier states” in laboratory animals and these would be expected in humans too. A “carrier” is a person infected with prions but who does not show any signs of disease in their natural lifetime. Such carrier states are well recognised with other infectious diseases in humans. In the UK population, following an anonymous study of archived tissue specimens, the Department of Health uses an estimate that 1 in 4000 individuals may be silently infected with vCJD prions in its risk calculations. There is considerable uncertainty about this figure, that is, the true number could be significantly higher or lower than 1 in 4000. It is also not known how many of those infected will eventually go on to develop the disease itself. We do know that incubation periods in human prion diseases can be very long, over 50 years in some cases. As these infected but healthy individuals cannot currently be identified in the population, many will be active blood donors and could pass on infection to other people in this way or by medical and surgical instruments used on them becoming contaminated by prions (since prions are quite resistant to normal sterilisation methods). The National Blood Service has taken several actions to try to minimise this risk, for example, by removing white cells from blood, however it is uncertain how effective these measures are at reducing risk, or indeed whether they are really justified should the real number of infected people turn out to be extremely small.

A future development of our blood test may allow us to screen donated blood and further increase the safety of blood transfusions. Also it may in the future allow individuals who have been exposed to vCJD infection to find out if there is evidence that the infection has taken hold in their body. However, considerable further research will need to be done first to find out how specific the test is when tested on large numbers of health donors and to understand how good the test will be at detecting infected blood from healthy individuals rather than those with the established disease.

How well does the test work? It has been hard to develop a test for prion disease because the body’s immune system does not fight off prion infection by making antibodies (that can be readily detected in a blood test) in the same way it does against germs like bacteria or viruses. It has been challenging to develop a test that can distinguish between the normal prion protein, which we all have in our blood, and the abnormal form linked to the disease which is chemically very similar.

Scientists in the MRC Prion Unit have developed a prototype test. This involves taking a small blood sample from a patient as with any other blood test. A small sample of blood is mixed with special metal beads to which the rogue prion proteins stick tightly. These are then washed to remove the normal prion protein and other blood components that would interfere with the test. Finally, the amount of rogue prion protein attached to the beads is measured using antibodies we have developed that bind very tightly to the prion protein.

The test was applied to a number patient samples including from patients with vCJD, those with sporadic CJD, other neurological diseases that might be confused with vCJD and a number of healthy blood donors. As vCJD is a rare disease, only relatively small numbers of samples were available for this testing. All samples were given code numbers and the scientists carrying out the test in our laboratory did not know which sample was which. We were able to try the test on 21 samples from different vCJD patients. 15 of these 21 patient samples (around 70%) were shown to be positive by the test. So far, all samples from other neurological diseases or healthy blood donors have tested negative but only relatively small numbers of these have been looked at so far (about 200). We are testing larger numbers of samples now. At present the test does not work in other forms of prion disease such as sporadic CJD but we are hoping this will be possible with further work in the future.

What happens now and how is the test going to be made available? We are ready to use the test to assist with diagnosis of patients who are suspected of having vCJD or other diseases that might be mistaken for vCJD. Working with neurological colleagues to begin to use the test will also help us get more information on the test itself and hopefully lead to further improvements and understanding of its usefulness. A request card needs to be completed which can be obtained here. We require at least 2 x 5ml EDTA vacutainer tubes. For sample delivery please see further details here. While we are working to increase the throughput of the test, at this stage it remains relatively labour intensive. Whilst we will attempt to return results at the earliest opportunity, clinicians should allow up to four weeks for results. Please call the Clinic for further details.

The National Prion Clinic at the National Hospital for Neurology is happy to take telephone enquiries about suspected prion disease patients. We are particularly interested in referrals of patients at an early stage of their illness when diagnosis is most difficult.

Please visit the NHS National Prion Clinic website http://www.uclh.nhs.uk/ourservices/servicea-z/neuro/npc/Pages/Home.aspx for more details and telephone/email contact details.

http://www.prion.ucl.ac.uk/clinic-services/investigations-tests/#BloodTest

hmmm, most all of the iatrogenic CJD cases have been sporadic type iCJD. only the 5 or so cases (documented) has been associated with blood transfusion from vCJD. seems it would be more important to find a blood test for the sporadic CJD types that pass it on via the friendly fire and or pass if forward mode. why is it the UK scientist can develop and implement a test for their vCJD cases, but yet it can’t be done for the sporadic CJD cases in North America and or the UK ?

from the iatrogenic aspect, and considering that 85%+ of all human TSE is of the sporadic CJD strains, and the potential of iatrogenic transmission there from, seems to me we should be testing for sporadic CJD, considering also now that science has linked atypical BSE and atypical Scrapie to the sporadic CJD in humans, and most likely some cases from CWD once science crawls along and finally admits..............oops, I mean proves it.

you know the rest of the story $$$

FC5.1.1

Transmission Results in Squirrel Monkeys Inoculated with Human sCJD, vCJD, and GSS Blood Specimens: the Baxter Study

Brown, P1; Gibson, S2; Williams, L3; Ironside, J4; Will, R4; Kreil, T5; Abee, C3 1Fondation Alliance BioSecure, France; 2University of South Alabama, USA; 3University of Texas MD Anderson Cancer Center, USA; 4Western General Hospital, UK; 5Baxter BioSience, Austria

Background: Rodent and sheep models of Transmissible Spongiform Encephalopathy (TSE) have documented blood infectivity in both the pre-clinical and clinical phases of disease. Results in a (presumably more appropriate) non-human primate model have not been reported.

Objective: To determine if blood components (red cells, white cells, platelets, and plasma) from various forms of human TSE are infectious.

Methods: Blood components were inoculated intra-cerebrally (0.1 ml) and intravenously (0.5 ml) into squirrel monkeys from 2 patients with sporadic Creutzfeldt- Jakob disease (sCJD) and 3 patients with variant Creutzfeldt-Jakob disease (vCJD). Additional monkeys were inoculated with buffy coat or plasma samples from chimpanzees infected with either sCJD or Gerstmann-StrÃ¤ussler-Scheinker disease (GSS). Animals were monitored for a period of 5 years, and all dying or sacrificed animals had post-mortem neuropathological examinations and Western blots to determine the presence or absence of the misfolded prion protein (PrPTSE).

Results: No transmissions occurred in any of the animals inoculated with blood components from patients with sporadic or variant CJD. All donor chimpanzees (sCJD and GSS) became symptomatic within 6 weeks of their pre-clinical phase plasmapheresis, several months earlier than the expected onset of illness. One monkey inoculated with purified leukocytes from a pre-clinical GSS chimpanzee developed disease after 36 months.

Conclusion: No infectivity was found in small volumes of blood components from 4 patients with sporadic CJD and 3 patients with variant CJD. ***However, a single transmission from a chimpanzee-passaged strain of GSS shows that infectivity may be present in leukocytes, and the shock of general anaesthesia and plasmspheresis appears to have triggered the onset of illness in pre-clinical donor chimpanzees.

Saturday, September 5, 2009

TSEAC MEETING FEBRUARY 12, 2004 THE BAXTER STUDY GSS

snip...

http://tseac.blogspot.com/2011/06/tseac-meeting-august-1-2011-donor.html

Wednesday, June 29, 2011

TSEAC Meeting August 1, 2011 donor deferral Saudi Arabia vCJD risk blood and blood products

http://tseac.blogspot.com/2011/06/tseac-meeting-august-1-2011-donor.html

Transmission of sporadic Creutzfeldt-Jakob disease by blood transfusion: risk factor or possible biases

Maria Puopolo, Anna Ladogana, Vito Vetrugno, Maurizio Pocchiari

Article first published online: 7 JAN 2011

DOI: 10.1111/j.1537-2995.2010.03004.x

© 2010 American Association of Blood Banks

BACKGROUND: The occurrence of transfusion transmissions of variant Creutzfeldt-Jakob disease (CJD) cases has reawakened attention to the possible similar risk posed by other forms of CJD.

STUDY DESIGN AND METHODS: CJD with a definite or probable diagnosis (sporadic CJD, n = 741; genetic CJD, n = 175) and no-CJD patients with definite alternative diagnosis (n = 482) with available blood transfusion history were included in the study. The risk of exposure to blood transfusion occurring more than 10 years before disease onset and for some possible confounding factors was evaluated by calculating crude odds ratios (ORs). Variables with significant ORs in univariate analyses were included in multivariate logistic regression analyses.

RESULTS: In the univariate model, blood transfusion occurring more than 10 years before clinical onset is 4.1-fold more frequent in sporadic CJD than in other neurologic disorders. This significance is lost when the 10-year lag time was not considered. Multivariate analyses show that the risk of developing sporadic CJD after transfusion increases (OR, 5.05) after adjusting for possible confounding factors. Analysis conducted on patients with genetic CJD did not reveal any significant risk factor associated with transfusion.

CONCLUSION: This is the first case-control study showing a significant risk of transfusion occurring more than 10 years before clinical onset in sporadic CJD patients. It remains questionable whether the significance of these data is biologically plausible or the consequence of biases in the design of the study, but they counterbalance previous epidemiologic negative reports that might have overestimated the assessment of blood safety in sporadic CJD.

http://onlinelibrary.wiley.com/doi/10.1111/j.1537-2995.2010.03004.x/abstract;jsessionid=656FB07EE4507FB2632BA3E691D3B824.d03t02

http://www.whale.to/v/singeltary4.html

http://www.whale.to/v/singeltary3.html

Wednesday, March 2, 2011

Transmissible Spongiform Encephalopathies Advisory Committee Meeting Transcript

Posted: 3/2/2011 Posted: 3/2/2011

http://tseac.blogspot.com/2011/03/transmissible-spongiform.html

Thursday, June 23, 2011

Experimental H-type bovine spongiform encephalopathy characterized by plaques and glial- and stellate-type prion protein deposits

http://transmissiblespongiformencephalopathy.blogspot.com/2011/06/experimental-h-type-bovine-spongiform.html

Thursday, July 21, 2011

A Second Case of Gerstmann-Sträussler-Scheinker Disease Linked to the G131V Mutation in the Prion Protein Gene in a Dutch Patient Journal of Neuropathology & Experimental Neurology:

August 2011 - Volume 70 - Issue 8 - pp 698-702

http://transmissiblespongiformencephalopathy.blogspot.com/2011/07/second-case-of-gerstmann-straussler.html

Wednesday, August 24, 2011

All Clinically-Relevant Blood Components Transmit Prion Disease following a Single Blood Transfusion: A Sheep Model of vCJD

http://transmissiblespongiformencephalopathy.blogspot.com/2011/08/all-clinically-relevant-blood.html

Wednesday, August 24, 2011

There Is No Safe Dose of Prions

http://transmissiblespongiformencephalopathy.blogspot.com/2011/08/there-is-no-safe-dose-of-prions.html

Saturday, December 3, 2011

Candidate Cell Substrates, Vaccine Production, and Transmissible Spongiform Encephalopathies

Volume 17, Number 12—December 2011

http://transmissiblespongiformencephalopathy.blogspot.com/2011/12/candidate-cell-substrates-vaccine.html

Friday, December 16, 2011

Creutzfeldt-Jacob Disease Question Asked by Lord Walton of Detchant P-Capt filter

http://creutzfeldt-jakob-disease.blogspot.com/2011/12/creutzfeldt-jacob-disease-question.html

Wednesday, May 11, 2011 House of Commons CJD May 2011 UPDATE

http://transmissiblespongiformencephalopathy.blogspot.com/2011/05/house-of-commons-cjd-may-2011-update.html

Monday, December 12, 2011

Second iatrogenic CJD case confirmed Korea

http://creutzfeldt-jakob-disease.blogspot.com/2011/12/second-iatrogenic-cjd-case-confirmed.html

Thursday, December 08, 2011

A case of Iatrogenic Creutzfeldt Jakob Disease (iCJD) in a patient who had received a German-manufactured human dura mater graft 23 years ago

http://creutzfeldt-jakob-disease.blogspot.com/2011/12/case-of-iatrogenic-creutzfeldt-jakob.html

Saturday, November 19, 2011

Novel Prion Protein in BSE-affected Cattle, Switzerland

http://transmissiblespongiformencephalopathy.blogspot.com/2011/11/novel-prion-protein-in-bse-affected.html

Saturday, December 3, 2011

Isolation of Prion with BSE Properties from Farmed Goat

Volume 17, Number 12—December 2011

http://transmissiblespongiformencephalopathy.blogspot.com/2011/12/isolation-of-prion-with-bse-properties.html

Saturday, June 25, 2011

Transmissibility of BSE-L and Cattle-Adapted TME Prion Strain to Cynomolgus Macaque

"BSE-L in North America may have existed for decades"

http://transmissiblespongiformencephalopathy.blogspot.com/2011/06/transmissibility-of-bse-l-and-cattle.html

Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.

snip...

The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...

http://web.archive.org/web/20030516051623/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf

2010-2011

When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE. In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.

This study will contribute to a correct definition of specified risk material (SRM) in atypical BSE. The incumbent of this position will develop new and transfer existing, ultra-sensitive methods for the detection of atypical BSE in tissue of experimentally infected cattle.

http://www.prionetcanada.ca/detail.aspx?menu=5&dt=293380&app=93&cat1=387&tp=20&lk=no&cat2

2011 Monday, September 26, 2011

L-BSE BASE prion and atypical sporadic CJD

http://bse-atypical.blogspot.com/2011/09/l-bse-base-prion-and-atypical-sporadic.html

Monday, June 27, 2011

Zoonotic Potential of CWD: Experimental Transmissions to Non-Human Primates

http://chronic-wasting-disease.blogspot.com/2011/06/zoonotic-potential-of-cwd-experimental.html

This is an interesting editorial about the Mad Cow Disease debacle, and it's ramifications that will continue to play out for decades to come ; Monday, October 10, 2011 EFSA Journal 2011 The European Response to BSE: A Success Story snip... EFSA and the European Centre for Disease Prevention and Control (ECDC) recently delivered a scientific opinion on any possible epidemiological or molecular association between TSEs in animals and humans (EFSA Panel on Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical BSE prions as the only TSE agents demonstrated to be zoonotic so far but the possibility that a small proportion of human cases so far classified as "sporadic" CJD are of zoonotic origin could not be excluded. Moreover, transmission experiments to non-human primates suggest that some TSE agents in addition to Classical BSE prions in cattle (namely L-type Atypical BSE, Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic wasting disease (CWD) agents) might have zoonotic potential. snip...

http://www.efsa.europa.eu/en/efsajournal/pub/e991.htm?emt=1

http://www.efsa.europa.eu/en/efsajournal/doc/e991.pdf

see follow-up here about North America BSE Mad Cow TSE prion risk factors, and the ever emerging strains of Transmissible Spongiform Encephalopathy in many species here in the USA, including humans ;

http://transmissiblespongiformencephalopathy.blogspot.com/2011/10/efsa-journal-2011-european-response-to.html

Thursday, August 4, 2011

Terry Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health Crisis, Date aired: 27 Jun 2011 (SEE VIDEO)

http://transmissiblespongiformencephalopathy.blogspot.com/2011/08/terry-singeltary-sr-on-creutzfeldt.html

Sunday, August 21, 2011

The British disease, or a disease gone global, The TSE Prion Disease (SEE VIDEO)

http://transmissiblespongiformencephalopathy.blogspot.com/2011/08/british-disease-or-disease-gone-global.html

Saturday, March 5, 2011

MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE RISE IN NORTH AMERICA

http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/mad-cow-atypical-cjd-prion-tse-cases.html

Tuesday, November 01, 2011

Could we face the return of CJD? Experts fear it may lie dormant in thousands

http://creutzfeldt-jakob-disease.blogspot.com/2011/11/could-we-face-return-of-cjd-experts.html

Tuesday, November 08, 2011

Can Mortality Data Provide Reliable Indicators for Creutzfeldt-Jakob Disease Surveillance? A Study in France from 2000 to 2008 Vol. 37, No. 3-4, 2011

Original Paper

Conclusions:These findings raise doubt about the possibility of a reliable CJD surveillance only based on mortality data.

http://creutzfeldt-jakob-disease.blogspot.com/2011/11/can-mortality-data-provide-reliable.html

Thursday, August 12, 2010

USA Blood products, collected from a donor who was at risk for vCJD, were distributed July-August 2010

http://creutzfeldt-jakob-disease.blogspot.com/2010/08/usa-blood-products-collected-from-donor.html

Sunday, August 01, 2010

Blood product, collected from a donors possibly at increased risk for vCJD only, was distributed USA JULY 2010

http://vcjdtransfusion.blogspot.com/2010/08/blood-product-collected-from-donors.html

http://vcjdtransfusion.blogspot.com/

Saturday, August 13, 2011

Sensitive detection of prion proteins in blood

http://transmissiblespongiformencephalopathy.blogspot.com/2011/08/sensitive-detection-of-prion-proteins.html

Friday, July 8, 2011

Blood Test Could Quickly Detect Prion Diseases

Prion Diseases

http://transmissiblespongiformencephalopathy.blogspot.com/2011/07/blood-test-could-quickly-detect-prion.html

Monday, February 7, 2011

FDA’s Currently-Recommended Policies to Reduce the Possible Risk of Transmission of CJD and vCJD by Blood and Blood Products 2011 ???

http://tseac.blogspot.com/2011/02/fdas-currently-recommended-policies-to.html

Tuesday, September 14, 2010

Transmissible Spongiform Encephalopathies Advisory Committee; Notice of Meeting October 28 and 29, 2010 (COMMENT SUBMISSION)

http://tseac.blogspot.com/2010/09/transmissible-spongiform_14.html

Saturday, October 24, 2009

SaBTO Advisory Committee on the Safety of Blood, Tissues and Organs 2nd Public Meeting 27 October 2009

SaBTO

http://seac992007.blogspot.com/2009/10/sabto-advisory-committee-on-safety-of.html

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, July 27, 2008

Docket No, 04-047-l Regulatory Identification No. (RIN) 091O-AF46 NEW BSE SAFEGUARDS (comment submission)

-------- Original Message --------

Subject: Docket No, 04-047-l Regulatory Identification No. (RIN) 091O-AF46 NEW BSE SAFEGUARDS (comment submission)

Date: Sun, 11 Jul 2004 21:34:22 –0500

From: "Terry S. Singeltary Sr."

To: fdadockets@oc.fda.gov CC: regulations@aphis.usda.gov, burt.pritchett@fda.gov

http://madcowfeed.blogspot.com/2008/07/docket-no-04-047-l-regulatory.html

Response to Public Comments

on the

Harvard Risk Assessment of Bovine Spongiform Encephalopathy Update, October 31, 2005

INTRODUCTION

The United States Department of Agriculture’s Food Safety and Inspection Service (FSIS) held a public meeting on July 25, 2006 in Washington, D.C. to present findings from the Harvard Risk Assessment of Bovine Spongiform Encephalopathy Update, October 31, 2005 (report and model located on the FSIS website: http://www.fsis.usda.gov/Science/Risk_Assessments/index.asp). Comments on technical aspects of the risk assessment were then submitted to FSIS. Comments were received from Food and Water Watch, Food Animal Concerns Trust (FACT), Farm Sanctuary, R-CALF USA, Linda A Detwiler, and Terry S. Singeltary. This document provides itemized replies to the public comments received on the 2005 updated Harvard BSE risk assessment. Please bear the following points in mind:

http://www.fsis.usda.gov/PDF/BSE_Risk_Assess_Response_Public_Comments.pdf

Suppressed peer review of Harvard study October 31, 2002.

October 31, 2002 Review of the Evaluation of the Potential for Bovine Spongiform Encephalopathy in the United States Conducted by the Harvard Center for Risk Analysis, Harvard School of Public Health and Center for Computational Epidemiology, College of Veterinary Medicine, Tuskegee University Final Report Prepared for U.S. Department of Agriculture Food Safety and Inspection Service Office of Public Health and Science Prepared by RTI Health, Social, and Economics Research Research Triangle Park, NC 27709 RTI Project Number 07182.024

http://www.fsis.usda.gov/oa/topics/BSE_Peer_Review.pdf

Sunday, February 14, 2010

[Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)

http://bseusa.blogspot.com/2010/02/docket-no-fsis-2006-0011-fsis-harvard.html

Thursday, April 9, 2009

Docket No. FDA2002N0031 (formerly Docket No. 2002N0273) RIN 0910AF46 Substances Prohibited From Use in Animal Food or Feed; Final Rule: Proposed Thursday, April 09, 2009

Docket No. FDA2002N0031 (formerly Docket No. 2002N0273) RIN 0910AF46 Substances Prohibited From Use in Animal Food or Feed; Final Rule: Proposed

mailto:burt.pritchett@fda.hhs.gov

Greetings FDA et al,

I kindly wish to comment on the following ;

[Docket No. FDA-2002-N-0031] (formerly Docket No. 2002N-0273) RIN 0910-AF46

[Federal Register: April 9, 2009 (Volume 74, Number 67)] [Proposed Rules] [Page 16160-16161] From the Federal Register Online via GPO Access [wais.access.gpo.gov] [DOCID:fr09ap09-18]

http://madcowfeed.blogspot.com/2009/04/docket-no-fda2002n0031-formerly-docket.html

Owens, Julie

From: Terry S. Singeltary Sr. [flounder9@verizon.net]

Sent: Monday, July 24, 2006 1:09 PM To: FSIS RegulationsComments

Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE) Page 1 of 98 8/3/2006

Greetings FSIS, I would kindly like to comment on the following ;

http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf

http://www.scribd.com/doc/1490709/USDA-200600111

03-025IFA

03-025IFA-2

Terry S. Singeltary

From: Terry S. Singeltary Sr. [flounder9@verizon.net]

Sent: Thursday, September 08, 2005 6:17 PM

To: fsis.regulationscomments@fsis.usda.gov

Subject: [Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and Requirements for the Disposition of Non-Ambulatory Disabled Cattle

Greetings FSIS,

I would kindly like to submit the following to [Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and Requirements for the Disposition of Non-Ambulatory Disabled Cattle

http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf

http://www.fda.gov/ohrms/dockets/ac/05/slides/2005-4189oph_1.pdf

PLEASE be aware, for 4 years, the USDA fed our children all across the Nation (including TEXAS) dead stock downer cows, the most high risk cattle for BSE aka mad cow disease and other dangerous pathogens.

who will watch our children for CJD for the next 5+ decades ???

WAS your child exposed to mad cow disease via the NSLP ???

SCHOOL LUNCH PROGRAM FROM DOWNER CATTLE UPDATE

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

http://downercattle.blogspot.com/

DID YOUR CHILD CONSUME SOME OF THESE DEAD STOCK DOWNER COWS, THE MOST HIGH RISK FOR MAD COW DISEASE ???

you can check and see here ;

http://www.fns.usda.gov/fns/safety/pdf/Hallmark-Westland_byState.pdf

Docket Management Docket: 02N-0276 - Bioterrorism Preparedness; Registration of Food Facilities, Section 305

Comment Number: EC –254

Accepted - Volume 11

Sources: World Trade Atlas What is the level of passenger traffic arriving in the United States from the affected country? Approximately 185,000 direct flights from Greece arrived to US airports in fiscal year 2000. Also, an unknown number of passengers from Greece arrived via indirect flights. Under APHIS-PPQ's agriculture quarantine inspection monitoring, 584 air passengers from Greece were sampled for items of agricultural interest in fiscal year 2000. Of these passengers, 14 carried meat (non-pork) items that could potentially transmit pathogens that cause BSE; most passengers carried from one to two kilograms (kg) of meat, although one passenger in November 1999 carried 23 kg of meat in a suitcase. Florida, Massachusetts, and New York were the reported destinations of these passengers. None of the passengers with meat items reported plans to visit or work on a ranch or farm while in the US. Source: US Department of Transportation, and APHIS-PPQ Agricultural Quarantine Inspection data base

http://www.aphis.usda.gov/vs/ceah/cei/bse_greece0701.htm

Greetings list members, i just cannot accept this;

> 23 kg of meat in a suitcase (suitcase bomb...TSS)

> The data do not provide a species of origin code for these

> products, therefore they may not contain any ruminant product.

what kind of statement is this? how stupid do they think we are? it could also very well mean that _all_ of it was ruminant based products !

http://www.fda.gov/ohrms/dockets/dockets/02n0276/02N-0276-EC-254.htm

Docket Management Docket: 96N-0417 - Current Good Manufacturing Practice in Manufacturing, Packing, or Holding Dietary Ingredients a

Comment Number: EC –2

Accepted - Volume 7

http://www.fda.gov/ohrms/dockets/dailys/03/Mar03/031403/96N-0417-EC-2.htm

Docket Management Docket: 96N-0417 - Current Good Manufacturing Practice in Manufacturing, Packing, or Holding Dietary Ingredients a Comment Number: EC -2 Accepted - Volume 7

snip...

what did Paul Brown say about this previously;

i bring your attention to (page 500) Dr. Paul Brown statements;

253 1 DR. BOLTON: I have an additional question about 2 that. What is the assurance that additional locally sourced 3 tracheas are not added into that manufacturing process, thus 4 boosting the yield, if you will, but being returned to the 5 U.S. as being produced from U.S.-sourced raw material? 6 DR. McCURDY: Are there data to indicate how many 7 grams, or whatever, of infected brain are likely to infect 8 an organism, either animal or man, when taken orally? 9 DR. BROWN: If I am not mistaken, and I can be 10 corrected, I think a half a gram is enough in a cow, orally; [FULL TEXT ABOUT 600 PAGES] 3681t2.rtf http://www.fda.gov/ohrms/dockets/ac/cber01.htm

snip...

http://www.fda.gov/OHRMS/DOCKETS/DOCKETS/96n0417/96N-0417-EC-2.htm

Unregulated "foods" such as 'nutritional supplements' containing various extracts from ruminants, whether imported or derived from 3 US cattle/sheep/cervids ("antler velvet" extracts!) should be forbidden or at least very seriously regulated.

(neighbors Mom, whom also died from CJD, had been taking bovine based supplement, which contained brain, eye, and many other bovine/ovine tissues for years, 'IPLEX').

http://www.fda.gov/OHRMS/DOCKETS/AC/01/slides/3681s2_09.pdf

my plight with metabolife and there 'bovine complex' about risk factors of TSE in there product ;

Terry S. Singeltary Sr. wrote:

######## Bovine Spongiform Encephalopathy #########

1. Dietary Supplements: Review of Health-Related Call Records for > Users of Metabolife 356. GAO-03-494, March 31.

http://www.gao.gov/cgi-bin/getrpt?GAO-03-494

http://www.gao.gov/highlights/d03494high.pdf

-------- Original Message --------

Subject: METABOLIFE AND TSEs GAO-03-494 ''URGENT DATA''

Date: Thu, 01 May 2003 11:23:01 –0500

From: "Terry S. Singeltary Sr."

To: NelliganJ at gao.gov

The General Accounting Office (GAO) today released the following reports > and testimonies:

REPORTS ;

1. Dietary Supplements: Review of Health-Related Call Records for > Users of Metabolife 356. GAO-03-494, March 31.

http://www.gao.gov/cgi-bin/getrpt?GAO-03-494

http://www.gao.gov/highlights/d03494high.pdf

> GREETINGS GAO:

> i was suprised that i did not see any listing of bovine tissue in metabolife > on it's label. have they ceased using these desiccated tissues???

> > i see that the lable on this product METABOLIFE 356, > does not state that it has any tissues of desiccated bovine organs? i no the product use to, so i am curious if they have

> ceased the use of the tissues of cattle they _use_ to use (see below)???

> METABOLIFE 356

> BOVINE COMPLEX/GLANDULAR SYSTEM

> OVARIES, PROSTATE, SCROTUM AND ADRENAL

> USDA SOURCE CATTLE

> > i tried warning them years ago of this potential threat of CJD/TSEs;

> > From: Randy Smith

> To: "'flounder at wt.net'"

> Subject: Metabolife

> Date: Mon, 7 Dec 1998 14:21:35 –0800

> > Dear Sir,

> > We are looking at reformulation. I agree that slow virus diseases > present a problem in some areas of the world.

> > Our product uses healthy USDA inspected cattle for the glandular > extract.

> > If you have any links to more information on this subject I would like > to examine them.

> > Thank you for your interest and concern,

> > Dr. Smith

> ============

> > From: Randy Smith

> To: "'flounder at wt.net'"

> Subject: RE: [Fwd: Your submission to the Inquiry]

> Date: Wed, 9 Dec 1998 10:37:07 –0800

> > Terry,

> > Thank you for your note and the information links you forwarded to me. > I am new to Metabolife International, however hopefully as my role here > enlarges I well have a greater impact on formulation and product > development.

> > Metabolife International does believe in placing safety first. And I am > going to do my best to see that we continue to do so.

> > Sincerely,

> Dr. Smith

> ============

> -----Original Message-----

> From: Terry S. Singeltary Sr. [mailto:flounder at wt.net]

> Sent: Wednesday, December 09, 1998 5:49 PM

> To: rsmith at metabolife.com

> Subject: [Fwd: Your submission to the Inquiry]

> > Dr. Smith, I am truly impressed with you honesty, THANKS.....I am not > just spouting off about the potential dangers, here. THEY ARE REAL.....I > have forwarded an e-mail from the BSE Inquiry, in which I made a > statement about them........You might want to go to the site and read > through it........IT WILL TAKE A WHILE........ THINGS ARE HAPPENING HERE > SIR, THAT YOU ARE NOT AWARE OF, AND AS MOST PEOPLE ARE > NOT...............I JUST HOPE, THAT THE REFORMULATION YOU SPEAK OF, IS > IN FACT GOING TO TAKE PLACE.

> The Department of Health, here in the U.S., is also worried about the > potential dangers involved hear............Terry/MADSON

> ================================================== =======

> From: Randy Smith

> To: "'flounder at wt.net'"

> Subject: RE: [Fwd: MEDICINES "GREATER BSE RISK THAN BEEF"!!!!]

> Date: Fri, 18 Dec 1998 09:55:17 –0800

> Return-Receipt-To: Randy Smith

> > Thanks very much for the info. I appreciate all these articles I can > get. It does sound very familiar - just follow the green ($) trail.

> > -----Original Message-----

> From: Terry S. Singeltary Sr. [mailto:flounder at wt.net]

> Sent: Friday, December 18, 1998 5:15 PM

> To: rsmith at metabolife.com

> Subject: [Fwd: MEDICINES "GREATER BSE RISK THAN BEEF"!!!!]

> > Randy, thought you might be interested in this...............MADSON!!!!!1

> > snip...

> ===============================

> Sender: "Patricia Cantos"

> To: "Terry S Singeltary Sr. (E-mail)"

> Subject: Your submission to the Inquiry

> Date: Fri, 3 Jul 1998 10:10:05 +0100

> > 3 July 1998

> Mr Terry S Singeltary Sr.

> E-Mail: Flounder at wt.net

> Ref: E2979

> > Dear Mr Singeltary,

> > Thank you for your E-mail message of the 30th of June 1998 providing the > Inquiry with your further comments. Thank you for offering to provide the > Inquiry with any test results on the nutritional supplements your > mother was taking before she died.

> > As requested I am sending you our general Information Pack and a copy of the > Chairman's letter. Please contact me if your system cannot read the attachments.

> > Regarding your question, the Inquiry is looking into many aspects of the > scientific evidence on BSE and nvCJD. I would refer you to the > transcripts > of evidence we have already heard which are found on our internet site at >

http://www.bse.org.uk.

> > Could you please provide the Inquiry with a > copy of > the press article you refer to in your e-mail? If not an approximate date > for the article so that we can locate it? > In the meantime, thank you for you comments. Please do not hesitate to > contact me on 0171 261 8332 should you have any queries.

> > Yours sincerely

> Patricia Cantos

> Families Team Leader

> Attachments

> TSS

> ==============

> -------- Original Message --------

> Subject: re: METABOLIFE AND TSEs GAO-03-494 ''URGENT DATA''

> Date: Thu, 01 May 2003 16:04:35 –0400

> From: "Marcia G Crosse"

> To:

> CC: "Charles W Davenport" , "Carolyn Feis Korman" > , "Martin Gahart" >

> Mr. Singletary,

> > We were informed by representatives of Metabolife, Inc. that Metabolife

> 356 was reformulated to remove bovine complex as an ingredient in the

> product, approximately September 2001. We did not independently verify

> the contents of the product.

> > Sincerely,

> Marcia Crosse

> Acting Director

> Health CarePublic Health and Science Issues

> U.S. General Accounting Office

> 441 G Street, N.W.

> Washington, D.C. 20548

> ===================

> > -------- Original Message --------

> Subject: Re: METABOLIFE AND TSEs GAO-03-494 ''URGENT DATA''

> Date: Thu, 01 May 2003 15:48:52 –0500

> From: "Terry S. Singeltary Sr."

> To: Marcia G Crosse

> CC: Charles W Davenport , Carolyn Feis Korman > , Martin Gahart > References: >

> THANK YOU! > > MIRACLES DO HAPPEN! ;-) > > now all we need to do is; >

> snip......

> > one small step for man, one giant leap for mankind ;-)

> > however; >

> ''We did not independently verify the contents of the product''

> > ???

> TSS >

> ####### http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html ########

http://bseinquiry.blogspot.com/

http://www.fda.gov/ohrms/dockets/dailys/03/Mar03/031403/96N-0417-EC-2.htm

Could you get mad cow from a pill ? Some doctors say a class of pills that promise smarts, energy, and sexual vitality may cause mad-cow disease.

The government isn't worried. Should you be?

June 1, 2001

Health Magazine

by Susan Freinkel

http://www.organicconsumers.org/madcow/pill6101.cfm

GERMAN DER SPIEGEL MAGAZINEDie

BSE-Angst erreicht Amerika: Trotz strikter Auflagen gelangte in Texas verbotenes Tiermehl ins Rinderfutter - die Kontrollen der Aufsichtsbehörden sind lax.

http://www.spiegel.de/spiegel/0,1518,119306,00.html

From: Terry S. Singeltary Sr. [flounder@wt.net]

Sent: Tuesday, July 29, 2003 1:03 PM

To: fdadockets@oc.fda.govCc: ggraber@cvm.fda.gov; Linda.Grassie@fda.gov; BSE-L

Subject: Docket No. 2003N-0312 Animal Feed Safety System [TSS SUBMISSIONTO DOCKET 2003N-0312]

http://www.fda.gov/ohrms/dockets/dockets/03n0312/03N-0312_emc-000001.txt

Docket No. 03-080-1 -- USDA ISSUES PROPOSED RULE TO ALLOW LIVE ANIMAL IMPORTS FROM CANADA [takes a few minutes to load...TSS]

https://web01.aphis.usda.gov/BSEcom.nsf/BSEFrameset?OpenFrameSet&Frame=BottomFrame&Src=_25t156hb3dtmisrjjconjcc9n6ph6ccr66oqjgdpo74qm6e1l68qjcp9hc4o30dhgckq34phfc8rjgoj16orjep9ic8o66c9i64s3achl6pi68cpg60r38eb675i3ujrgcln48rr3elmmarjk4p0nat3f8pp62rb5cg0_

Docket Management Docket: 02N-0273 - Substances Prohibited From Use in

Animal Food or Feed; Animal Proteins Prohibited in Ruminant Feed

Comment Number: EC -10

Accepted - Volume 2

http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004be07.html

PART 2

http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004be09.html

Asante/Collinge et al, that BSE transmission to the 129-methionine

genotype can lead to an alternate phenotype that is indistinguishable

from type 2 PrPSc, the commonest _sporadic_ CJD;

http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.htm

Docket Management Docket: 96N-0417 - Current Good Manufacturing Practice in Manufacturing, Packing, or Holding Dietary Ingredients a Comment Number: EC -2 Accepted - Volume 7

http://www.fda.gov/ohrms/dockets/dailys/03/Mar03/031403/96N-0417-EC-2.htm

Dockets Entered On October 2, 2003 Table of Contents, Docket #,Title, 1978N-0301,

OTC External Analgesic Drug Products, ... EMC 7, Terry S. Singeltary Sr. Vol #: 1, ...

www.fda.gov/ohrms/dockets/dailys/03/oct03/100203/100203.htm

Daily Dockets Entered on 02/05/03

DOCKETS ENTERED on 2/5/03. ... EMC 4 Terry S. Singeltary Sr. Vol#: 2. ... Vol#: 1.

03N-0009 Federal Preemption of State & Local Medical Device Requireme. ...

www.fda.gov/ohrms/dockets/dailys/03/Feb03/020503/020503.htm

Docket Management

Docket: 02N-0370 - Neurological Devices; Classification of Human Dura Mater

Comment Number: EC -1

Accepted - Volume 1

http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004be11.html

http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004bdfe.html

http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004bdfc.html

Freas, William

Terry S. Singeltary Sr. [flounder@wt.net]

Monday, January 08,200l 3:03 PM

freas@CBS5055530.CBER.FDA.GOV

CJD/BSE (aka madcow) Human/Animal TSE’s--U.S.--Submission To Scientific Advisors and Consultants Staff January 2001 Meeting (short version)

http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_09.pdf

[PDF] Appendices to PL107-9 Inter-agency Working Group Final Report 1-1

Agent, Weapons of Mass Destruction Operations Unit Federal Bureau of those who provided comments in response to Docket No. ... Meager 8/18/01 Terry S. Singeltary Sr ...

www.aphis.usda.gov/lpa/pubs/pubs/PL107-9_Appen.pdf

Tuesday, February 8, 2011 U.S.A. 50 STATE BSE MAD COW CONFERENCE CALL Jan. 9, 2001

http://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html

http://tseac.blogspot.com/

Friday, December 16, 2011

OIG VULNERABILITIES IN FDA’S OVER SIGHT OF STATE FOOD FACILITY INSPECTIONS

FDA faulted over state inspections

http://fdafailedus.blogspot.com/2011/12/oig-vulnerabilities-in-fdas-over-sight.html

Sunday, November 13, 2011

California BSE mad cow beef recall, QFC, CJD, and dead stock downer livestock

http://transmissiblespongiformencephalopathy.blogspot.com/2011/11/california-bse-mad-cow-beef-recall-qfc.html

TSS

HIQA says vCJD blood filter not justified

2011-07-27T09:54:00.000-07:00

HIQA says vCJD blood filter not justified

[Posted: Wed 27/07/2011 http://www.irishhealth.com/]

The health safety body HIQA has ruled that the cost of introducing a new filter system to prevent vCJD infection through blood transfusions in Ireland cannot be justified.

Implementing such a filter system would initially cost €11 million per year a would potentially prevent two deaths over a 10-year period, HIQA said.

HIQA has carried out a cost-benefit analysis on introducing a new filtering technology with the potential to reduce further the low risk of transmitting vCJD through transfusions.

It said the prion filters appeared to be safe and would remove almost all the residual risk of vCJD from blood for transfusion.

However, the safety body concluded that the introduction of prion filters, either for all people getting transfusions or for limited sub-groups of patients, could not be justified when the costs and benefits were weighed up.

The origin of vCJD infection has been linked to the consumption of BSE-infected beef. It may also be transmitted via a blood transfusion from an infected donor who has not developed symptoms of the disease.

HIQA said in arriving at its recommendation about prion filters, it noted the 'substantial undertainty around the risk of vCJD in Ireland.'

The safety body said the level of undiagnosed cases of the disease in Ireland wa slikely to be low and that the corresponding risk of transfusion-transmitted infection was extremely low.

Dr Patricia Harrington of HIQA said the cost of implementing universal prion filtration was high compared to the likely benefits.

HIQA has submitted its recommendation on prion filters to the Minister for Health for consideration.

http://www.irishhealth.com/article.html?id=19541

SEE UPDATE ;

HIQA publishes assessment of vCJD filter technology

Date of publication: Wednesday, July 27, 2011

The Health Information and Quality Authority has today (27 July 2011) published the results of a health technology assessment (HTA) of prion filters, a new technology with the potential to further reduce the low risk of transmitting variant-Creutzfeldt-Jakob disease (vCJD) through a blood transfusion.

The Authority's assessment found that, based on current published evidence, the filters appear to be safe and would remove almost all the residual risk of vCJD transmission from red cell concentrates (blood from which most of the plasma and platelets has been removed). However, introduction of prion filters, either for all transfusion recipients or for limited sub-groups, was found to be not cost-effective when measured against traditional standards of cost-effectiveness.

vCJD is one of a group of rare, progressive and ultimately fatal degenerative disease of the nervous system, also known as prion diseases. They are thought to be caused by an abnormal form of a naturally occurring protein in the brain (the prion protein) that has been acquired through infection. The origin of vCJD has been linked to the consumption of BSE-infected beef. However, vCJD may also be transmitted via a blood transfusion from an infected donor who has not developed symptoms of the disease. Prion filtration is a new technology to be used in conjunction with existing blood safety strategies which aim to reduce the risk of vCJD transmission. The filters are designed to remove infectious prion protein from donated blood rendering the transfused blood safer to recipients.

The assessment notes the substantial uncertainty around the risk of vCJD transmission in Ireland. It notes the level of undiagnosed vCJD in Ireland is likely to be low and that the corresponding risk of transfusion-transmitted infection would be extremely low.

Dr Patricia Harrington, Head of Assessment with the Authority's Health Technology Assessment Directorate, said: "The Authority's HTA found that to filter red cell concentrates as proposed by the blood service would initially cost ?11 million per year. It was estimated that such a measure would, over a 10-year period, potentially prevent two deaths from vCJD."

The Board of the Authority has approved the HTA report and it has been submitted to the Minister for Health for his consideration.

Dr Harrington concluded: "The HTA has concluded that the cost of universal prion filtration is substantial. This financial cost, of further minimising what is most likely a low risk, is high compared to the likely benefits. In Ireland, the risk of acquiring vCJD from a transfusion of red cell concentrates in the absence of prion filtration is low. It notes therefore, that in the context of a finite healthcare budget, a decision to invest in prion filtration may have implications for the funding of other technologies and services in our health system."

For a full copy of the report please go to: www.hiqa.ie. You can also find us on Facebook and Twitter by searching 'HIQA'.

Further Information:

Marty Whelan Head of Communications and Stakeholder Engagement Directorate 01 8147481 / 086 2447623 or email mwhelan@hiqa.ie

Notes to the Editor:

The Health Information and Quality Authority is the statutory organisation in Ireland with a responsibility to carry out national health technology assessments (HTAs) and to develop guidelines for the conduct of HTAs across our health system. Whole donated blood is not generally re-transfused to recipients. It is first separated into its constituent parts, including units of red blood cells (referred to as units or red cell concentrates or RCC), units of platelets and units of plasma. The term 'blood transfusion' typically refers to a transfusion of RCC. Prion filters are new technologies that aim to reduce any residual infectious prion protein that may be present in donated blood, rendering it safer for transfusion. The IBTS has proposed adoption of prion filtration of RCC as an additional safety control measure. There is currently no commercially available human blood test to identify the presence of the abnormal prion protein associated with vCJD. The origin of vCJD has been linked to the consumption of BSE-infected beef. The incidence of BSE and vCJD peaked in the UK in 1992-1993 and 2000, respectively, declining since. There have been 170 deaths from vCJD in the UK and 4 deaths in Ireland, two of which are thought to have originated in the UK. vCJD may potentially be transmitted via a blood transfusion from an infected donor who may not have developed symptoms of the disease. Worldwide there have been five documented cases of transfusion-related vCJD infection, resulting in three deaths from clinical vCJD. It is predicted that prion filtration of all RCCs will initially cost ?11 million per annum and, over a 10-year time period, will prevent two deaths from vCJD and result in 19.4 discounted life years gained. The incremental cost-effectiveness ratio (ICER) of prion filtration is ?2.6 million per quality of life year gained (QALY). As a comparison, a HTA of population-based colorectal cancer screening carried out by the Authority found that this measure had an estimated ICER of ?1,696 per QALY compared to a policy of no screening. This screening was estimated to cost ?15 million per annum at full implementation, averting 160 cases of colorectal cancer and 270 deaths from colorectal cancer in year 10 of the screening programme.

http://www.hiqa.ie/press-release/2011-07-27-hiqa-publishes-assessment-vcjd-filter-technology-0

The Health Information and Quality Authority has today (27 July 2011) published the results of a health technology assessment (HTA) of prion filters, a new technology with the potential to further reduce the low risk of transmitting variant-Creutzfeldt-Jakob disease (vCJD) through a blood transfusion.

http://www.hiqa.ie/system/files/HTA-Prion-Filtration.pdf

TSS

tell that to the victims of the nvCJD, there families and friends. ...TSS

----- Original Message -----

From: Emery, Bryan (CBER)

To: 'Terry S. Singeltary Sr.'

Cc: Emery, Bryan (CBER)

Sent: Friday, July 22, 2011 9:47 AM

Subject: RE: TSEAC Meeting August 1, 2011 donor deferral Saudi Arabia vCJD risk blood and blood products

Hi Mr. Singeltary,

Your statement will be provided to the members and will be placed in the meetings display folder for the public to see. Come or attend via webcast.

thanks for your public participation

LCDR Bryan Emery

--------------------------------------------------------------------------------

From: Terry S. Singeltary Sr. [mailto:flounder9@verizon.net]

Sent: Wednesday, June 29, 2011 3:54 PM

To: Emery, Bryan (CBER)

Cc: Harvey, Rosanna; BSE-L@LISTS.AEGEE.ORG; CJD-L@LISTS.AEGEE.ORG; cjdvoice@yahoogroups.com; BLOODCJD@YAHOOGROUPS.COM; Advocatejr@aol.com; COTTWEST@SILCOM.COM; Dave Cavenaugh

Subject: TSEAC Meeting August 1, 2011 donor deferral Saudi Arabia vCJD risk blood and blood products

Meeting of the Transmissible Spongiform Encephalopathies Advisory Committee

Center Date Time Location

CBER August 1, 2011 9:00 a.m. - 4:30 p.m.

Hilton Washington DC/North, 620 Perry Pkwy., Gaithersburg, MD

Agenda

snip...end...TSS

Wednesday, June 29, 2011

TSEAC Meeting August 1, 2011 donor deferral Saudi Arabia vCJD risk blood and blood products

http://tseac.blogspot.com/2011/06/tseac-meeting-august-1-2011-donor.html

Bio.108: Transmission of Prion Disease by Multiple, Clinically-Relevant Blood Components Following a Single Blood Transfusion

Sandra McCutcheon,2,† Anthony R. Alejo Blanco,2 E. Fiona Houston,1 Christopher de Wolf,2 Boon Chin Tan,2 Nora Hunter,2 Valerie Hornsey,3 Ian R. MacGregor,3 Christopher V. Prowse,3 Marc Turner3, 4 and Jean C. Manson2

1The University of Glasgow; Glasgow, UK; 2The Roslin Institute and R(D)SVS, University of Edinburgh; Edinburgh, UK; 3Scottish National Blood Transfusion Service; Edinburgh, UK; 4The University of Edinburgh; Edinburgh, UK;†Presenting author; Email: sandra.mccutcheon@roslin.ed.ac.uk

Variant CJD (vCJD) is an incurable, infectious human disease, likely arising from the consumption of BSE-contaminated meat products. While the epidemic appears to be waning, there is much concern that vCJD infection may be amplified/prolonged in humans by the transfusion of contaminated blood products. Since 2004, several cases of transfusion-associated vCJD transmission have been reported. Using the most appropriate animal model available, in which the disease manifested resembles that of humans affected with vCJD, we examined which blood components used in human medicine are likely to pose the greatest risk of transmitting vCJD via transfusion. We collected two full units of blood from BSE-infected donor animals during the pre-clinical phase of infection. Using methods employed by transfusion services we prepared red cell concentrates, plasma and platelets units (including leucoreduced equivalents). Following transfusion to recipients, we showed that all components contain sufficient levels of infectivity to cause disease following only a single transfusion and also that leucoreduction did not prevent disease transmission. These data suggest that all blood components can act as potential vectors for prion transmission and highlight the importance of multiple control measures to minimize the risk of human to human transmission of vCJD by blood transfusion.

PPPM.18: Induction of Ab Amyloidogenesis In Vivo by Blood Transfusion

Rodrigo Morales,1,† Claudia Duran-Aniotz,1, 2 Akihiko Urayama,1 Lisbell Estrada,3 Diego Morales-Scheihing1, 2 and Claudio Soto1

1University of Texas Health Science Center at Houston; Houston, TX USA; 2Universidad de Los Andes; Santiago, Chile; 3Universidad Catolica de Chile; Santiago, Chile†Presenting author; Email: Rodrigo.MoralesLoyola@uth.tmc.edu

Alzheimer disease (AD) is the most common type of senile dementia. Disease manifestation is characterized by progressive impairment of memory and cognition which is triggered by synaptic dysfunction and neuronal loss. Compelling evidence suggests that misfolding and aggregation of Ab is a hallmark event in the disease pathogenesis. An important unanswered question related to AD involves its etiology since over 90% of the AD cases arise sporadically. Interestingly, misfolding and aggregation of proteins is the main feature of other diseases -termed Protein Misfolding Disorders (PMDs)] which include Transmissible Spongiform Encephalopathies (TSEs), among others. Convincing experimental evidences have shown that the only component of the infectious agent in TSEs is the misfolded form of the prion protein. Strikingly, the molecular mechanisms responsible for prion replication are very similar to the process of amyloid formation in all PMDs, suggesting that all these diseases have the inherent capability of being transmissible. Recent reports have shown that intra-cerebral and intra-peritoneal administration of brain homogenates containing Ab aggregates can accelerate the generation of senile plaques in mice models of AD. However, it remains to be demonstrated if this phenomenon can occur by more relevant routes of administration. The aim of this study was to assess whether AD pathogenesis can be induced intravenously, mimicking the know transmission of prion diseases through blood transfusion. For this purpose we used young tg2576 mice which were injected with blood obtained from 12 months old tg2576 mice (AD-blood) that contains a substantial quantity of cerebral Ab deposits. Mice were sacrificed at 250 days old, a time in which these animals scarcely develop Ab deposits. Interestingly, we observed that infusion of AD-blood induces substantial Ab accumulation in animals that without treatment or injected with wild type blood would barely have any detectable Ab lesion. Plaque deposition was mainly present in cortex and hippocampus, being more abundant in the former. In addition, we observed a decrease in memory in mice challenged with AD-blood. Other features such as brain inflammation and synaptic integrity were also measured. Importantly, similar results were obtained in a second and independent experiment performed in a double transgenic mouse model that develops AD plaques as early as 4 months old. Our results indicate that an AD-like pathogenesis can be induced by intravenous administration of AD-blood, presumably through induction of protein misfolding in a similar way as prion diseases. These findings may open a new avenue to understand the origin of sporadic AD and may provide new strategies for disease intervention and prevention.

http://www.prion2011.ca/files/PRION_2011_-_Posters_(May_5-11).pdf

Sir Paul Beresford: To ask the Secretary of State for Health whether his Department has conducted a cost analysis to compare the proposed use of prion filters and existing costs of risk reduction measures against the introduction of a variant Creutzfeldt-Jakob disease blood screening test which may replace or remove the need for these measures. [54816]

Anne Milton: There is currently no blood screening test that is proven to identify asymptomatic variant Creutzfeldt-Jakob disease infection. For this reason it is not possible to carry out a cost analysis to compare these measures.

Sir Paul Beresford: To ask the Secretary of State for Health pursuant to the contribution of the Minister of State for Health of 28 April 2011, Official Report, column 430 on variant Creutzfeldt-Jakob Disease (vCJD), what pathway his Department proposes to use to develop a prototype vCJD blood test in the event that no commercial company believes there is a business case to develop such a test. [54896]

Anne Milton: The Department is aware of a number of commercial organisations and academic institutions currently developing prototype blood tests for the abnormal prion protein associated with variant Creutzfeldt-Jakob Disease.

http://www.publications.parliament.uk/pa/cm201011/cmhansrd/cm110509/text/110509w0004.htm#11050951000005

SEE FULL TEXT ;

Wednesday, May 11, 2011

House of Commons CJD May 2011 UPDATE

Jason McCartney: To ask the Secretary of State for Health how many people died from variant Creutzfeldt-Jakob disease in England in each of the last five years. [54620] Anne Milton: The National Creutzfeldt-Jakob disease Research and Surveillance Unit (NCJDRSU) has provided the following information about deaths from variant Creutzfeldt-Jakob disease (vCJD). This information is available on NCJDRSU website at:

http://transmissiblespongiformencephalopathy.blogspot.com/2011/05/house-of-commons-cjd-may-2011-update.html

Tuesday, July 26, 2011

Irhad Rizvo Durakovic has lost his fight to nvCJD R.I.P.

http://creutzfeldt-jakob-disease.blogspot.com/2011/07/irhad-rizvo-durakovic-has-lost-his.html

http://transmissiblespongiformencephalopathy.blogspot.com/

http://vcjdtransfusion.blogspot.com/

http://creutzfeldt-jakob-disease.blogspot.com/

http://www.wellsphere.com/cjd-article/large-scale-immunohistochemical-examination-for-lymphoreticular-prion-protein-in-tonsil-specimens-collected-in-britain/1242673

TSS

Estimation of variant Creutzfeldt-Jakob disease infectivity titers in human blood

2011-06-03T19:54:00.000-07:00

Estimation of variant Creutzfeldt-Jakob disease infectivity titers in human blood

Luisa Gregori, Hong Yang, Steven AndersonArticle first published online: 3 JUN 2011

DOI: 10.1111/j.1537-2995.2011.03199.x

Gregori, L., Yang, H. and Anderson, S. (2011), Estimation of variant Creutzfeldt-Jakob disease infectivity titers in human blood. Transfusion, 51: no. doi: 10.1111/j.1537-2995.2011.03199.x

Author Information From the Division of Emerging and Transfusion-Transmitted Diseases, Office of Blood Research and Review, Rockville, Maryland; and the Office of Biostatistics and Epidemiology, Center for Biologics Evaluation and Research, Food and Drug Administration, Rockville, Maryland. *Correspondence: Luisa Gregori, DETTD/OBRR/CBER/FDA, 1401 Rockville Pike, FDA HFM-313, Rockville, MD 20852; e-mail: luisa.gregori@fda.hhs.gov.

This work was funded by the US Food and Drug Administration.

The findings and conclusions in this article have not been formally disseminated by the Food and Drug Administration and should not be construed to represent any Agency determination or policy.

Publication History Article first published online: 3 JUN 2011 Received for publication January 4, 2011; revision received April 7, 2011, and accepted April 10, 2011.

BACKGROUND: Blood of individuals with variant Creutzfeldt-Jakob disease (vCJD) is infectious but the titer is unknown. Current estimates of possible vCJD infectivity titers in blood have largely relied on an assumption that the titers of vCJD agent in human blood are likely to be similar to those in blood of rodents infected with model transmissible spongiform encephalopathy agents, assayed by intracerebral inoculations of rodents of the same species.

STUDY DESIGN AND METHODS: We analyzed published descriptions of experimental transfusion-transmitted (TT) bovine spongiform encephalopathy and scrapie in sheep and reports of TTvCJD in humans, applying statistical approaches to estimate the probable number of intravenous infectious doses (IDiv) per unit of transfused blood (IDiv/unit). For humans, IDiv/unit of nonleukoreduced red blood cells (NLR-RBCs) were estimated by two statistical models.

RESULTS: Sheep blood collected at or near onset of clinical illness contained a mean of 0.80 IDiv/unit. Estimates of infectivity in NLR-RBCs from donors incubating vCJD indicated a probable mean infectivity of 0.29 IDiv/unit (Model 1) and 0.75 IDiv/unit (Model 2). The analysis predicted a mean of 21 vCJD-infected recipients expected in a cohort transfused with vCJD-implicated NLR-RBCs in the United Kingdom.

CONCLUSION: Our analysis suggested that, while less than one IDiv is likely to be present in a given unit of NLR-RBCs collected from a donor incubating vCJD, there is a high probability of TT infection among recipients of vCJD-implicated blood components. The analysis supports continuing measures currently recommended to reduce the risk of TTvCJD.

http://onlinelibrary.wiley.com/doi/10.1111/j.1537-2995.2011.03199.x/abstract

FC5.1.1

Transmission Results in Squirrel Monkeys Inoculated with Human sCJD, vCJD, and GSS Blood Specimens: the Baxter Study

Brown, P1; Gibson, S2; Williams, L3; Ironside, J4; Will, R4; Kreil, T5; Abee, C3 1Fondation Alliance BioSecure, France; 2University of South Alabama, USA; 3University of Texas MD Anderson Cancer Center, USA; 4Western General Hospital, UK; 5Baxter BioSience, Austria

Background: Rodent and sheep models of Transmissible Spongiform Encephalopathy (TSE) have documented blood infectivity in both the pre-clinical and clinical phases of disease. Results in a (presumably more appropriate) non-human primate model have not been reported.

Objective: To determine if blood components (red cells, white cells, platelets, and plasma) from various forms of human TSE are infectious.

Methods: Blood components were inoculated intra-cerebrally (0.1 ml) and intravenously (0.5 ml) into squirrel monkeys from 2 patients with sporadic Creutzfeldt- Jakob disease (sCJD) and 3 patients with variant Creutzfeldt-Jakob disease (vCJD). Additional monkeys were inoculated with buffy coat or plasma samples from chimpanzees infected with either sCJD or Gerstmann-StrÃ¤ussler-Scheinker disease (GSS). Animals were monitored for a period of 5 years, and all dying or sacrificed animals had post-mortem neuropathological examinations and Western blots to determine the presence or absence of the misfolded prion protein (PrPTSE).

Results: No transmissions occurred in any of the animals inoculated with blood components from patients with sporadic or variant CJD. All donor chimpanzees (sCJD and GSS) became symptomatic within 6 weeks of their pre-clinical phase plasmapheresis, several months earlier than the expected onset of illness. One monkey inoculated with purified leukocytes from a pre-clinical GSS chimpanzee developed disease after 36 months.

Conclusion: No infectivity was found in small volumes of blood components from 4 patients with sporadic CJD and 3 patients with variant CJD. ***However, a single transmission from a chimpanzee-passaged strain of GSS shows that infectivity may be present in leukocytes, and the shock of general anaesthesia and plasmspheresis appears to have triggered the onset of illness in pre-clinical donor chimpanzees.

http://www.prion2010.org/bilder/prion_2010_program_latest_w_posters_4_.pdf?139&PHPSESSID=a30a38202cfec579000b77af81be3099

Saturday, September 5, 2009

TSEAC MEETING FEBRUARY 12, 2004 THE BAXTER STUDY GSS

snip...

But the first thing is our own study, and as I mentioned, it's a Baxter primate study, and those are the major participants. And the goal was twofold, and here is the first one: to see whether CJD, either sporadic or familial -- actually it turns out to be the familial CJD is incorrect. It really should be the Fukuoka strain of Gerstmann-Straussler-Scheinker disease. So it's really GSS instead of familial CJD -- when passaged through chimps into squirrel monkeys using purified blood components, very pure blood components.

So this addresses the question that was raised just recently about whether or not red cell infectivity that's been found in rodents is really in the red cells or is it contaminated.

We prepared these samples with exquisite care, and they are ultra-ultra-ultra purified. There's virtually no contamination of any of the components that we looked at ? platelets, red cells, plasma, white cells -- with any other component.

These are a sort of new set of slides, and what I've tried to do is make them less complicated and more clear, but I'm afraid I haven't included the build. So you'll just have to try and follow what I explain with this little red pointer.

There were three initial patients. Two of them had sporadic CJD. One of them had Gerstmann-Straussler-Scheinker syndrome. Brain tissue from each individual patient was inoculated intracerebrally into a pair of chimpanzees. All right?

From those chimps, either plasma or ultra purified -- in fact, everything is ultra-purified. I'll just talk about purified plasma, purified white cells -- were inoculated intracerebrally and intravenously to get the maximum amount of infective load into a pair of squirrel monkeys.

The same thing was done for each of these three sets. This monkey died from non-CJD causes at 34 months post inoculation.

Let me go back for a second. I didn't point out the fact that these were not sacrificed at this point. These chimpanzees were apheresed at 27 weeks when they were still asymptomatic. In this instance, we apheresed them terminally when they were symptomatic.

And before I forget, I want to mention just a little sidelight of this. Chimpanzees in our experience -- and I think we may be the only people that have ever inoculated chimpanzees, and that's no longer a possibility, so this was 20, 30 years ago -- the shortest incubation period of any chimpanzee that we have ever seen with direct intracerebral inoculation is 13 months.

So we chose 27 weeks, which is about seven months, and incidentally typically the incubation period is more like 16 or 18 months. The shortest was 13 months. We chose the 27th week, which is about six and a half months, thinking that this would be about halfway through the incubation period, which we wanted to check for the presence or absence of infectivity.

But within four weeks after the apheresis, which was conducted under general anesthesia for three or four hours apiece, every single one of the six chimpanzees became symptomatic. That is another experiment that I would love to conclude, perhaps because this is simply not heard of, and it very much smells like we triggered clinical illness. We didn't trigger the disease, but it certainly looks like we triggered symptomatic disease at a point that was much earlier than one would have possibly expected.

Maybe it will never be done because it would probably open the floodgates of litigation. There's no end of little things that you can find out from CJD patients after the fact. For example, the neighbor's dog comes over, barks at a patient, makes him fall down, and three weeks later he gets CJD. So you have a lawsuit against the neighbor.

I mean, this is not an unheard of matter, but I do think that physical stress in the form of anesthesia and four hours of whatever goes on with anesthesia, low blood pressure, sometimes a little hypoxemia looks like it's a bad thing.

So here we have the 31st week. All of the chimps are symptomatic, and here what we did was in order to make the most use of the fewest monkeys, which is always a problem in primate research, we took these same three patients and these six chimps. Only now we pooled these components; that is to say, we pooled the plasma from all six chimps. We pooled ultra-purified white cells from all six chimps because here we wanted to see whether or not we could distinguish a difference between intracerebral route of infection and intravenous route of infection.

With respect to platelets and red blood cells, we did not follow that. We inoculated both intracerebral and intravenously, as we had done earlier because nobody has any information on whether or not platelets and red cells are infectious, and so we wanted again to get the maximum.

This is an IV versus IC goal. This one, again, is just getting the maximum load in to see whether there is, in fact, any infectivity in pure platelets, in pure red cells.

And of all of the above, the only transmission of disease related to the inoculation was in a squirrel monkey that received pure leukocytes from the presymptomatic apheresis. So that goes some way to address the question as to whether or not it's a matter of contamination. To date the red cells have not been -- the monkeys that receive red cells have not been observed for more than a year because that was a later experiment.

So we still can't say about red cells, but we're about four and a half years down the road now, and we have a single transmission from purified leukocytes, nothing from plasma and nothing from platelets.

That was the first part of the experiment. The second part was undertaken with the cooperation of Bob Will and others supplying material to us. These were a couple of human, sporadic cases of CJD and three variant cases of CJD from which we obtained buffy coat and plasma separated in a normal way. That is, these are not purified components.

The two cases of sporadic CJD, the plasma was pooled from both patients. The buffy coat was pooled from both patients, and then inoculated intracerebrally and intravenously into three squirrel monkeys each. This is a non-CJD death five years after inoculation. The other animals are still alive.

For variant CJD we decided not to pool. It was more important to eliminate the possibility that there was just a little bit of infectivity in one patient that would have been diluted to extinction, if you like, by mixing them if it were to so occur with two patients, for example, who did not have infectivity. So each one of these was done individually, but the principle was the same: plasma and buffy coat for each patient was inoculated into either two or three squirrel monkeys. This is, again, a non-CJD related death.

In addition to that, we inoculated rain as a positive control from the two sporadic disease cases of human -- from the two human sporadic cases at ten to the minus one and ten to the minus three dilutions. We have done this many, many times in the past with other sporadic patients. So we knew what to expect, and we got exactly what we did expect, namely, after an incubation period not quite two years, all four monkeys developed disease at this dilution and at the minus three dilution, not a whole lot of difference between the two.

Now, these are the crucial monkeys because each one of these monkeys every three to four months was bled and the blood transfused into a new healthy monkey, but the same monkey all the time. So this monkey, for example, would have received in the course of 21 months about six different transfusions of blood from this monkey into this monkey, similarly with this pair, this pair, and this pair. So you can call these buddies. This is sort of the term that was used. These monkeys are still alive.

In the same way, the three human variant CJD specimens, brain, were inoculated into four monkeys, and again, each one of these monkeys has been repeatedly bled at three to four month intervals and that blood transfused into a squirrel monkey, the same one each time. Ideally we would love to have taken bleeding at three months and inoculated a monkey and then let him go, watch him, and then done the same thing at six months. It would have increased the number of monkeys eightfold and just unacceptably expensive. So we did the best we could.

That, again, is a non-CJD death, as is this.

This was of interest mainly to show that the titer of infectivity in brain from variant CJD is just about the same as it from sporadic. We didn't do a minus five and a minus seven in sporadic because we have an enormous experience already with sporadic disease in squirrel monkeys, and we know that this is exactly what happens. It disappears at about ten to the minus five. So the brain titer in monkeys receiving human vCJD is identical to the brain titer in monkeys that have been inoculated with sporadic CJD.

That's the experiment. All of the monkeys in aqua are still alive. They are approaching a five-year observation period, and I think the termination of this experiment will now need to be discussed very seriously in view of a probable six-year incubation period in the U.K. case. The original plan was to terminate the experiment after five years of observation with the understanding that ideally you would keep these animals for their entire life span, which is what we used to do when had unlimited space, money, and facilities. We can't do that anymore.

It's not cheap, but I think in view of the U.K. case, it will be very important to think very seriously about allowing at least these buddies and the buddies from the sporadic CJD to go on for several more years because although you might think that the U.K. case has made experimental work redundant, in point of fact, anything that bears on the risk of this disease in humans is worthwhile knowing, and one of the things we don't know is frequency of infection. We don't know whether this case in the U.K. is going to be unique and never happen again or whether all 13 or 14 patients have received blood components are ultimately going to die. Let's hope not.

The French primate study is primarily directed now by Corinne Lasmezas. As you know, the late Dominique Dromont was the original, originally initiated this work, and they have very active primate laboratory in France, and I'm only going to show two very simple slides to summarize what they did.

The first one is simply to show you the basis of their statement that the IV route of infection looks to be pretty efficient because we all know that the intracerebral route of infection is the most efficient, and if you look at this where they inoculated the same infective load either intracerebrally or intravenously, the incubation periods were not substantially different, which suggests but doesn't prove, but doesn't prove that the route of infection is pretty efficient.

Lower doses of brain material given IV did extend the incubation period and presumably it's because of the usual dose response phenomenon that you see in any infectious disease.

With a whopping dose of brain orally, the incubation period was even lower. Again, just one more example of inefficiency of the route of infection and the necessity to use more infective material to get transmissions.

And they also have blood inoculated IV which is on test, and the final slide or at least the penultimate slide shows you what they have on test and the time of observation, that taken human vCJD and like us inoculated buffy coat, they've also inoculated whole blood which we did not do.

So to a great extent their studies are complementary to ours and makes it all worthwhile.

We have about -- oh, I don't know -- a one to two-year lead time on the French, but they're still getting into pretty good observation periods. Here's three-plus years.

They have variant CJD adapted to the macaque. That is to say this one was passaged in macaque monkeys, the cynomolgus, and they did the same thing. Again, we're talking about a study here in which like ours there are no transmissions. I mean, we have that one transmission from leukocytes, and that's it.

Here is a BSE adapted to the macaque. Whole blood, and then they chose to inoculate leukodepleted whole blood, in both instances IV. Here they are out to five years without a transmission.

And then finally oral dosing of the macaque, which had been infected with -- which was infected with BSE, but a macaque passaged BSE, whole blood buffy coat and plasma, all by the IC route, and they're out to three years.

So with the single exception of the leukocyte transmission from our chimp that was inoculated with a sporadic case of CJD or -- excuse me -- with a GSS, Gerstmann-Straussler, in neither our study nor the French study, which are not yet completed have we yet seen a transmission.

And I will just close with a little cartoon that appeared in the Washington Post that I modified slightly lest you get too wound up with these questions of the risk from blood. This should be a "corrective."

(Laughter.)

DR. BROWN: Thanks.

Questions?

CHAIRPERSON PRIOLA: Yes. Any questions for Dr. Brown? Dr. Linden.

DR. LINDEN: I just want to make sure I understand your study design correctly. When you mention the monkeys that have the IV and IC inoculations, the individual monkeys had both or --

DR. BROWN: Yes, yes, yes. That's exactly right.

DR. LINDEN: So an individual monkey had both of those as opposed to some monkeys had one and some had the other?

DR. BROWN: Correct, correct. Where IC and IV are put down together was IC plus IV into a given monkey.

DR. LINDEN: Into a given monkey. Okay.

And the IC inoculations, where were those given?

DR. BROWN: Right parietal cortex, Southern Alabama.

(Laughter.)

DR. BROWN: Oh, it can't be that clear. Yeah, here, Pierluigi.

CHAIRPERSON PRIOLA: Dr. Epstein.

DR. BROWN: Pierluigi always damns me with feint praise. He always says that's a very interesting study, but. I'm waiting for that, Pierluigi.

I think Jay Epstein --

DR. GAMBETTI: I will say that there's an interesting study and will say, but I just --

(Laughter.)

DR. GAMBETTI: -- I just point of review. You talk about a point of information. You say that -- you mention GSS, I guess, and the what, Fukuowa (phonetic) --

DR. BROWN: Yes, Fukuoka 1.

DR. GAMBETTI: Fukuowa, and is that from the 102, if I remember correctly, of the --

DR. BROWN: Yes, that is correct.

DR. GAMBETTI: Because that is the only one that also --

DR. BROWN: No, it's not 102. It's 101. It's the standard. It's a classical GSS. Oh, excuse me. You're right. One, oh, two is classical GSS. It's been so long since I've done genetics. You're right.

DR. GAMBETTI: Because that is the only one I know, I think, that I can remember that has both the seven kv fragment that is characteristic of GSS, but also the PrPsc 2730. So in a sense, it can be stretching a little bit compared to the sporadic CJD.

DR. BROWN: Yeah, I think that's right. That's why I want to be sure that I made you aware on the very first slide that that was not accurate, that it truly was GSS.

There's a GSS strain that has been adapted to mice, and it's a hot strain, and therefore, it may not be translatable to sporadic disease, correct. All we can say for sure is that it is a human TSE, and it is not variant. I think that's about it.

DR. GAMBETTI: I agree, but this is also not perhaps the best --

DR. BROWN: No, it is not the best. We understand --

DR. GAMBETTI: -- of GSS either.

DR. BROWN: Yeah. If we had to do it over again, we'd look around for a -- well, I don't know. We'd probably do it the same way because we have two sporadics already on test they haven't transmitted, and so you can take your pick of what you want to pay attention to.

Jay?

DR. EPSTEIN: Yes, Paul. Could you just comment? If I understood you correctly, when you did the pooled apheresis plasma from the six chimps when they were symptomatic at 31 weeks, you also put leukocytes into squirrel monkeys in that case separately IV and IC, but in that instance you have not seen an infection come down in squirrel monkey, and the question is whether it's puzzling that you got transmission from the 27-week asymptomatic sampling, whereas you did not see transmission from the 31-week sampling in symptomatic animals.

DR. BROWN: Yes, I think there are two or three possible explanations, and I don't know if any of them are important. The pre-symptomatic animal was almost symptomatic as it turned out so that we were pretty close to the period at which symptoms would being, and whether you can, you know, make much money on saying one was incubation period and the other was symptomatic in this particular case because both bleedings were so close together. That's one possibility.

The other possibility is we're dealing with a very irregular phenomenon and you're not surprised at all by surprises, so to speak so that a single animal, you could see it almost anywhere.

The third is that we, in fact, did just what I suggested we didn't want to do for the preclinical, namely, by pooling we got under the threshold. See?

You can again take that for what it's worth. It is a possible explanation, and again, until we know what the levels of infectivity are and whether by pooling we get under the threshold of transmission, we simply cannot make pronouncements.

CHAIRPERSON PRIOLA: Dr. DeArmond.

DR. DeARMOND: Yeah, it was very interesting data, but the --

(Laughter.)

DR. BROWN: I just love it. Go ahead.

DR. DeARMOND: Two comments. The first one was that the GSS cases, as I remember from reading your publications -- I think Gibbs was involved with them -- when you transmitted the GSS into animals, into monkeys, perhaps I think it was chimps, the transmission was more typical of CJD rather than GSS. There were no amyloid plaques. It was vacuolar degeneration so that you may be transmitting a peculiar form, as I criticized once in Bali and then you jumped all over me about.

DR. BROWN: I may do it again.

DR. DeARMOND: Calling me a bigot and some other few things like that.

(Laughter.)

DR. BROWN: Surely not. I wouldn't have said that.

DR. DeARMOND: So there could be something strange about that particular --

DR. BROWN: Yeah. I think you and Pierluigi are on the same page here. This may be an unusual strain from a number of points of view.

DR. DeARMOND: The other question though has to do with species barrier because the data you're showing is kind of very reassuring to us that it's hard to transmit from blood, but the data from the sheep and from the hamsters and some of the work, I think, that has been done by others, that it's easy in some other animals to transmit, hamster to hamster, mouse to mouse.

Could you comment on the --

DR. BROWN: That's exactly why we went to primates. That's exactly it, because a primate is closer to a human than a mouse is, and that's just common sense.

And so to try and get a little closer to the human situation and not totally depend on rodents for transferrable data, that is why you would use a primate. Otherwise you wouldn't use them. They're too expensive and they cause grief to animal care study people and protocol makers and the whole thing.

Primate studies are a real pain.

DR. DeARMOND: But right now it's inconclusive and you need more time on it.

DR. BROWN: I believe that's true. I think if we cut it off at six years you could still say it was inconclusive, and cutting it off at all will be to some degree inconclusive, and that's just the way it is.

DR. DeARMOND: So what has to be done? Who do you have to convince, or who do we all have to convince to keep that going?

DR. BROWN: Thomas?

Without trying to be flip at all, the people that would be the first people to try to convince would be the funders of the original study. If that fails, and it might for purely practical reasons of finance, then we will have to look elsewhere because I really don't want to see those animals sacrificed, not those eight buddies. Those are crucial animals, and they don't cost a whole lot to maintain. You can maintain eight -- well, they cost a lot from my point of view, but 15 to $20,000 a year would keep them going year after year.

CHAIRPERSON PRIOLA: Dr. Johnson.

DR. JOHNSON: Yeah, Paul, I'm intrigued as you are by the shortening of the incubation period. Have you in all of the other years of handling these animals when they were transfused, when they were flown out to Louisiana at night -- a lot of the stressful things have happened to some of these chimps. Have you ever noticed that before or is this a new observation?

DR. BROWN: Brand new.

MR. JOHNSON: Brand new. Okay.

CHAIRPERSON PRIOLA: Bob, did you want to say something? Dr. Rohwer.

DR. ROHWER: The Frederick fire, wasn't that correlated with a lot of --

DR. BROWN: Not that I k now of, but you may --

DR. ROHWER: Well, that occurred shortly after I came to NIH, and what I remember is that there were a whole bunch of conversions that occurred within the few months following the fire. That was fire that occurred adjacent to the NINDS facility, but in order to protect it, they moved the monkeys out onto the tarmac because they weren't sure it wouldn't burn as well.

DR. BROWN: Well, if you're right, then it's not brand new, but I mean, I'm not sure how we'll ever know because if I call Carlton and ask him, I'm not sure but what I would trust the answer that he gives me, short of records.

You know, Carlot is a very enthusiastic person, and he might say, "Oh, yeah, my God, the whole floor died within three days," but I would want to verify that.

On the other hand, it may be verifiable. There possibly are records that are still extant.

DR. ROHWER: Actually I thought I heard the story from you.

(Laughter.)

DR. BROWN: You didn't because it's brand new for me. I mean, either that or I'm on the way

(Laughter.)

CHAIRPERSON PRIOLA: Dr. Bracey.

DR. BRACEY: I was wondering if some of the variability in terms of the intravenous infection route may be related to intraspecies barriers, that is, the genetic differences, the way the cells, the white leukocytes are processed, whether or not microchimerism is established, et cetera.

DR. BROWN: I don't think that processing is at fault, but the question, the point that you raise is a very good one, and needless to say, we have material with which we can analyze genetically all of the animals, and should it turn out that we get, for example, -- I don't know -- a transmission in one variant monkey and no transmissions in another and a transmission in three sporadic monkeys, we will at that point genetically analyze every single animal that has been used in this study, but we wanted to wait until we could see what would be most useful to analyze.

but the material is there, and if need be, we'll do it.

CHAIRPERSON PRIOLA: Okay. Thank you very much, Dr. Brown.

I think we'll move on to the open public hearing section of the morning.

snip...

http://www.fda.gov/ohrms/dockets/ac/04/transcripts/4019t1.DOC

snip...

see full text ;

http://tseac.blogspot.com/2009/09/tseac-meeting-february-12-2004-baxter.html

Tuesday, May 17, 2011

blood supply Creutzfeldt-Jakob Disease 16 May 2011 : Column 61W Commons Hansard

Creutzfeldt-Jakob Disease

http://vcjdtransfusion.blogspot.com/2011/05/blood-supply-creutzfeldt-jakob-disease.html

http://vcjdtransfusion.blogspot.com/

Saturday, May 14, 2011

USA Blood products, collected from a donor who was at risk for vCJD, were distributed Nationally and Internationally MAY 11, 2011

http://vcjdtransfusion.blogspot.com/2011/05/usa-blood-products-collected-from-donor.html

Wednesday, February 2, 2011

Detection of prion infection in variant Creutzfeldt-Jakob disease: a blood-based assay

http://transmissiblespongiformencephalopathy.blogspot.com/2011/02/detection-of-prion-infection-in-variant.html

Monday, February 7, 2011

FDA's Currently-Recommended Policies to Reduce the Possible Risk of Transmission of CJD and vCJD by Blood and Blood Products 2011 ???

http://tseac.blogspot.com/2011/02/fdas-currently-recommended-policies-to.html

Thursday, August 12, 2010

USA Blood products, collected from a donor who was at risk for vCJD, were distributed July-August 2010

http://creutzfeldt-jakob-disease.blogspot.com/2010/08/usa-blood-products-collected-from-donor.html

Sunday, August 01, 2010

Blood product, collected from a donors possibly at increased risk for vCJD only, was distributed USA JULY 2010

http://vcjdtransfusion.blogspot.com/2010/08/blood-product-collected-from-donors.html

Tuesday, September 14, 2010

Transmissible Spongiform Encephalopathies Advisory Committee; Notice of Meeting October 28 and 29, 2010 (COMMENT SUBMISSION)

http://tseac.blogspot.com/2010/09/transmissible-spongiform_14.html

Sunday, May 10, 2009

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

TO : william.freas@fda.hhs.gov

May 8, 2009

Greetings again Dr. Freas, TSEAC et al,

I would kindly, once again, wish to comment at this meeting about the urgent actions that need to be taken asap, to the Meeting of the Transmissible Spongiform Encephalopathies Committee On June 12, 2009. Due to my disability from my neck injury, I will not be attending this meeting either, however I hope for my submission to be read and submitted. ...

IN reply to ;

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

From: Terry S. Singeltary Sr.

To: FREAS@CBER.FDA.GOV

Cc: william.freas@fda.hhs.gov ; rosanna.harvey@fda.hhs.gov

Sent: Friday, December 01, 2006 2:59 PM

Subject: Re: TSE advisory committee for the meeting December 15, 2006 [TSS SUBMISSION

snip...

ONE FINAL COMMENT PLEASE, (i know this is long Dr. Freas but please bear with me)

THE USA is in a most unique situation, one of unknown circumstances with human and animal TSE. THE USA has the most documented TSE in different species to date, with substrains growing in those species (BSE/BASE in cattle and CWD in deer and elk, there is evidence here with different strains), and we know that sheep scrapie has over 20 strains of the typical scrapie with atypical scrapie documented and also BSE is very likely to have passed to sheep. all of which have been rendered and fed back to animals for human and animal consumption, a frightening scenario. WE do not know the outcome, and to play with human life around the globe with the very likely TSE tainted blood from the USA, in my opinion is like playing Russian roulette, of long duration, with potential long and enduring consequences, of which once done, cannot be undone.

These are the facts as i have come to know through daily and extensive research of TSE over 9 years, since 12/14/97. I do not pretend to have all the answers, but i do know to continue to believe in the ukbsenvcjd only theory of transmission to humans of only this one strain from only this one TSE from only this one part of the globe, will only lead to further failures, and needless exposure to humans from all strains of TSE, and possibly many more needless deaths from TSE via a multitude of proven routes and sources via many studies with primates and rodents and other species. ...

Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518

snip... 48 pages...

http://www.regulations.gov/fdmspublic/ContentViewer?objectId=09000064801f3413&disposition=attachment&contentType=msw8

APHIS-2006-0041-0006 TSE advisory committee for the meeting December 15, 2006

(this starts out in part III, then part II, and part I and the beginning is at the bottom. ...tss)

http://www.regulations.gov/fdmspublic/ContentViewer?objectId=09000064801f3413&disposition=attachment&contentType=msw8

Owens, Julie

From: Terry S. Singeltary Sr. [flounder9@verizon.net]

Sent: Monday, July 24, 2006 1:09 PM

To: FSIS Regulations

Comments

Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)

Page 1 of 98

8/3/2006

Greetings FSIS,

I would kindly like to comment on the following ;

http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf

http://www.scribd.com/doc/1490709/USDA-200600111

[Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)

http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf

suppressed peer review of Harvard study October 31, 2002

http://www.fsis.usda.gov/oa/topics/BSE_Peer_Review.pdf

Harvard Risk Assessment of Bovine Spongiform Encephalopathy Update, October 31, 2005

INTRODUCTION

The United States Department of Agriculture’s Food Safety and Inspection Service (FSIS) held a public meeting on July 25, 2006 in Washington, D.C. to present findings from the Harvard Risk Assessment of Bovine Spongiform Encephalopathy Update, October 31, 2005 (report and model located on the FSIS website:

http://www.fsis.usda.gov/Science/Risk_Assessments/index.asp).

Comments on technical aspects of the risk assessment were then submitted to FSIS. Comments were received from Food and Water Watch, Food Animal Concerns Trust (FACT), Farm Sanctuary, R-CALF USA, Linda A Detwiler, and Terry S. Singeltary.

This document provides itemized replies to the public comments received on the 2005 updated Harvard BSE risk assessment. Please bear the following points in mind:

http://www.fsis.usda.gov/PDF/BSE_Risk_Assess_Response_Public_Comments.pdf

-----Original Message-----

From: Terry S. Singeltary Sr. [mailto:flounder@wt.net]

Sent: Tuesday, February 18, 2003 12:45 PM

To: Freas, William

Cc: Langford, Sheila

Subject: Re: re-vCJD/blood and meeting of Feb. 20, 2003

Greetings FDA,

Asante/Collinge et al, that BSE transmission to the 129-methionine

genotype can lead to an alternate phenotype that is indistinguishable

from type 2 PrPSc, the commonest _sporadic_ CJD;

http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.htm

PDF]Freas, William TSS SUBMISSION

File Format: PDF/Adobe Acrobat -

Page 1. J Freas, William From:

Sent: To:

Subject: Terry S. Singeltary Sr. [flounder@wt.net]

Monday, January 08, 200l 3:03 PM freas ...

http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_09.pdf

Tuesday, February 8, 2011

U.S.A. 50 STATE BSE MAD COW CONFERENCE CALL Jan. 9, 2001

http://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html

Monday, May 30, 2011

CEPs for gelatin and impact of the revised EU Note for Guidance on the TSE risk EMEA/410/01 Rev.3) will come into force in July 2011

http://transmissiblespongiformencephalopathy.blogspot.com/2011/05/ceps-for-gelatin-and-impact-of-revised.html

Friday, May 13,

2011 EFSA Joint Scientific Opinion on any possible epidemiological or molecular association between TSEs in animals and humans

http://transmissiblespongiformencephalopathy.blogspot.com/2011/05/efsa-joint-scientific-opinion-on-any.html

Tuesday, May 24, 2011 2:24 PM

O.I.E. Terrestrial Animal Health Standards Commission and prion (TSE) disease reporting 2011

http://transmissiblespongiformencephalopathy.blogspot.com/2011/05/oie-terrestrial-animal-health-standards.html

Topics in Current Chemistry, 2011, 1-28, DOI: 10.1007/128_2011_161

Atypical Prion Diseases in Humans and Animals

Michael A. Tranulis, Sylvie L. Benestad, Thierry Baron and Hans Kretzschmar

Abstract

Although prion diseases, such as Creutzfeldt–Jakob disease (CJD) in humans and scrapie in sheep, have long been recognized, our understanding of their epidemiology and pathogenesis is still in its early stages. Progress is hampered by the lengthy incubation periods and the lack of effective ways of monitoring and characterizing these agents. Protease-resistant conformers of the prion protein (PrP), known as the “scrapie form” (PrPSc), are used as disease markers, and for taxonomic purposes, in correlation with clinical, pathological, and genetic data. In humans, prion diseases can arise sporadically (sCJD) or genetically (gCJD and others), caused by mutations in the PrP-gene (PRNP), or as a foodborne infection, with the agent of bovine spongiform encephalopathy (BSE) causing variant CJD (vCJD). Person-to-person spread of human prion disease has only been known to occur following cannibalism (kuru disease in Papua New Guinea) or through medical or surgical treatment (iatrogenic CJD, iCJD). In contrast, scrapie in small ruminants and chronic wasting disease (CWD) in cervids behave as infectious diseases within these species. Recently, however, so-called atypical forms of prion diseases have been discovered in sheep (atypical/Nor98 scrapie) and in cattle, BSE-H and BSE-L. These maladies resemble sporadic or genetic human prion diseases and might be their animal equivalents. This hypothesis also raises the significant public health question of possible epidemiological links between these diseases and their counterparts in humans.

Keywords Animal - Atypical - Atypical/Nor98 scrapie - BSE-H - BSE-L - Human - Prion disease - Prion strain - Prion type

http://resources.metapress.com/pdf-preview.axd?code=f433r34h34ugg617&size=largest

Monday, May 23, 2011

Atypical Prion Diseases in Humans and Animals 2011

Top Curr Chem (2011)

DOI: 10.1007/128_2011_161

# Springer-Verlag Berlin Heidelberg 2011

http://bse-atypical.blogspot.com/2011/05/atypical-prion-diseases-in-humans-and.html

Wednesday, March 31, 2010

Atypical BSE in Cattle

To date the OIE/WAHO assumes that the human and animal health standards set out in the BSE chapter for classical BSE (C-Type) applies to all forms of BSE which include the H-type and L-type atypical forms. This assumption is scientifically not completely justified and accumulating evidence suggests that this may in fact not be the case. Molecular characterization and the spatial distribution pattern of histopathologic lesions and immunohistochemistry (IHC) signals are used to identify and characterize atypical BSE. Both the L-type and H-type atypical cases display significant differences in the conformation and spatial accumulation of the disease associated prion protein (PrPSc) in brains of afflicted cattle. Transmission studies in bovine transgenic and wild type mouse models support that the atypical BSE types might be unique strains because they have different incubation times and lesion profiles when compared to C-type BSE.

When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE. In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.

This study will contribute to a correct definition of specified risk material (SRM) in atypical BSE. The incumbent of this position will develop new and transfer existing, ultra-sensitive methods for the detection of atypical BSE in tissue of experimentally infected cattle.

http://www.prionetcanada.ca/detail.aspx?menu=5&dt=293380&app=93&cat1=387&tp=20&lk=no&cat2

Thursday, August 12, 2010

Seven main threats for the future linked to prions

First threat

The TSE road map defining the evolution of European policy for protection against prion diseases is based on a certain numbers of hypotheses some of which may turn out to be erroneous. In particular, a form of BSE (called atypical Bovine Spongiform Encephalopathy), recently identified by systematic testing in aged cattle without clinical signs, may be the origin of classical BSE and thus potentially constitute a reservoir, which may be impossible to eradicate if a sporadic origin is confirmed.

***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases.

Second threat

snip...

http://www.neuroprion.org/en/np-neuroprion.html

http://prionpathy.blogspot.com/2010/08/seven-main-threats-for-future-linked-to.html

http://prionpathy.blogspot.com/

Rural and Regional Affairs and Transport References Committee

The possible impacts and consequences for public health, trade and agriculture of the Government’s decision to relax import restrictions on beef Final report June 2010

2.65 At its hearing on 14 May 2010, the committee heard evidence from Dr Alan Fahey who has recently submitted a thesis on the clinical neuropsychiatric, epidemiological and diagnostic features of Creutzfeldt-Jakob disease.48 Dr Fahey told the committee of his concerns regarding the lengthy incubation period for transmissible spongiform encephalopathies, the inadequacy of current tests and the limited nature of our current understanding of this group of diseases.49

2.66 Dr Fahey also told the committee that in the last two years a link has been established between forms of atypical CJD and atypical BSE. Dr Fahey said that: They now believe that those atypical BSEs overseas are in fact causing sporadic Creutzfeldt-Jakob disease. They were not sure if it was due to mad sheep disease or a different form. If you look in the textbooks it looks like this is just arising by itself. But in my research I have a summary of a document which states that there has never been any proof that sporadic Creutzfeldt-Jakob disease has arisen de novo—has arisen of itself. There is no proof of that. The recent research is that in fact it is due to atypical forms of mad cow disease which have been found across Europe, have been found in America and have been found in Asia. These atypical forms of mad cow disease typically have even longer incubation periods than the classical mad cow disease.50

http://www.aph.gov.au/senate/committee/rrat_ctte/mad_cows/report/report.pdf

Wednesday, January 19, 2011

EFSA and ECDC review scientific evidence on possible links between TSEs in animals and humans Webnachricht 19 Januar 2011

http://transmissiblespongiformencephalopathy.blogspot.com/2011/01/efsa-and-ecdc-review-scientific.html

Tuesday, January 18, 2011

Agent strain variation in human prion disease: insights from a molecular and pathological review of the National Institutes of Health series of experimentally transmitted disease

http://transmissiblespongiformencephalopathy.blogspot.com/2011/01/agent-strain-variation-in-human-prion.html

something that disturbs me very much, iatrogenic prion TSE exposure and accumulation there from all of the above ???

Tuesday, March 29, 2011

TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY EXPOSURE SPREADING VIA HOSPITALS AND SURGICAL PROCEDURES AROUND THE GLOBE

http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/transmissible-spongiform-encephalopathy.html

Saturday, March 5, 2011

MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE RISE IN NORTH AMERICA

http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/mad-cow-atypical-cjd-prion-tse-cases.html

Tuesday, April 26, 2011

sporadic CJD RISING Text and figures of the latest annual report of the NCJDRSU covering the period 1990-2009 (published 11th March 2011)

http://creutzfeldt-jakob-disease.blogspot.com/2011/04/sporadic-cjd-rising-text-and-figures-of.html

PRION MAD COW UDPATE NORTH AMERICA 2011

Sunday, April 3, 2011

PRION 2011 NEWWORLD MONTREAL CANADA MAY 16 - 19

http://transmissiblespongiformencephalopathy.blogspot.com/2011/04/prion-2011-newworld-montreal-canada-may.html

Tuesday, April 5, 2011

Action Plan National Program 103 Animal Health 2012-2017 PRIONS AND TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY

Action Plan National Program 103 Animal Health 2012-2017

http://transmissiblespongiformencephalopathy.blogspot.com/2011/04/action-plan-national-program-103-animal.html

Greetings,

WITH more and more atypical Transmissible Spongiform Encephalopathy cases showing up in more and more species here in North America, and the enormous monumental amount of banned mad cow protein in commerce since the infamous partial and voluntary mad cow feed ban inked on paper, with tons and tons crossing back and forth between the USA, Canada, and Mexico, it just does not surprise me of all these "PENDING CLASSIFICATIONS" of human TSE in Canada, and the USA. UK c-BSE transmitted to humans became nvCJD. WE now have atypical strains of BSE in cattle. Mission Texas experiments long ago showed that transmitted USA sheep scrapie to USA bovine, produced a TSE much different than the UK typical c-BSE. SO why would human TSE in the USA look like UK human TSE ? The corruption is mind boggling. The UK saw a suspicious TSE in humans, and science linked it to cattle. North America is awash with human and animal TSE, CJD is rising in young and old, with the same pathology and same symptoms, and none of it is related to the other. isn't that nice. who, what, bestowed such miracles upon North America $

Archive Number 20100405.1091 Published Date 05-APR-2010

Subject PRO/AH/EDR> Prion disease update 1010 (04)

snip...

[Terry S. Singeltary Sr. has added the following comment:

"According to the World Health Organisation, the future public health threat of vCJD in the UK and Europe and potentially the rest of the world is of concern and currently unquantifiable. However, the possibility of a significant and geographically diverse vCJD epidemic occurring over the next few decades cannot be dismissed.

The key word here is diverse. What does diverse mean? If USA scrapie transmitted to USA bovine does not produce pathology as the UK c-BSE, then why would CJD from there look like UK vCJD?"

http://www.promedmail.org/pls/apex/f?p=2400:1001:568933508083034::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1000,82101

CANADA CJD UPDATE 2011

CJD Deaths Reported by CJDSS1, 1994-20112 As of January 31, 2011

3. Final classification of 49 cases from 2009, 2010, 2011 is pending.

snip...

http://www.phac-aspc.gc.ca/hcai-iamss/cjd-mcj/cjdss-ssmcj/pdf/stats_0111-eng.pdf

USA 2011

USA

National Prion Disease Pathology Surveillance Center

Cases Examined1

(November 1, 2010)

Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD

1996 & earlier 51 33 28 5 0 0

1997 114 68 59 9 0 0

1998 87 51 43 7 1 0

1999 121 73 65 8 0 0

2000 146 103 89 14 0 0

2001 209 119 109 10 0 0

2002 248 149 125 22 2 0

2003 274 176 137 39 0 0

2004 325 186 164 21 0 13

2005 344 194 157 36 1 0

2006 383 197 166 29 0 24

2007 377 214 187 27 0 0

2008 394 231 205 25 0 0

2009 425 258 215 43 0 0

2010 333 213 158 33 0 0

TOTAL 38315 22656 1907 328 4 3

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 was acquired in the United Kingdom in one case and in Saudi Arabia in the other case;

5 Includes 18 cases in which the diagnosis is pending, and 18 inconclusive cases;

6 Includes 23 (22 from 2010) cases with type determination pending in which the diagnosis of vCJD has been excluded.

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

Please notice where sporadic CJD cases in 1996 went from 28 cases, to 215 cases in 2009, the highest recorded year to date. sporadic CJD is on a steady rise, and has been since 1996.

I also urge you to again notice these disturbing factors in lines 5 and 6 ;

5 Includes 18 cases in which the diagnosis is pending, and 18 inconclusive cases;

6 Includes 23 (22 from 2010) cases with type determination pending in which the diagnosis of vCJD has been excluded.

========end=====tss=====2011

Monday, August 9, 2010

National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010)

(please watch and listen to the video and the scientist speaking about atypical BSE and sporadic CJD and listen to Professor Aguzzi)

http://prionunitusaupdate2008.blogspot.com/2010/08/national-prion-disease-pathology.html

DID EVERYONE THAT LOST A LOVED ONE FILL OUT THEIR CJD QUESIONNAIRE FROM THE CDC AND OR THE CJD FOUNDATION ???

Friday, November 30, 2007

CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION

http://cjdquestionnaire.blogspot.com/2007/11/cjd-questionnaire.html

http://cjdquestionnaire.blogspot.com/

The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.

http://www.cjdfoundation.org/fact.html

http://cjdusa.blogspot.com/

http://transmissiblespongiformencephalopathy.blogspot.com/

PLEASE REMEMBER ;

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.

snip.

http://web.archive.org/web/20010305222642/www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf

OR nvCJD. ......could look like anyone or all of the sporadic CJD's. ...TSS

Friday, April 15, 2011

PRION TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY PROJECTS, RESEARCH FUNDING, BSE VOLUNTARY TESTING UPDATE IN NORTH AMERICA 2011

http://prionunitusaupdate2008.blogspot.com/2011/04/prion-transmissible-spongiform.html

Wednesday, March 9, 2011

27 U.S. Senators want to force feed Japan Highly Potential North America Mad Cow Beef TSE PRION CJD March 8, 2011

President Barack Obama The White House

1600 Pennsylvania Avenue, W Washington, DC 20500

Dear President Obama:

http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/27-us-senators-want-to-force-feed-japan.html

Thursday, May 26, 2011

Travel History, Hunting, and Venison Consumption Related to Prion Disease Exposure, 2006-2007 FoodNet Population Survey

Journal of the American Dietetic Association Volume 111, Issue 6 , Pages 858-863, June 2011.

http://transmissiblespongiformencephalopathy.blogspot.com/2011/05/travel-history-hunting-and-venison.html

WHAT about funding in the USA for 2010 on prion disease. a big fat zero dollars $ 0.0 $

PRION TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY RESEARCH FUNDING U.S.A.

COMPARE TO USA PRION FUNDING 2011

"which includes the ___elimination___ of Prion activities ($5,473,000),"

All Other Emerging and Zoonotic Infectious Diseases CDC‘s FY 2012 request of $52,658,000 for all other emerging and zoonotic infectious disease activities is a decrease of $13,607,000 below the FY 2010 level, which includes the elimination of Prion activities ($5,473,000), a reduction for other cross-cutting infectious disease activities, and administrative savings. These funds support a range of critical emerging and zoonotic infectious disease programs such Lyme Disease, Chronic Fatigue Syndrome, and Special Pathogens, as well as other activities described below.

http://www.cdc.gov/fmo/topic/Budget%20Information/appropriations_budget_form_pdf/FY2012_CDC_CJ_Final.pdf

Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518

blood supply Creutzfeldt-Jakob Disease 16 May 2011 : Column 61W Commons Hansard

2011-05-17T11:49:00.000-07:00

Creutzfeldt-Jakob Disease

Jason McCartney: To ask the Secretary of State for Health what steps his Department is taking to protect the blood supply from variant Creutzfeldt-Jakob disease. [55158]

Anne Milton: The following precautionary measures have been implemented to protect the blood supply and products made by fractionating plasma:

From December 1997, blood components, plasma products or tissues obtained from any individual who later develops variant Creutzfeldt-Jakob disease (vCJD), have been withdrawn/recalled to prevent their use;

From October 1999, white blood cells (which may carry a risk of transmitting vCJD) have been reduced in all blood used for transfusion, a process known as leucodepletion or leucoreduction;

Following the report of the first possible case of transmission of vCJD by blood transfusion in December 2003, individuals who had themselves received a transfusion of blood components since January 1980 were excluded from donating blood. This took effect from April 2004, and in July 2004, this exclusion criterion for blood donation was extended to include two further groups, who had received transfusions of blood components since 1980:

Previously transfused platelet donors,

Donors who were unsure if they had previously had a blood transfusion. This now applies to donors who have been transfused anywhere in the world;

Since 1999, plasma for the manufacture of fractionated plasma products, such as clotting factors, has been obtained from non-United Kingdom sources;

16 May 2011 : Column 61W

Since 2004, fresh frozen plasma for treating babies and young children born on or after 1 January 1996 has been obtained from non-UK sources, and from July 2005 its use was extended to all children up to the age of 16; and

Cryoprecipitate, for use in the under-16s, is now produced from imported methylene blue treated-plasma.

All of these measures were recommended or endorsed by the Advisory Committee on the Safety of Blood, Tissues and Organs (which first met in January 2008) or its predecessor committees.

Additionally, considerable effort is being extended to promote appropriate use of blood throughout the national health service, to target blood use to where it is clinically essential, and for bleeding disorder patients (such as haemophiliacs) UK plasma has not been used for the manufacture of clotting factors since 1999 and recombinant clotting factors are now available for all patients for whom they are suitable.

Jason McCartney: To ask the Secretary of State for Health (1) what estimate he has made of the number of people who are considered to be at risk of contracting variant Creuztfeldt-Jakob disease; [55157]

(2) what estimate his Department has made of the number of people carrying the infective prions that cause variant Creuztfeldt-Jakob disease; and what proportion of such people he estimates are registered as blood donors. [55160]

Anne Milton: A study published in 2004 of stored appendix and tonsil tissue samples found abnormal prion protein in three appendices out of 12,674 samples. This suggested a population prevalence of about one in 4,000, though with very wide confidence interval of between one in 1,400 and one in 20,000. A further study of over 96,000 tonsils pairs is nearing completion, and a study of 30,000 appendix samples is due to be completed in 2012. Prevalence estimates are kept under active review by the relevant expert scientific advisory committees, who will review all the evidence on the completion of these ongoing studies.

In England about 4.4% of the population are blood donors, and the prevalence of potentially infective blood donors remains unknown. Not all of the individuals in the 2004 published study would be of an age eligible to donate blood, nor is it clear whether presence of abnormal prion protein in tissues such as the appendix or tonsils indicates that the blood of such a donor would transmit variant Creutzfeldt-Jakob disease. All precautionary measures are assessed in the context of the fundamental uncertainties about prevalence.

Jason McCartney: To ask the Secretary of State for Health what estimate his Department has made of the number of people diagnosed with variant Creuztfeldt-Jakob disease. [55159]

Anne Milton: Since 1995 175 patients have been identified with definite or probable variant Creutzfeldt-Jakob disease (vCJD) in the United Kingdom.

The National Creutzfeldt-Jakob disease Research and Surveillance Unit publishes monthly figures on all cases of human prion disease, including vCJD, on the website at:

www.cjd.ed.ac.uk/

16 May 2011 : Column 62W

Dental Services

Andrew Rosindell: To ask the Secretary of State for Health how many dentists' surgeries he has visited in an official capacity in the last 12 months. [55229]

Mr Simon Burns: In the last 12 months my right hon. Friend the Secretary of State for Health (Mr Lansley) has visited one community dental service in an official capacity. My noble Friend the Parliamentary Under-Secretary of State (Earl Howe) leads on dentistry within the ministerial team and has visited two dental practices in the last 12 months, one in Worcester and one in London. He has also visited dentists providing oral health promotion services for children provided in a children's centre in Preston.

http://www.publications.parliament.uk/pa/cm201011/cmhansrd/cm110516/text/110516w0003.htm#11051625000037

CREUTZFELDT JAKOB DISEASE

Monday, May 16, 2011

Does Poor Dental Health Have a Role in the Emergence of Variant Creutzfeldt Jakob Disease in the United Kingdom?

http://transmissiblespongiformencephalopathy.blogspot.com/2011/05/does-poor-dental-health-have-role-in.html

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

Since 2004, fresh frozen plasma for treating babies and young children born on or after 1 January 1996 has been obtained from non-UK sources, and from July 2005 its use was extended to all children up to the age of 16; and Cryoprecipitate, for use in the under-16s, is now produced from imported methylene blue treated-plasma.

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

Saturday, May 14, 2011

USA Blood products, collected from a donor who was at risk for vCJD, were distributed Nationally and Internationally MAY 11, 2011

http://vcjdtransfusion.blogspot.com/2011/05/usa-blood-products-collected-from-donor.html

Tuesday, March 29, 2011

TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY EXPOSURE SPREADING VIA HOSPITALS AND SURGICAL PROCEDURES AROUND THE GLOBE

http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/transmissible-spongiform-encephalopathy.html

Tuesday, April 26, 2011

sporadic CJD RISING Text and figures of the latest annual report of the NCJDRSU covering the period 1990-2009 (published 11th March 2011)

http://creutzfeldt-jakob-disease.blogspot.com/2011/04/sporadic-cjd-rising-text-and-figures-of.html

Friday, May 13,

2011 EFSA Joint Scientific Opinion on any possible epidemiological or molecular association between TSEs in animals and humans

http://transmissiblespongiformencephalopathy.blogspot.com/2011/05/efsa-joint-scientific-opinion-on-any.html

Sunday, May 01, 2011

STUDY OF ATYPICAL BSE 2010 Annual Report May 2011

http://bse-atypical.blogspot.com/2011/05/study-of-atypical-bse-2010-annual.html

Saturday, March 5, 2011

MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE RISE IN NORTH AMERICA

http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/mad-cow-atypical-cjd-prion-tse-cases.html

TSS

USA Blood products, collected from a donor who was at risk for vCJD, were distributed Nationally and Internationally MAY 11, 2011

2011-05-14T11:07:00.000-07:00

PRODUCT

1) Red Blood Cells. Recall # B-1215-11;

2) Plasma Frozen. Recall # B-1216-11

CODE

1) and 2) Unit: W071910008550

RECALLING FIRM/MANUFACTURER

So Ca Permamente Med Group Blood Donor Center, Los Angeles, CA, by facsimile and letter on February 01, 2011. Firm initiated recall is complete.

REASON

Blood products, collected from a donor who was at risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.

VOLUME OF PRODUCT IN COMMERCE

2 units

DISTRIBUTION

CA

___________________________________

PRODUCT

1) Platelets Pheresis Leukocytes Reduced. Recall # B-1235-11;

2) Red Blood Cells (Apheresis). Recall # B-1236-11;

3) Platelets Pheresis Leukocytes Reduced Irradiated. Recall # B-1237-11;

4) Fresh Frozen Plasma (Apheresis). Recall # B-1238-11

CODE

1) Units: FM16083, FM16085, FM23853, FM16158, FM16180, FM25344 (part 1), FM25344 (part 2), FM25353, FM16234, FM16235 (part 1), FM16235 (part 2), FM16235 (part 3);

2) Units: FM16085, FM16180;

3) Unit: FM16085;

4) Unit: FM16180

RECALLING FIRM/MANUFACTURER

Sacramento Medical Foundation, Sacramento, CA, by telephone on August 19, 2004, October 25, 2004 and October 28, 2004. Firm initiated recall is complete.

REASON

Blood products, collected from unsuitable donors based on risk factors for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.

VOLUME OF PRODUCT IN COMMERCE

16 units

DISTRIBUTION

CA, TN, MA, AZ

___________________________________

PRODUCT

Source Plasma. Recall # B-1275-11

CODE

Units: 07JWIE6717; 07JWIE6226; 07JWIE5416; 07JWIE4636; 07JWIE4074; 07JWIE2386; 07JWIE1783; 07JWID7602; 07JWID7072; 07JWID2830; 07JWID2280; 07JWID1683; 07JWID1149; 07JWID0420; 07JWIC9864; 07JWIC9102; 07JWIC8527; 07JWIC7817 06JWID8466; 06JWID8869; 06JWIE0195; 06JWIE0828 ; 06JWIE1608; 06JWIE1927; 06JWIE2619; 06JWIE2943; 06JWIE3592 ; 06JWIE4190; 06JWIE4779; 06JWIE5392; 06JWIE5762; 06JWIE6218; 06JWIE6577; 07JWIA0385; 07JWIA0858; 06JWIA1602; 07JWIA2048; 07JWIA3215; 07JWIA3495; 07JWIA4652; 07WIA5096; 07JWIB1404 ; 07JWIB1837; 07JWIB2611; 07JWIB3529; 07JWIB3976; 07JWIC4846; 07JWIC5307; 07JWIC5970; 07JWIC6517; 07JWIC7252; 07JWIB5981; 07JWIB7770 07JWIB8514; 07JWIB9064; 07JWIC0083; 07JWIC0561; 07JWIC1550; 07JWIC2286; 07JWIC2808; 07JWIC3548; 07JWIC4104

RECALLING FIRM/MANUFACTURER

Recalling Firm: BioLife Plasma Services LP, Deerfield, IL, by facsimile on December 7, 2007.
Manufacturer: BioLife Plasma Services, L.P., Janesville, WI. Firm initiated recall is complete.

REASON

Blood products, collected from a donor who was at risk for variant Creutzfeldt-Jakob disease (vCJD), were distributed.

VOLUME OF PRODUCT IN COMMERCE

62 units

DISTRIBUTION

Austria, CA

___________________________________

END OF ENFORCEMENT REPORT FOR MAY 11, 2011

#

http://www.fda.gov/Safety/Recalls/EnforcementReports/ucm255019.htm

Friday, May 13,

2011 EFSA Joint Scientific Opinion on any possible epidemiological or molecular association between TSEs in animals and humans

http://transmissiblespongiformencephalopathy.blogspot.com/2011/05/efsa-joint-scientific-opinion-on-any.html

Tuesday, May 3, 2011

PRION, TSE, typical, atypical BSE, aka mad cow disease, spray dried blood, feed, and a recipe for disaster

http://transmissiblespongiformencephalopathy.blogspot.com/2011/05/prion-tse-typical-atypical-bse-aka-mad.html

Sunday, May 01, 2011

STUDY OF ATYPICAL BSE 2010 Annual Report May 2011

http://bse-atypical.blogspot.com/2011/05/study-of-atypical-bse-2010-annual.html

Sunday, May 1, 2011

W.H.O. T.S.E. PRION Blood products and related biologicals May 2011

http://transmissiblespongiformencephalopathy.blogspot.com/2011/05/who-tse-prion-blood-products-and.html

Saturday, April 30, 2011

Blood product, collected from a donor who was at risk for variant Creutzfeldt-Jakob disease (vCJD), was distributed APRIL 27, 2011

http://vcjdtransfusion.blogspot.com/2011/04/blood-product-collected-from-donor-who.html

Saturday, March 5, 2011

MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE RISE IN NORTH AMERICA

http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/mad-cow-atypical-cjd-prion-tse-cases.html

TSS

Blood product, collected from a donor who was at risk for variant Creutzfeldt-Jakob disease (vCJD), was distributed APRIL 27, 2011

2011-04-30T20:00:00.000-07:00

Blood product, collected from a donor who was at risk for variant Creutzfeldt-Jakob disease (vCJD), was distributed APRIL 27, 2011

PRODUCT Recovered Plasma. Recall # B-0017-11 CODE Unit: W086510003037 RECALLING FIRM/MANUFACTURER Central Blood Bank, Pittsburgh, PA, by electronic mail on January 9, 2011. Firm initiated recall is complete. REASON Blood product, collected from a donor who was at risk for variant Creutzfeldt-Jakob Disease (vCJD), was distributed. VOLUME OF PRODUCT IN COMMERCE 1 unit DISTRIBUTION IN

___________________________________

PRODUCT Source Plasma. Recall # B-0781-11 CODE Unit:07LWIG5594 RECALLING FIRM/MANUFACTURER Recalling Firm: BioLife Plasma Services LP, Deerfield, IL, by fax on January 17, 2008. Manufacturer: BioLife Plasma Services, L.P., Onalaska, WI. Firm initiated recall is complete. REASON Blood product, collected from a donor who did not respond to questions regarding increased risk for vCJD, was distributed. VOLUME OF PRODUCT IN COMMERCE 1 unit DISTRIBUTION Austria

___________________________________

PRODUCT Source Plasma. Recall # B-1212-11 CODE Unit:08LWIB5812 RECALLING FIRM/MANUFACTURER Recalling Firm: BioLife Plasma Services LP, Deerfield, IL, by fax, on June 3, 2008. Manufacturer: BioLife Plasma Services, L.P., Onalaska, WI. Firm initiated recall is complete. REASON Blood product, collected from a donor who did not respond to questions regarding increased risk for vCJD, was distributed. VOLUME OF PRODUCT IN COMMERCE 1 unit DISTRIBUTION Austria

___________________________________

PRODUCT Recovered Plasma. Recall # B-1229-11 CODE Unit: W036510029868 RECALLING FIRM/MANUFACTURER LifeShare Blood Centers, Monroe, LA, by electronic notification on March 8, 2011. Firm initiated recall is complete. REASON Blood product, collected from a donor who was at risk for variant Creutzfeldt-Jakob disease (vCJD), was distributed. VOLUME OF PRODUCT IN COMMERCE 1 unit DISTRIBUTION Switzerland

___________________________________

END OF ENFORCEMENT REPORT FOR APRIL 27, 2011

#

http://www.fda.gov/Safety/Recalls/EnforcementReports/ucm252912.htm

Friday, September 24, 2010

USA Blood products, collected from a donor who was at risk for vCJD, were distributed SEPTEMBER 2010

http://vcjdtransfusion.blogspot.com/2010/09/usa-blood-products-collected-from-donor.html

Thursday, August 12, 2010

USA Blood products, collected from a donor who was at risk for vCJD, were distributed July-August 2010

http://creutzfeldt-jakob-disease.blogspot.com/2010/08/usa-blood-products-collected-from-donor.html

Thursday, May 27, 2010

Guidance for Industry: Revised Preventive Measures to Reduce Possible Risk of Transmission of CJD and vCJD by blood and blood products; Availability

[Federal Register: May 27, 2010 (Volume 75, Number 102)] [Notices] [Page 29768-29769] From the Federal Register Online via GPO Access [wais.access.gpo.gov] [DOCID:fr27my10-66]

http://vcjdtransfusion.blogspot.com/2010/05/guidance-for-industry-revised.html

Monday, February 7, 2011

FDA's Currently-Recommended Policies to Reduce the Possible Risk of Transmission of CJD and vCJD by Blood and Blood Products 2011 ???

http://tseac.blogspot.com/2011/02/fdas-currently-recommended-policies-to.html

Sunday, January 30, 2011

Vaccines and Transmissible Spongiform Encephalopathy the Prion Disease, what if ?

COMMERCIAL IN CONFIDENCE

http://transmissiblespongiformencephalopathy.blogspot.com/2011/01/vaccines-and-transmissible-spongiform.html

Saturday, February 26, 2011

Supreme Court Protects Vaccine Manufacturers, Not Injured Children there from Bruesewitz vs Wyeth

http://vcjdtransfusion.blogspot.com/2011/02/supreme-court-protects-vaccine.html

Wednesday, January 19, 2011

EFSA and ECDC review scientific evidence on possible links between TSEs in animals and humans Webnachricht 19 Januar 2011

http://transmissiblespongiformencephalopathy.blogspot.com/2011/01/efsa-and-ecdc-review-scientific.html

Tuesday, January 18, 2011

Agent strain variation in human prion disease: insights from a molecular and pathological review of the National Institutes of Health series of experimentally transmitted disease

http://transmissiblespongiformencephalopathy.blogspot.com/2011/01/agent-strain-variation-in-human-prion.html

Tuesday, December 14, 2010

Infection control of CJD, vCJD and other human prion diseases in healthcare and community settings part 4, Annex A1, Annex J, UPDATE DECEMBER 2010

http://creutzfeldt-jakob-disease.blogspot.com/2010/12/infection-control-of-cjd-vcjd-and-other.html

Wednesday, September 08, 2010

Emerging Infectious Diseases: CJD, BSE, SCRAPIE, CWD, PRION, TSE Evaluation to Implementation for Transfusion and Transplantation September 2010

http://vcjdtransfusion.blogspot.com/2010/09/emerging-infectious-diseases-cjd-bse.html

Saturday, January 20, 2007

Fourth case of transfusion-associated vCJD infection in the United Kingdom

http://vcjdtransfusion.blogspot.com/2007_01_01_archive.html

http://vcjdtransfusion.blogspot.com/2010/07/cjd-uk-parliament-12-july-2010-column.html

http://vcjdtransfusion.blogspot.com/

The EMBO Journal (2002) 21, 6358 - 6366 doi:10.1093/emboj/cdf653

BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein

Emmanuel A. Asante1, Jacqueline M. Linehan1, Melanie Desbruslais1, Susan Joiner1, Ian Gowland1, Andrew L. Wood1, Julie Welch1, Andrew F. Hill1, Sarah E. Lloyd1, Jonathan D.F. Wadsworth1 and John Collinge1

1.MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College, Queen Square, London WC1N 3BG, UK Correspondence to:

John Collinge, E-mail: j.collinge@prion.ucl.ac.uk

Received 1 August 2002; Accepted 17 October 2002; Revised 24 September 2002

--------------------------------------------------------------------------------

Abstract

Variant Creutzfeldt–Jakob disease (vCJD) has been recognized to date only in individuals homozygous for methionine at PRNP codon 129. Here we show that transgenic mice expressing human PrP methionine 129, inoculated with either bovine spongiform encephalopathy (BSE) or variant CJD prions, may develop the neuropathological and molecular phenotype of vCJD, consistent with these diseases being caused by the same prion strain. Surprisingly, however, BSE transmission to these transgenic mice, in addition to producing a vCJD-like phenotype, can also result in a distinct molecular phenotype that is indistinguishable from that of sporadic CJD with PrPSc type 2. These data suggest that more than one BSE-derived prion strain might infect humans; it is therefore possible that some patients with a phenotype consistent with sporadic CJD may have a disease arising from BSE exposure.

Keywords:BSE, Creutzfeldt–Jakob disease, prion, transgenic

http://www.nature.com/emboj/journal/v21/n23/abs/7594869a.html

Wednesday, March 31, 2010

Atypical BSE in Cattle

To date the OIE/WAHO assumes that the human and animal health standards set out in the BSE chapter for classical BSE (C-Type) applies to all forms of BSE which include the H-type and L-type atypical forms. This assumption is scientifically not completely justified and accumulating evidence suggests that this may in fact not be the case. Molecular characterization and the spatial distribution pattern of histopathologic lesions and immunohistochemistry (IHC) signals are used to identify and characterize atypical BSE. Both the L-type and H-type atypical cases display significant differences in the conformation and spatial accumulation of the disease associated prion protein (PrPSc) in brains of afflicted cattle. Transmission studies in bovine transgenic and wild type mouse models support that the atypical BSE types might be unique strains because they have different incubation times and lesion profiles when compared to C-type BSE. When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE. In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.

This study will contribute to a correct definition of specified risk material (SRM) in atypical BSE. The incumbent of this position will develop new and transfer existing, ultra-sensitive methods for the detection of atypical BSE in tissue of experimentally infected cattle.

http://www.prionetcanada.ca/detail.aspx?menu=5&dt=293380&app=93&cat1=387&tp=20&lk=no&cat2

U.S.A. HIDING MAD COW DISEASE VICTIMS AS SPORADIC CJD ? see video at bottom

http://creutzfeldt-jakob-disease.blogspot.com/2009/07/usa-hiding-mad-cow-disease-victims-as.html

Thursday, August 12, 2010

Seven main threats for the future linked to prions

First threat

The TSE road map defining the evolution of European policy for protection against prion diseases is based on a certain numbers of hypotheses some of which may turn out to be erroneous. In particular, a form of BSE (called atypical Bovine Spongiform Encephalopathy), recently identified by systematic testing in aged cattle without clinical signs, may be the origin of classical BSE and thus potentially constitute a reservoir, which may be impossible to eradicate if a sporadic origin is confirmed. ***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases.

Second threat

snip...

http://www.neuroprion.org/en/np-neuroprion.html

Archive Number 20101206.4364 Published Date 06-DEC-2010 Subject PRO/AH/EDR> Prion disease update 2010 (11)

PRION DISEASE UPDATE 2010 (11)

http://www.promedmail.org/pls/apex/f?p=2400:1001:5492868805159684::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1000,86129

14th ICID International Scientific Exchange Brochure -

Final Abstract Number: ISE.114

Session: International Scientific Exchange

Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009

T. Singeltary

Bacliff, TX, USA

Background:

An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.

Methods:

12 years independent research of available data

Results:

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 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.

Conclusion:

I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.

http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf

International Society for Infectious Diseases Web: http://www.isid.org/

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.html

The most recent assessments (and reassessments) were published in June 2005 (Table I; 18), and included the categorisation of Canada, the USA, and Mexico as GBR III. Although only Canada and the USA have reported cases, the historically open system of trade in North America suggests that it is likely that BSE is present also in Mexico.

http://www.oie.int/boutique/extrait/06heim937950.pdf

Archive Number 20101206.4364 Published Date 06-DEC-2010 Subject PRO/AH/EDR> Prion disease update 2010 (11)

PRION DISEASE UPDATE 2010 (11)

SEE;

TAFS1 Position Paper on Position Paper on Relaxation of the Feed Ban in the EU Berne, 2010 TAFS

INTERNATIONAL FORUM FOR TRANSMISSIBLE ANIMAL DISEASES AND FOOD SAFETY a non-profit Swiss Foundation

http://www.promedmail.org/pls/apex/f?p=2400:1001:5492868805159684::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1000,86129

10,000,000+ LBS. of PROHIBITED BANNED MAD COW FEED I.E. BLOOD LACED MBM IN COMMERCE USA 2007

Date: March 21, 2007 at 2:27 pm PST

RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINES -- CLASS II

___________________________________

PRODUCT

Bulk cattle feed made with recalled Darling's 85% Blood Meal, Flash Dried, Recall # V-024-2007

CODE

Cattle feed delivered between 01/12/2007 and 01/26/2007

RECALLING FIRM/MANUFACTURER

Pfeiffer, Arno, Inc, Greenbush, WI. by conversation on February 5, 2007.

Firm initiated recall is ongoing.

REASON

Blood meal used to make cattle feed was recalled because it was cross- contaminated with prohibited bovine meat and bone meal that had been manufactured on common equipment and labeling did not bear cautionary BSE statement.

VOLUME OF PRODUCT IN COMMERCE

42,090 lbs.

DISTRIBUTION

WI

___________________________________

PRODUCT

Custom dairy premix products: MNM ALL PURPOSE Pellet, HILLSIDE/CDL Prot- Buffer Meal, LEE, M.-CLOSE UP PX Pellet, HIGH DESERT/ GHC LACT Meal, TATARKA, M CUST PROT Meal, SUNRIDGE/CDL PROTEIN Blend, LOURENZO, K PVM DAIRY Meal, DOUBLE B DAIRY/GHC LAC Mineral, WEST PIONT/GHC CLOSEUP Mineral, WEST POINT/GHC LACT Meal, JENKS, J/COMPASS PROTEIN Meal, COPPINI - 8# SPECIAL DAIRY Mix, GULICK, L-LACT Meal (Bulk), TRIPLE J - PROTEIN/LACTATION, ROCK CREEK/GHC MILK Mineral, BETTENCOURT/GHC S.SIDE MK-MN, BETTENCOURT #1/GHC MILK MINR, V&C DAIRY/GHC LACT Meal, VEENSTRA, F/GHC LACT Meal, SMUTNY, A- BYPASS ML W/SMARTA, Recall # V-025-2007

CODE

The firm does not utilize a code - only shipping documentation with commodity and weights identified.

RECALLING FIRM/MANUFACTURER

Rangen, Inc, Buhl, ID, by letters on February 13 and 14, 2007. Firm initiated recall is complete.

REASON

Products manufactured from bulk feed containing blood meal that was cross contaminated with prohibited meat and bone meal and the labeling did not bear cautionary BSE statement.

VOLUME OF PRODUCT IN COMMERCE

9,997,976 lbs.

DISTRIBUTION

ID and NV

END OF ENFORCEMENT REPORT FOR MARCH 21, 2007

http://www.fda.gov/Safety/Recalls/EnforcementReports/2007/ucm120446.htm

Discussion: The C, L and H type BSE cases in Canada exhibit molecular characteristics similar to those described for classical and atypical BSE cases from Europe and Japan. This supports the theory that the importation of BSE contaminated feedstuff is the source of C-type BSE in Canada. *It also suggests a similar cause or source for atypical BSE in these countries.

http://www.prion2009.com/sites/default/files/Prion2009_Book_of_Abstracts.pdf

Saturday, November 6, 2010

TAFS1 Position Paper on Position Paper on Relaxation of the Feed Ban in the EU Berne, 2010 TAFS

INTERNATIONAL FORUM FOR TRANSMISSIBLE ANIMAL DISEASES AND FOOD SAFETY a non-profit Swiss Foundation

http://madcowfeed.blogspot.com/2010/11/tafs1-position-paper-on-position-paper.html

Saturday, August 14, 2010

BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY

(see mad cow feed in COMMERCE IN ALABAMA...TSS)

http://prionpathy.blogspot.com/2010/08/bse-case-associated-with-prion-protein.html

Tuesday, September 14, 2010

Transmissible Spongiform Encephalopathies Advisory Committee; Notice of Meeting October 28 and 29, 2010 (COMMENT SUBMISSION)

http://tseac.blogspot.com/2010/09/transmissible-spongiform_14.html

Wednesday, March 9, 2011

27 U.S. Senators want to force feed Japan Highly Potential North America Mad Cow Beef TSE PRION CJD March 8, 2011

President Barack Obama The White House

1600 Pennsylvania Avenue, W Washington, DC 20500

Dear President Obama:

http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/27-us-senators-want-to-force-feed-japan.html

Sunday, April 17, 2011

Transmission of Prion Strains in a Transgenic Mouse Model Overexpressing Human A53T Mutated [alpha]-Synuclein

Journal of Neuropathology & Experimental Neurology:

POST AUTHOR CORRECTIONS, 8 April 2011 doi: 10.1097/NEN.0b013e318217d95f

http://bse-atypical.blogspot.com/2011/04/transmission-of-prion-strains-in.html

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-same-old-bse.html

Sunday, September 6, 2009

MAD COW USA 1997 SECRET VIDEO

http://madcowusda.blogspot.com/2009/09/mad-cow-usa-1997-video.html

Friday, March 4, 2011

Alberta dairy cow found with mad cow disease

http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/alberta-dairy-cow-found-with-mad-cow.html

Wednesday, August 11, 2010

REPORT ON THE INVESTIGATION OF THE SIXTEENTH CASE OF BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN CANADA

http://bse-atypical.blogspot.com/2010/08/report-on-investigation-of-sixteenth.html

Thursday, August 19, 2010

REPORT ON THE INVESTIGATION OF THE SEVENTEENTH CASE OF BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN CANADA

http://bseusa.blogspot.com/2010/08/report-on-investigation-of-seventeenth.html

Thursday, February 10, 2011

TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY REPORT UPDATE CANADA FEBRUARY 2011 a nd how to hide mad cow disease in Canada Current as of: 2011-01-31

http://madcowtesting.blogspot.com/2011/02/transmissible-spongiform-encephalopathy.html

i wonder if CFIA Canada uses the same OBEX ONLY diagnostic criteria as the USDA ?

Tuesday, November 02, 2010

BSE - ATYPICAL LESION DISTRIBUTION (RBSE 92-21367) statutory (obex only) diagnostic criteria CVL 1992

http://bse-atypical.blogspot.com/2010/11/bse-atypical-lesion-distribution-rbse.html

Monday, April 25, 2011

Experimental Oral Transmission of Atypical Scrapie to Sheep

Volume 17, Number 5-May 2011

http://nor-98.blogspot.com/2011/04/experimental-oral-transmission-of.html

PUTTING THE CART BEFORE THE HORSE, in terms of human health risk $$$

Monday, November 30, 2009

USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH CODE

http://nor-98.blogspot.com/2009/11/usda-and-oie-collaborate-to-exclude.html

Saturday, December 18, 2010

OIE Global Conference on Wildlife Animal Health and Biodiversity - Preparing for the Future (TSE AND PRIONS) Paris (France), 23-25 February 2011

snip...

Greetings,

"Thank for your support to the OIE objectives for a safe world."

NOT !

I see again that the OIE has done little to help eradicate all animal TSE from the globe, and in fact in my opinion, have help enhance the spread of BSE and other animal TSE globally by their industry friendly regulations. I tried to warn the OIE in 2002 about CWD and the potential, but very real threat of CWD to humans. I was told that they were seriously considering this. what happened ? NOW, the OIE and the USDA collaborate to make legal the trading of all strains of atypical BSE legal, and in fact have done so with the atypical scrapie, when science has made perfectly clear the risk factors to humans and other species. I have said it once (see below), and i will say again ;

"THE OIE has now shown they are nothing more than a National Trading Brokerage for all strains of animal TSE. AS i said before, OIE should hang up there jock strap now, since it appears they will buckle every time a country makes some political hay about trade protocol, commodities and futures. IF they are not going to be science based, they should do everyone a favor and dissolve there organization."

NOW, some history on the failed OIE BSE/TSE policy, and why the OIE allowed BSE and other TSE to spread around the globe $$$

snip...

http://transmissiblespongiformencephalopathy.blogspot.com/2010/12/oie-global-conference-on-wildlife.html

Sunday, March 27, 2011

SCRAPIE USA UPDATE FEBRUARY 2011

http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/scrapie-usa-update-february-2011.html

Sunday, April 18, 2010

SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010

http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html

Wednesday, February 16, 2011

IN CONFIDENCE

SCRAPIE TRANSMISSION TO CHIMPANZEES

IN CONFIDENCE

http://scrapie-usa.blogspot.com/2011/02/in-confidence-scrapie-transmission-to.html

Thursday, April 28, 2011

Chronic Wasting Disease Testing and Prevalence Wisconsin April 2011

http://chronic-wasting-disease.blogspot.com/2011/04/chronic-wasting-disease-testing-and.html

Wednesday, January 5, 2011

ENLARGING SPECTRUM OF PRION-LIKE DISEASES Prusiner Colby et al 2011

Prions

David W. Colby1,* and Stanley B. Prusiner1,2

http://betaamyloidcjd.blogspot.com/2011/01/enlarging-spectrum-of-prion-like.html

Saturday, January 2, 2010

Human Prion Diseases in the United States January 1, 2010 ***FINAL***

http://prionunitusaupdate2008.blogspot.com/2010/01/human-prion-diseases-in-united-states.html

my comments to PLosone here ;

http://www.plosone.org/annotation/listThread.action?inReplyTo=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd&root=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd

Friday, March 25, 2011

Detection of Prion Protein in Urine-Derived Injectable Fertility Products by a Targeted Proteomic Approach

http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/detection-of-prion-protein-in-urine.html

Saturday, March 5, 2011

MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE RISE IN NORTH AMERICA

http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/mad-cow-atypical-cjd-prion-tse-cases.html

Tuesday, April 26, 2011

sporadic CJD RISING Text and figures of the latest annual report of the NCJDRSU covering the period 1990-2009 (published 11th March 2011)

http://creutzfeldt-jakob-disease.blogspot.com/2011/04/sporadic-cjd-rising-text-and-figures-of.html

Tuesday, March 29, 2011

TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY EXPOSURE SPREADING VIA HOSPITALS AND SURGICAL PROCEDURES AROUND THE GLOBE

http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/transmissible-spongiform-encephalopathy.html

Thursday, July 08, 2010

GLOBAL CLUSTERS OF CREUTZFELDT JAKOB DISEASE - A REVIEW 2010

http://creutzfeldt-jakob-disease.blogspot.com/2010/07/global-clusters-of-creutzfeldt-jakob.html

Tuesday, September 28, 2010

Variant CJD: where has it gone, or has it?

Pract Neurol 2010; 10: 250–251

http://vcjdtransfusion.blogspot.com/2010/09/variant-cjd-where-has-it-gone-or-has-it.html

http://vcjdtransfusion.blogspot.com/

DID EVERYONE THAT LOST A LOVED ONE FILL OUT THEIR CJD QUESIONNAIRE FROM THE CDC AND OR THE CJD FOUNDATION ???

Friday, November 30, 2007

CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION

http://cjdquestionnaire.blogspot.com/

NOW, tell me again how safe the blood supply is from TSE prions in North America ???

WITH an incubation period of up to 50 years ???

Prion infection begins after one minute of exposure 26 April 2011

http://www.ucl.ac.uk/ion/articles/news/110419

Rapid cell-surface prion protein conversion revealed using a novel cell system

R. Goold,1, 4 S. Rabbanian,1, 4 L. Sutton,1 R. Andre,1 P. Arora,2 J. Moonga,1 A.R. Clarke,2 G. Schiavo,3 P. Jat,1 J. Collinge1, 2 & S.J. Tabrizi1 AffiliationsContributionsCorresponding author Journal name: Nature Communications Volume: 2,Article number:281 DOI: doi:10.1038/ncomms1282 Received08 October 2010Accepted17 March 2011Published19 April 2011

Abstract

Prion diseases are fatal neurodegenerative disorders with unique transmissible properties. The infectious and pathological agent is thought to be a misfolded conformer of the prion protein. Little is known about the initial events in prion infection because the infecting prion source has been immunologically indistinguishable from normal cellular prion protein (PrPC). Here we develop a unique cell system in which epitope-tagged PrPC is expressed in a PrP knockdown (KD) neuroblastoma cell line. The tagged PrPC, when expressed in our PrP-KD cells, supports prion replication with the production of bona fide epitope-tagged infectious misfolded PrP (PrPSc). Using this epitope-tagged PrPSc, we study the earliest events in cellular prion infection and PrP misfolding. We show that prion infection of cells is extremely rapid occurring within 1 min of prion exposure, and we demonstrate that the plasma membrane is the primary site of prion conversion.

http://www.nature.com/ncomms/journal/v2/n4/abs/ncomms1282.html

http://www.nature.com/ncomms/journal/v2/n4/full/ncomms1282.html

http://www.nature.com/ncomms/journal/v2/n4/extref/ncomms1282-s1.pdf

Subject: Transmission of BSE by blood transfusion in sheep...

Date: Thu, 14 Sep 2000 18:19:06 -0700

From: "Terry S. Singeltary Sr."

Reply-To: Bovine Spongiform Encephalopathy

To: BSE-L@uni-karlsruhe.de

######### Bovine Spongiform Encephalopathy #########

Greetings List Members,

More Dredful news, but predictable...

kind regards, Terry S. Singeltary Sr., Bacliff, Texas USA

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

It is possible to transmit BSE to a sheep by transfusion with whole blood taken from another sheep during the symptom-free phase of an experimental BSE infection'

It is well known that variant Creutzfeldt-Jakob disease (vCJD) is caused by the same strain of agent that causes bovine spongiform encephalopathy (BSE) in cattle. F Houston and colleagues report the preliminary findings of transfusing blood from 19 UK Cheviot sheep fed with 5 g BSE-affected cattle brain into Cheviot sheep from scrapie-free flock of New Zealand-derived animals. The investigators found BSE clinical signs and pathology in one recipient of blood taken from a BSE infected animal. Immunocytochemistry on tissues taken from the transfused sheep showed widespread PrPSC deposition throughout the brain and the periphery. This finding suggests that blood donated by symptom-free vCJD-infected human beings could transmit infection to recipients of blood transfusions. In a Commentary, Paul Brown states that these observations are consistent with previous reports in experimentally infected rodents.

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

Research letters Volume 356, Number 9234 16 September 2000

Transmission of BSE by blood transfusion in sheep

Lancet 2000; 356: 999 - 1000 Download PDF (1 Mb)

F Houston, J D Foster, Angela Chong, N Hunter, C J Bostock

See Commentary

We have shown that it is possible to transmit bovine spongiform encephalopathy (BSE) to a sheep by transfusion with whole blood taken from another sheep during the symptom-free phase of an experimental BSE infection. BSE and variant Creutzfeldt-Jakob disease (vCJD) in human beings are caused by the same infectious agent, and the sheep-BSE experimental model has a similar pathogenesis to that of human vCJD. Although UK blood transfusions are leucodepleted--a possible protective measure against any risk from blood transmission--this report suggests that blood donated by symptom-free vCJD-infected human beings may represent a risk of spread of vCJD infection among the human population of the UK.

The demonstration that the new variant of Creutzfeldt-Jakob disease (vCJD) is caused by the same agent that causes bovine spongiform encephalopathy (BSE) in cattle1 has raised concerns that blood from human beings in the symptom-free stages of vCJD could transmit infection to recipients of blood transfusions. There is no evidence that iatrogenic CJD has ever occurred as a result of the use of blood or blood products, but vCJD has a different pathogenesis and could present different risks. CJD is one of the transmissible spongiform encephalopathies (TSEs) characterised by the deposition of an abnormal form of a host protein, PrPSc; the normal isoform (PrPC) is expressed in many body tissues. Available evidence, based on detection of infectivity in blood in rodent models, and absence of infectivity in naturally occurring TSEs, adds to the uncertainty in risk assessments of the safety of human blood. PrPSc has been reported in blood taken from preclinical TSE-infected sheep,2 but it does not follow that blood is infectious. Bioassays of human blood can only be carried out in non-human species, limiting the sensitivity of the test. One way of avoiding such a species barrier is to transfer blood by transfusion in an appropriate animal TSE model. BSE-infected sheep harbour infection in peripheral tissues3 and are thus similar to humans infected with vCJD.4 BSE infectivity in cattle does not have widespread tissue distribution.

We report preliminary data from a study involving blood taken from UK Cheviot sheep challenged orally with 5 g BSE-affected cattle brain and transfused into Cheviot sheep from a scrapie-free flock of New Zealand-derived animals (MAFF/SF flock). MAFF/SF sheep do not develop spontaneous TSE and the transfused animals are housed separately from other sheep. All sheep in the study have the PrP genotype AA136QQ171 which has the shortest incubation period of experimental BSE in sheep.5 19 transfusions from BSE-challenged sheep have been done, mostly with whole blood. Sheep have complex blood groups and only simple cross-matching can be done by mixing recipient serum and donor erythrocytes and vice versa. Therefore single transfusions only were made between sedated cross-matched animals to minimise the risk of severe reactions. Negative controls were MAFF/SF sheep transfused with blood from uninfected UK Cheviot sheep. As a positive control, MAFF/SF sheep were intravenously injected with homogenised BSE-affected cattle brain.

We have seen BSE clinical signs and pathological changes in one recipient of blood from a BSE-infected animal, and we regard this finding as sufficiently important to report now rather than after the study is completed, several years hence. The blood donation resulting in transmission of BSE to the recipient was 400 mL of whole blood taken from a healthy sheep 318 days after oral challenge with BSE. BSE subsequently developed in this donor animal 629 days after challenge, indicating that blood was taken roughly half way through the incubation period. 610 days after transfusion, the transfused sheep (D505) itself developed typical TSE signs: weight loss, moderate pruritus, trembling and licking of the lips, hind-limb ataxia, and proprioceptive abnormalities. This is the first experimental transmission of BSE from sheep to sheep and so we have nothing with which to compare this incubation period directly. In cross-species transmissions, bovine BSE injected intracerebrally gives incubation periods of about 450 days in these sheep,5 and the donor animal had an oral BSE incubation period of 629 days (see above). There are no similar data available on other infection routes. Immunocytochemistry with the antibody BG4 on tissues taken from sheep D505 showed widespread PrPSc deposition throughout the brain and periphery. Western blot analysis of brain tissue with the antibody 6H4 showed that the PrPSc protein had a glycoform pattern similar to that of experimental BSE in sheep and unlike that of UK natural scrapie (figure), indicating that the TSE signs resulted from transmission of the BSE agent. All other recipients of transfusions and positive and negative controls are alive and healthy. The positive controls, which involve a species barrier, are expected to have lengthy incubation periods. With one exception, all transfused animals are at earlier stages post-transfusion than was D505. The exception is a sheep which is healthy 635 days after transfusion with BSE-blood donated at less than 30% of the BSE incubation period of the donor sheep.

PrPSc (proteinase K treated) analysed by SDS-PAGE, immunoblotted with 6H4, and visualised with a chemiluminescent substrate

All lanes are from the same gel with different exposure times. Size markers are to the left of lane 1. Lane1: natural scrapie sheep brain, 3 min exposure. Lane 2: as lane 1, 10 min exposure. Lane 3: sheep D505, blood-transfusion recipient, 10 min exposure. Lane 4: experimental BSE-affected sheep brain, 30 s exposure. Lane 5: as lane 4, 10 min exposure. Each lane loaded with amount of protein extracted from 0·1 g wet weight of brain, except lane 3 which was extracted from 0·2 g brain.

Although this result was in only one animal, it indicates that BSE can be transmitted between individuals of the same species by whole-blood transfusion. We have no data on blood fractions or on levels of infectivity in blood of preclinical vCJD cases, but whole blood is not now used in UK transfusions. The presence of BSE infectivity in sheep blood at an early stage in the incubation period suggests that it should be possible to identify which cells are infected, to test the effectiveness of leucodepletion, and to develop a diagnostic test based on a blood sample.

We thank Karen Brown, Moira Bruce, Calum McKenzie, David Parnham, Diane Ritchie, and the Scottish Blood Transfusion Service. The project is funded by the Department of Health.

1 Bruce ME, Will RG, Ironside JW, et al. Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent. Nature 1997; 389: 488-501 [PubMed].

2 Schmerr MJ, Jenny A, Cutlip RC. Use of capillary sodium dodecyl sulfate gel electrophoresis to detect the prion protein extracted from scrapie-infected sheep. J Chromatogr B Biomed Appl 1997; 697: 223-29 [PubMed].

3 Foster JD, Bruce M, McConnell I, Chree A, Fraser H. Detection of BSE infectivity in brain and spleen of experimentally infected sheep. Vet Rec 1996; 138: 546-48 [PubMed].

4 Hill AF, Zeidler M, Ironside J, Collinge J. Diagnosis of new variant Creutzfeldt-Jakob disease by tonsil biopsy. Lancet 1997; 349: 99-100.

5 Goldmann W, Hunter N, Smith G, Foster J, Hope J. PrP genotype and agent effects in scrapie: change in allelic interaction with different isolates of agent in sheep, a natural host of scrapie. J Gen Virol 1994; 75: 989-95 [PubMed].

Institute for Animal Health, Compton, Newbury, UK (F Houston PhD, CJ Bostock PhD); and Institute for Animal Health, Neuropathogenesis Unit, Edinburgh, EH9 3JF, UK (N Hunter PhD, JD Foster BSc, Angela Chong BSc)

Correspondence to: Dr N Hunter

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

Commentary Volume 356, Number 9234 16 September 2000

BSE and transmission through blood

Lancet 2000; 356: 955 - 956 Download PDF (55 Kb) Wether the outbreak of variant Creutzfeldt-Jakob disease (vCJD) in the UK will ultimately affect hundreds, or tens of thousands of people, cannot yet be predicted.1 If large numbers of apparently healthy people are now silently incubating infections with bovine spongiform encephalopathy (BSE), the implications for public health include the possiblity that blood from such individuals may be infectious. Established facts about infectivity in the blood of human beings and animals with transmissible spongiform encephalopathies (TSEs) are as follows:2-4

Blood, especially the buffy-coat component, from animals experimentally infected with scrapie or CJD and from either a clinical or preclinical incubation phase, is consistently infectious when bioassayed by intracerebral or intraperitoneal inoculation into the same species;

In naturally infected animals (sheep and goats with scrapie, mink with transmissible mink encephalopathy, and cows with BSE), all attempts to transmit disease through the inoculation of blood have failed;

Blood from four of 37 human beings with clinically evident sporadic CJD has been reported to transmit the disease after intracerebral inoculation into guineapigs, mice, or hamsters. But each success has been questioned on technical grounds and has not been reproducible; and

Epidemiological data have not revealed a single case of CJD that could be attributed to the administration of blood or blood products among patients with CJD, or among patients with haemophilia and other congenital clotting or immune deficiencies who receive repeated doses of plasma concentrates.

No comparable information about vCJD is available. However, since lymphoreticular organs, such as tonsils have been shown to contain the prion protein (which is an excellent index of infectivity), whereas it is not detectable in patients with sporadic CJD, there is some reason to worry that blood from individuals incubating vCJD might be infectious.5 Data from studies into the ability of blood from experimentally infected rodents and primates with vCJD to transmit the disease will not be available for months or years.

In this issue of The Lancet, F Houston and co-workers report convincing evidence that blood from a seemingly healthy sheep incubating BSE (infected by the oral route with brain from a diseased cow) was able to cause the disease when transfused into another sheep. This observation is entirely consistent with past experience in experimentally infected rodents. It extends current knowledge about blood infectivity in experimental models to a host/TSE strain pair that is closer to the human vCJD situation than the earlier rodent studies. It is also the first successful transfusion of BSE from blood taken during the all-important incubation period of infection. This result is part of a larger study (n=19) that includes both positive and negative control animals, all still healthy and in various early stages of the incubation period.

Is it appropriate to publish an experimental result from a single animal in a study that is not far enough along even to have validated its positive controls? Especially a result that does not in any fundamental way change our current thinking about BSE and vCJD and which would not seem to have any practical consequences for public health? The UK National Blood Transfusion Service has already implemented leucodepletion of donated blood, and imports all plasma and plasma derivatives from BSE-free countries. No further measures would seem possible--short of a draconian decision to shut down the whole UK blood-donor system. What, therefore, is the rationale for this publishing urgency? The answer, evidently, is a perceived need to "defuse", by an immediate and accurate scientific report, public reaction to possibly inaccurate media accounts. The full study, when it appears, will be an important addition to our knowledge of TSEs, but science should not be driven to what in certain medical quarters might be termed a premature emission through fear of media misrepresentation.

Paul Brown

Laboratory of Central Nervous System Studies, National Institutes of Health, Bethesda, MD 20892, USA

1 Ghani AC, Ferguson NM, Donnelly CA, Anderson RM. Predicted vCJD mortality in Great Britain. Nature 2000; 406: 583-84 [PubMed].

2 Brown P. Can Creutzfeldt-Jakob disease be transmitted by transfusion? Curr Opin Hematol 1995; 2: 472-77 [PubMed].

3 Brown P, Cervenáková L, McShane LM, Barber P, Rubenstein R, Drohan WN. 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 1999; 39: 1169-78 [PubMed].

4 Rohwer RG. Titer, distribution, and transmissibility of blood-borne TSE infectivity. Presented at Cambridge Healthtech Institute 6th Annual Meeting "Blood Product Safety: TSE, Perception versus Reality", MacLean, VA, USA, Feb 13-15, 2000.

5 Hill AF, Butterworth RJ, Joiner S, et al. Investigation of variant Creutzfeldt-Jakob disease and other human prion diseases with tonsil biopsy samples. Lancet 1999; 353: 183-89.

http://www.thelancet.com/

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

Journal of General Virology (2002), 83, 2897–2905. Printed in Great Britain Published ahead of print (16 July 2000) in JGV Direct as DOI 10.1099/vir.0.18580-0 Transmission of prion diseases by blood transfusion Nora Hunter,1 James Foster,1 Angela Chong,1 Sandra McCutcheon,2 David Parnham,1 Samantha Eaton,1 Calum MacKenzie1 and Fiona Houston2

Abstract

Attempts to detect infectivity in the blood of humans and animals affected with transmissible spongiform encephalopathies (TSEs or prion diseases) have often been inconclusive because of the limitations of cross-species bioassays and the small volumes of blood that can be injected by the intracerebral route. A model has been developed for the experimental study of TSE transmission by blood transfusion using sheep experimentally infected with bovine spongiform encephalopathy (BSE) or natural scrapie as donors and susceptible scrapie-free sheep as recipients. Donors and recipients of the same species greatly increase the sensitivity of the bioassay and in sheep large volumes of blood can be injected by the intravenous (i.v.) route. Transmission of BSE to a single animal using this approach was reported recently. This study confirms this result with a second transmission of BSE and four new cases of transmission of natural scrapie. Positive transmissions occurred with blood taken 2 at pre-clinical and clinical stages of infection. Initial studies indicate that following such infection by the i.v. route, deposition of the abnormal prion protein isoform, PrPSc, in peripheral tissues may be much more limited than is seen following oral infection. These results confirm the risks of TSE infection via blood products and suggest that the measures taken to restrict the use of blood in the UK have been fully justified.

http://www.socgenmicrobiol.org.uk/JGVDirect/18580/18580ft.pdf

TSS

Supreme Court Protects Vaccine Manufacturers, Not Injured Children there from Bruesewitz vs Wyeth

2011-02-26T20:46:00.000-08:00

OCTOBER TERM, 2010

Syllabus

NOTE: Where it is feasible, a syllabus (headnote) will be released, as isbeing done in connection with this case, at the time the opinion is issued.The syllabus constitutes no part of the opinion of the Court but has been prepared by the Reporter of Decisions for the convenience of the reader. See United States v. Detroit Timber & Lumber Co., 200 U. S. 321, 337.

SUPREME COURT OF THE UNITED STATES

Syllabus

BRUESEWITZ ET AL. v. WYETH LLC, FKA WYETH, INC., ET AL.

CERTIORARI TO THE UNITED STATES COURT OF APPEALS FOR THE THIRD CIRCUIT

No. 09–152. Argued October 12, 2010—Decided February 22, 2011

The National Childhood Vaccine Injury Act of 1986 (NCVIA or Act) created a no-fault compensation program to stabilize a vaccine market adversely affected by an increase in vaccine-related tort litigation and to facilitate compensation to claimants who found pursuing legitimate vaccine-inflicted injuries too costly and difficult. The Act provides that a party alleging a vaccine-related injury may file a petition for compensation in the Court of Federal Claims, naming the Health and Human Services Secretary as the respondent; that the court must resolve the case by a specified deadline; and that the claimant can then decide whether to accept the court’s judgment or reject it and seek tort relief from the vaccine manufacturer. Awards are paid out of a fund created by an excise tax on each vaccine dose. As a quid pro quo, manufacturers enjoy significant tort-liability protections. Most importantly, the Act eliminates manufacturer liability for a vaccine’s unavoidable, adverse side effects. Hannah Bruesewitz’s parents filed a vaccine-injury petition in the Court of Federal Claims, claiming that Hannah became disabled after receiving a diphtheria, tetanus, and pertussis (DTP) vaccine manufactured by Lederle Laboratories (now owned by respondent Wyeth). After that court denied their claim, they elected to reject the unfavorable judgment and filed suit in Pennsylvania state court, alleging, inter alia, that the defective design of Lederle’s DTP vaccine caused Hannah’s disabilities, and that Lederle was subject to strictliability and liability for negligent design under Pennsylvania common law. Wyeth removed the suit to the Federal District Court. It granted Wyeth summary judgment, holding that the relevant Pennsylvania law was preempted by 42 U. S. C. §300aa–22(b)(1), which 2 BRUESEWITZ v. WYETH LLC

Syllabus

provides that "[n]o vaccine manufacturer shall be liable in a civil action for damages arising from a vaccine-related injury or death associated with the administration of a vaccine after October 1, 1988, if the injury or death resulted from side-effects that were unavoidable even though the vaccine was properly prepared and was accompanied by proper directions and warnings." The Third Circuit affirmed.

Held: The NCVIA preempts all design-defect claims against vaccine manufacturers brought by plaintiffs seeking compensation for injury or death caused by a vaccine’s side effects. Pp. 7–19.

(a) Section 300aa–22(b)(1)’s text suggests that a vaccine’s design is not open to question in a tort action. If a manufacturer could be held liable for failure to use a different design, the "even though" clause would do no work. A vaccine side effect could always have been avoidable by use of a different vaccine not containing the harmful element. The language of the provision thus suggests the design is not subject to question in a tort action. What the statute establishes as a complete defense must be unavoidability (given safe manufacture and warning) with respect to the particular design. This conclusion is supported by the fact that, although products-liability law establishes three grounds for liability—defective manufacture, inadequate directions or warnings, and defective design—the Act mentions only manufacture and warnings. It thus seems that the Act’s failure to mention design-defect liability is "by deliberate choice, not in advertence." Barnhart v. Peabody Coal Co., 537 U. S. 149, 168. Pp. 7–8.

(b) Contrary to petitioners’ argument, there is no reason to believe that §300aa–22(b)(1)’s term "unavoidable" is a term of art incorporating Restatement (Second) of Torts §402A, Comment k, which exempts from strict liability rules "unavoidably unsafe products." "Unavoidable" is hardly a rarely used word, and cases interpreting comment k attach special significance only to the term "unavoidably unsafeproducts," not the word "unavoidable" standing alone. Moreover, reading the phrase "side effects that were unavoidable" to exempt injuries caused by flawed design would require treating "even though"as a coordinating conjunction linking independent ideas when it is a concessive, subordinating conjunction conveying that one clause weakens or qualifies the other. The canon against superfluity does not undermine this Court’s interpretation because petitioners’ competing interpretation has superfluity problems of its own. Pp. 8–12.

(c) The structure of the NCVIA and of vaccine regulation in general reinforces what §300aa–22(b)(1)’s text suggests. Design defects do not merit a single mention in the Act or in Food and Drug Administration regulations that pervasively regulate the drug manufacturing process. This lack of guidance for design defects, combined with

3 Cite as: 562 U. S. ____ (2011) Syllabus

the extensive guidance for the two liability grounds specifically mentioned in the Act, strongly suggests that design defects were not mentioned because they are not a basis for liability. The Act’s mandates lead to the same conclusion. It provides for federal agency improvement of vaccine design and for federally prescribed compensation,which are other means for achieving the two beneficial effects of design-defect torts—prompting the development of improved designs, and providing compensation for inflicted injuries. The Act’s structural quid pro quo also leads to the same conclusion. The vaccine manufacturers fund an informal, efficient compensation program for vaccine injuries in exchange for avoiding costly tort litigation and the occasional disproportionate jury verdict. Taxing their product to fund the compensation program, while leaving their liability for design defect virtually unaltered, would hardly coax them back into the market. Pp. 13–16.

561 F. 3d 233, affirmed.

SCALIA, J., delivered the opinion of the Court, in which ROBERTS,

C. J., and KENNEDY, THOMAS, BREYER, and ALITO, JJ., joined. BREYER, J., filed a concurring opinion. SOTOMAYOR, J., filed a dissenting opinion, in which GINSBURG, J., joined. KAGAN, J., took no part in the consideration or decision of the case. _________________ _________________ 1 Cite as: 562 U. S. ____ (2011)

Opinion of the Court

NOTICE: This opinion is subject to formal revision before publication in thepreliminary print of the United States Reports. Readers are requested tonotify the Reporter of Decisions, Supreme Court of the United States, Washington, D. C. 20543, of any typographical or other formal errors, in orderthat corrections may be made before the preliminary print goes to press.

SUPREME COURT OF THE UNITED STATES

No. 09–152

RUSSELL BRUESEWITZ, ET AL., PETITIONERS v. WYETH LLC, FKA WYETH, INC., FKA WYETHLABORATORIES, ET AL.

ON WRIT OF CERTIORARI TO THE UNITED STATES COURT OFAPPEALS FOR THE THIRD CIRCUIT

[February 22, 2011]

JUSTICE SCALIA delivered the opinion of the Court.

We consider whether a preemption provision enacted inthe National Childhood Vaccine Injury Act of 1986(NCVIA)1 bars state-law design-defect claims againstvaccine manufacturers. .....

snip...please see full text ;

http://www.supremecourt.gov/opinions/10pdf/09-152.pdf

Is polio still a disease seen in the United States? The last cases of naturally occurring paralytic polio in the United States were in 1979, when an outbreak occurred among the Amish in several Midwestern states. From 1980 through 1999, there were 152 confirmed cases of paralytic polio cases reported. Of the 152 cases, eight cases were acquired outside the United States and imported. The last imported case caused by wild poliovirus into the United States was reported in 1993.

The remaining 144 cases were vaccine-associated paralytic polio (VAPP) caused by live oral polio vaccine (OPV).

http://www.cdc.gov/vaccines/vpd-vac/polio/dis-faqs.htm

How do Democrats and Republicans stand on tort reform issues?

"Tort Reform" is a very partisan issue. At both the state and national levels, Republicans overwhelmingly support tort reform and Democrats oppose it. The trial lawyer associations which represent plaintiff lawyers are major contributors to the Democrats. Insurance and medical interests contribute heavily to Republicans. In fact, the tort reform debate can be considered as an aspect of the overall political dynamic involving distribution of the nation's wealth. The most contentious issues involve medical malpractice and product liability. The result of settlements and verdicts of these cases is a transfer of wealth from groups which tend to be wealthy to victims and their lawyers. Virtually all the reform proposals ultimately attempt to limit the amount of funds which are distributed in this fashion.

http://www.newsbatch.com/tort.htm

February 18, 2011 It’s Time to Call Tort Reform What it Really Is Standing Up Against Corporate Tyranny

Jacob Diesselhorst, Nix Law Group, PLLC, Edmond, Oklahoma

I love Oklahoma. I love the people of Oklahoma, all of them. The hard-working men and woman, the elderly-who have paved the way for us and the children-who we are grooming to carry on the legacy for our state’s future.

I love the idea of democracy and freedom. This great country of ours’ was founded based on principles…certain unalienable rights that are bestowed upon all of us– The principle that us all, whether white or black, rich or poor, republican or democrat, should be treated equally and be allowed to play on a level playing field. In turn, our political system, the 3 branches of government, should continue to exemplify this ideal and support our efforts to uphold it. Unfortunately, politics in Oklahoma has deteriorated into a power struggle for control of money and resources as opposed to an effort to embody the principles of freedom, equality and justice.

Like many of us, I am disgusted by the ugly politics and lack of civility that has invaded government. Party lines are now clearly drawn, as both sides teeter for control of a system that is now broken due to the power of special interests. Special interests have infested our politics to the point now there is no way to differentiate between two. Oklahoma politics are no different. Money talks.

The Chamber, big business, big insurance and big oil have a tight grip on this State and the brass of the current leadership of the Oklahoma Republican party. Don’t take my word for it? Let the evidence speak for itself. Look at the campaign finance reports for Oklahoma’s current GOP leadership, they read like a who’s who of Oklahoma’s business community, the super-rich and the PACS (Political Action Committees) formed by the special interests that now dictate Oklahoma politics.

Unfortunately, this influence of the money and power of big business and corporations has infected Republican leadership and convinced them to pursue policy changes that do nothing to protect or assist the general public, but act only to further enrich and empower the voracious and self-serving interests of Corporations and Insurance Companies. One disturbing example of special interests’ invasion into democracy is taking place in Oklahoma right now—with the efforts of a chosen few to force more so-called “tort reform” on the people of Oklahoma.

Tort Reform (as it is called) is the effort by the Big Business wing of the Republican party (at the direction of the corporations who fund their political campaigns and reelections) to invade the sanctity of the judicial branch and legislate in the court room by taking away the autonomy of the civil jury and dictating how juries must decide cases and what evidence is and is not allowed to come in to evidence at trial.

•Politicans want to place an arbitrary, one-size-fits all hard cap of $250k on the amount of damages a civil jury can award a catstrophically injured Oklahoman for the rest of his or her lifetime no matter how severe the injuries and no matter how disgusting, reckless and wanton the conduct of the negligent Defendant.

•GOP Idealogs claim hard caps “deter frivolous lawsuits.” This is a BLATANT LIE. Frivolous lawsuits never see the light of day in Oklahoma. Numerous laws already in place require Expert Reports before lawsuits can be filed. Moreover, Hard Caps do nothing to prevent minor injury lawsuits from being filed. What arbitratry hard caps do cause—is for people with legitimate, long term catstrophic injuries (a brain injured child or a blinded teenager or a badly burned stay-at-home mom or a nursing home resident who is beaten to death and raped in his nursing home by a bad employee) from obtaining full and fair compensation for their injuries.

•Non-economic damages are meant to serve as a deterence to Corporations and individual and ensure that our highways, schools, airports, public transportaion, workplaces, products, etc.) are SAFE. CAPS ELIMINATE THIS DETERENCE and actually give corporations, bad doctors, bad nurses, truck drivers, etc. a free license to run amuck in our state with no fear of every being punished above 250k. (which is like a parking ticket to many multi-million dollar businesses, corporations and insurance companies)

•Further, Policitians want to tip the scales of justice in favor of negligent defendants by giving a negligent tortfeasor’s insurance company and CREDIT for any collateral source of insurance an injured Oklahoman has in awarding damages.

•In other words, if you are horribly injured in a trucking accident and have $150k in medical bills–the jury gets to know your health insurance company paid part of the bills, that part was written off and that part came out of your pocket. Meanwhile, the fact that the negligent trucking company, hospital, oil and gas company etc. has millions of dollars in liability insurance will remain hidden from the jury.

•Even more disturbing, if you had the forsight to purcahse disability insurance or life insurance (in the case of a wrongful death case)–the negligent corporation’s liability insurance company will also get a credit for that insurance money (even though it is from another seperate insurance company). In other words, everyone benefits in this equation (the insurance companies and negligent defendants) EXCEPT THE ONE WHO SHOULD—THE CATASTROPHICALY INJURED OKLAHOMAN.

Our civil justice system is built on a principle of a balance of power and a weighing of the evidence to determine fault, liability and damages. The scales of justice illustrate this ideal. Our court system has successfully existed for hundreds of years built around the JURY. The Jury being a group of citizens like you and me who are entrusted to hear the evidence of a particular case and work together to render a fair and reasoned verdict. Now, however, politicians, not jurors, will decide the amount of damages to award in civil cases involving human injury and death by forcing upon a one-size fits all law that caps the maximum damages at $250,000.00 and decreases any economic damages awarded (medical bills, future medical bills, disability, loss of earnings, etc.) by the amounts of any collateral source of compensation that exists to pay part of them.

In other words, “tort reform” serves to cripple the very purpose of the jury system—to leave it to the people to decide the damages to render against a defendant who has been found to be negligent, reckless or wanton in its conduct and, as a result, caused harm to another person—by taking away the right of jurors (OKLAHOMA CITIZENS) to fairly decide legal disputes.

I for one stand with the people of Oklahoma and their constitutional right to a fair jury trial by the people…not politicians. With “tort reform”—Special Interests are completing the trifecta of infecting all 3 branches of government with their greed and self-serving influence. Meanwhile, the citizens of Oklahoma stand by, unknowlingly, while their rights are taken away by corrupt politicans whose only motivation is to satisfy their biggest campaign donors (i.e., BIG BUSINESS/BIG INSURANCE) so they can hope to get relected.

Sad but true.

Politics as usual in this State cannot be allowed to continue. The very rights to which each of us is entitled now hang in the balance.

David Farnbauch at 7:55 am. (General)

http://www.sweeneylawfirm.com/blog/its-time-to-call-tort-reform-what-it-really-is.htm

Wisconsin May Cap Punitive Damages At $200000 | Tort Reform ...Jan 14, 2011 ... GOP caps punitive damages in tort reform bill - WBAY-TV Green Bay-Fox ... Whenever the Republican party issues their issues platform, they're always for the death ... 2011 Looking Like A Bad Year For Civil Justice (JL) ...

www.tortdeform.com/.../2011/.../wisconsin_may_cap_punitive_dam.html

Justinian Lane Wisconsin May Cap Punitive Damages At $200,000 Continuing the “2011 will be a bad year for civil justice” theme:

The Senate judiciary committee voted 3-2 on Friday to cap punitive damages at $200,000 or twice the amount of compensatory damages, whichever is greater. Current state law does not lay out any caps on punitive damages.

Source: Wis. GOP caps punitive damages in tort reform bill - WBAY-TV Green Bay-Fox Cities-Northeast Wisconsin News

Capping punitive damages at $200k will only encourage corporations to sell profitable but defective products. If it weren’t for punitive damage awards well in excess of $200k, Johns Manville might still be pumping out asbestos products.

I don’t understand Republican ideology. On one hand, most of them support the death penalty. But on the other hand, they don’t like punitive damages. How can you rationalize putting a man to death for committing a crime, but not allowing the family of his victims to take more than $200k?

http://www.tortdeform.com/archives/2011/01/wisconsin_may_cap_punitive_dam.html

http://www.wisbar.org/AM/Template.cfm?Section=News&Template=/CM/ContentDisplay.cfm&ContentID=99772

Archives for February 2011 >

Arbitrary Damage Caps Reward Negligent and Wrongful Actors and Punish the Innocent Citizens of Oklahoma

Posted by Jacob Diesselhorst on February 17, 2011

In 2009 (less than 2 years ago), Oklahoma passed what the then-newly-annointed Republican Majority Senate and House Leadership coined "Landmark Comprehensive Lawsuit Reform."

Per the Oklahoma GOP's own Press Release of May 11, 2009--

“ The agreement is multi-faceted, including several key components of reform which will improve health care access to all Oklahomans, as well as assuring small business’ health and viability in the state.

”“I’m very pleased with the agreement,” said Glen Coffee (the then Republican Senate Majority Leader and now, somehow, Secretary of State (We will save that for another day). In May 2009, Coffee proclaimed “Republicans have promised reform of the legal system, the people expect us to deliver, and we have in fact delivered. The spirit of cooperation exhibited by all parties in this negotiation was gratifying, and is indicative of what can be done when people negotiate in good faith toward a mutually beneficial goal,” Coffee continued. “We look forward to sending this historic reform to Governor Henry to ratify the hard work all the parties have accomplished for the state.”

Current House Speaker Benge (who is now acting like 2009 never happened in pushing the GOP's new Corporate Welfare and Lawsuit Immunity laws) said back in 2009 about the then passed broadsweeping lawsuit reform:

“We have said since the beginning of session that lawsuit reform was one of our top priorities, and this agreement represents a huge victory for the people of Oklahoma today. All interested parties have worked together to make this deal a reality and I am proud of the collaboration that has occurred. This legislation will ensure access to the courts for all who have legitimate claims, but will also bring some certainty to a system that has recently become a hindrance to economic development in our state. I look forward to Gov. Henry signing this bill.”

”Dan Sullivan, a prominent lawyer for insurance companies and large corporations in Oklahoma and GOP Representative from Tulsa, also said in 2009:

“This agreement provides additional protections to the medical and business community from frivolous suits while protecting the right to a trial by a jury of their peers for those that are truly injured.

”HOW SOON WE FORGET. Now, the Oklahoma GOP Big Business Republicans, drunk with power in Oklahoma, having swept their way into the Governor's office and total, unfettered control of Oklahoma, plans to tip the scales of justice completely over in favor of negligent and reckless defendant tort feasors by passing the most broadsweeping and unconstitional civil justice changes in modern US History. These laws should not and in fact do not appeal to social minded and reasonable Conservative Oklahomans. See Example 1 and Example 2.

The Oklahoma GOP is slithering these bills through at the Capital with very little fanfare and Oklahoma is just sitting back while the bandits are making away with the take. In fact, Most Oklahomans do not know about these additional draconian changes and clearly do not know that not a single one of the "reforms" the GOP are now proposing favor individual Oklahomans- they all favor negligent and reckless civil defendants.

ARBITRARY $250K DAMAGE CAPS THAT RESTRICT JURY AWARDS NO MATTER HOW BAD A DEFENDANT ACTS AND HOW HORRIBLY AND PERMANENTLY AN INNOCENT OKLAHOMAN IS INJURED OR MAIMED = NO ACCOUNTABILITY.

And who bears the burden of this Corporate Bailout/Corporate Welfare?---regular Oklahomans- You, me, our kids, our parents, especially the must vulnerable of all:

•children •the elderly •stay at home moms ' •lower wage earners like pastors, fireman, police officers, teachers, etc. According to the Oklahoma GOP, The most damages a jury can ever award these type of Oklahomans, no matter how catastrophic and long-term their injuries may be, is only 250k. 90% of Oklahomans would never support this type of law--yet it will likely sail through the on the backs of the politicians the Chamber put in place, right onto the desk of Mary Failing who will not even read it before she signs it.

After these horrific and unconstitutional bills become law, Oklahomans who have LEGITIMATE CIVIL CASES and whose only avenue for justice is the COURTHOUSE, will be the one's left out in the cold with no way to get JUSTICE for their loss of quality of life---pain and suffering, disability, inability to earn a living, etc.

The Oklahoma GOP's new, so-called "Tort Reform" rewards negligent corporations cutting corners for profits, bad nursing homes, foreign produce manufacturers like drug companies and medical device companies, bad doctors, unsafe truck drivers, drunk drivers, texting drivers etc. and their multi-billion dollar Insurance Company allies. All this at the expense of your constitutional right to a non-biased and fair trial by jury of your peers- Oklahoma citizens.

How about we let Oklahoma juries sort out legitimate civil disputes and not well-greased and bought-and-paid-for politicians (who talk out of both sides of their mouths depending on what year it is) and whose only interests they apparently protect are those of the corporate donors who got them elected and will keep them in office.

Tags:anthony sykes, lawsuit reform, mary fallin, nix law group, oklahoma law, rob johnson, tort reform

http://www.oklahomainjurylawadvocate.com/2011/02/

Sunday, January 30, 2011

Vaccines and Transmissible Spongiform Encephalopathy the Prion Disease, what if ?

COMMERCIAL IN CONFIDENCE

http://transmissiblespongiformencephalopathy.blogspot.com/2011/01/vaccines-and-transmissible-spongiform.html

http://www.whale.to/v/singeltary.html

http://bseinquiry.blogspot.com/2008/05/mad-cow-disease-bse-cjd-children.html

please note ;

PPo2-26:

Transmission of Classical and Atypical (L-type) Bovine Spongiform Encephalopathy (BSE) Prions to Cynomolgus macaques

Fumiko Ono,1 Yoshio Yamakawa,2 Minoru Tobiume,3 Yuko Sato,3 Harutaka Katano,3 Kenichi Hagiwara,2 Iori Itagaki,1 Akio Hiyaoka,1 Katuhiko Komatuzaki,1 Yasunori Emoto,1 Hiroaki Shibata,4 Yuichi Murayama,5 Keiji Terao,4 Yasuhiro Yasutomi4 and Tetsutaro Sata3

1The Corporation for Production and Research of Laboratory Primates; Tsukuba City, Japan; 2Departments of Cell Biology and Biochemistry; and 3Pathology; National Institute of Infectious Diseases; Tokyo, Japan; 4Tsukuba Primate Research Center; National Institute of Biomedical Innovation; Tsukuba City, Japan; 5Prion Disease Research Team; National Institute of Animal Health; Tsukuba City, Japan

Key words: L-type BSE, cBSE, cynomolgus macaques, transmission

BSE prion derived from classical BSE (cBSE) or L-type BSE was characterized by inoculation into the brain of cynomolgus macaques. The neurologic manifestation was developed in all cynomolgus macaques at 27–43 months after intracerebral inoculation of brain homogenate from cBSE-affected cattle (BSE JP/6). Second transmission of cBSE from macaque to macaque shortened incubation period to 13–18 months. cBSE-affected macaques showed the similar clinical signs including hyperekplexia, tremor and paralysis in both primary and second transmission.

Two macaques were intracerebrally inoculated brain homogenate from the L-type BSE-affected cattle (BSE JP/24). The incubation periods were 19–20 months in primary transmission.

The clinical course of the L-type BSE-affected macaques differed from that in cBSE-affected macaques in the points of severe myoclonus without hyperekplexia. The glycoform profile of PrPSc detected in macaque CNS was consistent with original pattern of either cBSE or L-typeBSE PrPSc, respectively. Although severe spongiform change in the brain was remarkable in all BSE-affected macaques, severe spongiform spread widely in cerebral cortex in L-type BSE-affected macaques. Heavy accumulation of PrPSc surrounded by vacuola formed florid plaques in cerebral cortex of cBSE-affected macaques. Deposit of PrPSc in L-type BSE-affected macaque was weak and diffuse synaptic pattern in cerebrum, but large PrPSc plaques were evident at cerebellum. MRI analysis, T2, T1, DW and flair sequences, at the time of autopsy revealed that brain atrophy and dilatation of cerebral ventricles were significantly severe in L-type BSE-affected macaques. These results suggest that L-type BSE is more virulent strain to primates comparing to cBSE.

http://www.prion2010.org/bilder/prion_2010_program_latest_w_posters_4_.pdf?139&PHPSESSID=a30a38202cfec579000b77af81be3099

Saturday, January 29, 2011

Atypical L-Type Bovine Spongiform Encephalopathy (L-BSE) Transmission to Cynomolgus Macaques, a Non-Human Primate

Jpn. J. Infect. Dis., 64 (1), 81-84, 2011

http://transmissiblespongiformencephalopathy.blogspot.com/2011/01/atypical-l-type-bovine-spongiform.html

Wednesday, February 16, 2011 IN CONFIDENCE SCRAPIE TRANSMISSION TO CHIMPANZEES

IN CONFIDENCE

http://scrapie-usa.blogspot.com/2011/02/in-confidence-scrapie-transmission-to.html

Vaccines and Transmissible Spongiform Encephalopathy the Prion Disease, what if ?

COMMERCIAL IN CONFIDENCE

3.2 Minute 5.3 - 5.4 Bovine Spongiform Encephalopathy

It was reported that some replies had been received from Companies using pituitary glands in their products. Copies of the BSE document had also been sent to DHSS and NIBSC.

and then another 3 + pages of blank space. ...TSS

http://collections.europarchive.org/tna/20080102164813/http://www.bseinquiry.gov.uk/files/yb/1988/09/06005001.pdf

COMMERCIAL IN CONFIDENCE

BSE - CURRENT POSITION WITH VETERINARY LICENCED PRODUCTS (MA.1968)

There are three areas of particular concern, vaccines (including emergency vaccines), pharmaceuticals which are covered by MA licences and unlicenses hormonal products produced under exemptions claimed under (Section 9(2) Medicines Act).

1) Vaccines

http://collections.europarchive.org/tna/20080103033809/http://www.bseinquiry.gov.uk/files/yb/1988/10/06005001.pdf

NOT FOR PUBLICATION

another 6 pages of blank space. ...TSS

http://collections.europarchive.org/tna/20080103032658/http://www.bseinquiry.gov.uk/files/yb/1988/11/01012001.pdf

http://collections.europarchive.org/tna/20080103032631/http://www.bseinquiry.gov.uk/files/yb/1988/11/04003001.pdf

http://collections.europarchive.org/tna/20080103033926/http://www.bseinquiry.gov.uk/files/yb/1988/04/00007001.pdf

COMMERCIAL IN CONFIDENCE

http://collections.europarchive.org/tna/20080103034137/http://www.bseinquiry.gov.uk/files/yb/1988/07/00007001.pdf

COMMERCIAL IN CONFIDENCE

Medicines Act - Veterinary Products Committee

http://collections.europarchive.org/tna/20080103034140/http://www.bseinquiry.gov.uk/files/yb/1988/09/00004001.pdf

COMMERCIAL IN CONFIDENCE

NOT FOR PUBLICATION

COMMITTEE ON SAFETY OF MEDICINES

another 6 pages or so that are blank. ...TSS

http://collections.europarchive.org/tna/20080102185137/http://www.bseinquiry.gov.uk/files/yb/1989/01/26007001.pdf

http://collections.europarchive.org/tna/20080102184613/http://www.bseinquiry.gov.uk/files/yb/1989/01/30001001.pdf

COMMERCIAL IN CONFIDENCE

NOT FOR PUBLICATION

COMMITTEE ON SAFETY OF MEDICINES

WORKING PARTY ON BOVINE SPONGIFORM ENCEPHALOPATHY

7.2.1. Products with bovine brain/lymphoid tissue as ingredients and administered by injection...[111]

7.2.2 Products with bovine ingredients (other than brain/lymphoid tissue) and administered by injection...[135]

7.2.3 Tissue implants, open wound dressings, surgical materials, dental and opthalmic products with bovine ingredients...[27]

7.2.4. Products with bovine ingredients and administered topically...[5]

7.2.5 Products with bovine ingredients and administered orally...[9]

7.2.6 Products with other animal/insect/bird ingredients and administered:

a. by injection a: 117

b. by topically b: 6

c. orally c: 8

7.2.7 Products with materials produced from animal material by chemical processes, eg stearic acid, gelatin and lanolin...[156]

With two exceptions, the replies to date have not given any immediate cause for concern, although 176 products do not conform to the CSM/VPC guidelines.

8. The first exception was from which gave very limited information about a very large number of homoepathic medicines with material obtained from cattle and a number with material from the brain. Of these, 53 were injectable products of which 20 were derived from cattle brain. A list of these products is attached as Appendix 1 to Annex D. The second exception relates to the product, 'Surgical Catgut', which is sourced from UK bovine intestines and will contain lymphoid material...

see full text ;

http://collections.europarchive.org/tna/20080102164420/http://www.bseinquiry.gov.uk/files/yb/1989/09/06011001.pdf

COMMERCIAL IN CONFIDENCE

http://collections.europarchive.org/tna/20080102164744/http://www.bseinquiry.gov.uk/files/yb/1988/10/00003001.pdf

MANAGEMENT IN CONFIDENCE

CERTIFIED BSE-FREE HERDS FOR SOURCE OF MATERIAL FOR BIOLOGICAL PRODUCTS

http://collections.europarchive.org/tna/20080102184729/http://www.bseinquiry.gov.uk/files/yb/1989/01/04001001.pdf

Subject: BSE--U.S. 50 STATE CONFERENCE CALL Jan. 9, 2001

Date: Tue, 9 Jan 2001 16:49:00 -0800

From: "Terry S. Singeltary Sr."

Reply-To: Bovine Spongiform Encephalopathy

To: BSE-L@uni-karlsruhe.de

[host Richard Barns] and now a question from Terry S. Singeltary of CJD Watch.

[TSS] yes, thank you, U.S. cattle, what kind of guarantee can you give for serum or tissue donor herds?

[no answer, you could hear in the back ground, mumbling and 'we can't. have him ask the question again.]

[host Richard] could you repeat the question?

[TSS] U.S. cattle, what kind of guarantee can you give for serum or tissue donor herds?

[not sure whom ask this] what group are you with?

[TSS] CJD Watch, my Mom died from hvCJD and we are tracking CJD world-wide.

[not sure who is speaking] could you please disconnect Mr. Singeltary

[TSS] you are not going to answer my question?

[not sure whom speaking] NO

from this point, i was still connected, got to listen and tape the whole conference. at one point someone came on, a woman, and ask again;

[unknown woman] what group are you with?

[TSS] CJD Watch and my Mom died from hvCJD we are trying to tract down CJD and other human TSE's world wide. i was invited to sit in on this from someone inside the USDA/APHIS and that is why i am here. do you intend on banning me from this conference now?

at this point the conference was turned back up, and i got to finish listening. They never answered or even addressed my one question, or even addressed the issue. BUT, i will try and give you a run-down for now, of the conference.

snip...full text ;

http://bse-atypical.blogspot.com/2010/01/14th-international-congress-on.html

The documents below were provided by Terry S. Singeltary Sr on 8 May 2000.

They are optically character read (scanned into computer) and so may contain typos and unreadable parts.

TIP740203/l 0424 CONFIDENTIAL

snip...

The responses by the companies were presented by Ms Turner and were categorised by MCA standards, the products that were discussed were all low volume usage products eg sutures, heart valves.

8. As the responses included some materials of human origin it was decided that more information should be sought about CJD. There had been 2 recent deaths reported associated with human growth hormone. These were being investigated.

snip...

http://www.mad-cow.org/00/may00_news.html#aaa

5.3.3 The greatest risk, in theory, would be from parenteral injection of material derived from bovine brain or lymphoid tissue. Medicinal products for injection or surgical implantation which are prepared from bovine tissues, or which utilise bovine serum albumin or similar agents in their manufacture, might also be capable of transmitting infectious agents. All medicinal products are licensed under the Medicines Act by the Licensing Authority following guidance, for example from the Committee on Safety of Medicines (CSM), the Committee on Dental and Surgical Materials (CDSM) and their subcommittees. The Licensing Authority have been alerted to potential concern about BSE in medicinal products and will ensure that scrutiny of source materials and manufacturing processes now takes account of BSE agent.

see all 76 pages ;

http://collections.europarchive.org/tna/20080102132706/http://www.bseinquiry.gov.uk/files/ib/ibd1/tab02.pdf

EXPORT OF BRITISH BIOLOGICAL PHARMACEUTICALS...

snip...please see full text ;

Sunday, January 30, 2011

Vaccines and Transmissible Spongiform Encephalopathy the Prion Disease, what if ?

COMMERCIAL IN CONFIDENCE

http://transmissiblespongiformencephalopathy.blogspot.com/2011/01/vaccines-and-transmissible-spongiform.html

TSS

Saturday, February 26, 2011

Supreme Court Protects Vaccine Manufacturers, Not Injured Children there from Bruesewitz vs Wyeth

http://vcjdtransfusion.blogspot.com/2011/02/supreme-court-protects-vaccine.html

The risk of variant Creutzfeldt-Jakob disease among UK patients with bleeding disorders, known to have received potentially contaminated plasma products

2011-02-24T13:54:00.000-08:00

The risk of variant Creutzfeldt-Jakob disease among UK patients with bleeding disorders, known to have received potentially contaminated plasma products

S. M. A. ZAMAN1, F. G. H. HILL2,9, B. PALMER3, C. M. MILLAR4,9, A. BONE1, A. M. MOLESWORTH1, N. CONNOR1, C. A. LEE5, G. DOLAN6, J. T. WILDE7,9, O. N. GILL1, M. MAKRIS8,9

Article first published online: 23 FEB 2011

DOI: 10.1111/j.1365-2516.2011.02508.x

© 2011 Blackwell Publishing Ltd

Keywords: haemophilia; inherited bleeding disorders; risk assessment; UK plasma products; variant Creutzfeldt-Jakob disease

Summary. The risk of variant Creutzfeldt-Jakob disease (vCJD) from potentially infected plasma products remains unquantified. This risk has been assessed for 787 UK patients with an inherited bleeding disorder prospectively followed-up for 10–20 years through the UK Haemophilia Centre Doctors’ Organisation (UKHCDO) Surveillance Study. These patients had been treated with any of 25 ‘implicated’ clotting factor batches from 1987 to 1999, which included in their manufacture, plasma from eight donors who subsequently developed clinical vCJD. Variant CJD infectivity of these batches was estimated using plasma fraction infectivity estimates and batch-manufacturing data. Total potential vCJD infectivity received by each patient has been estimated by cumulating estimated infectivity from all doses received during their lifetime. Of 787 patients, 604 (77%) were followed-up for over 13 years following exposure to an implicated batch. For these 604 patients, the estimated vCJD risk is =1% for 595, =50% for 164 and 100% for 51. This is additional to background UK population risk due to dietary exposure. Of 604 patients, 94 (16%) received implicated batches linked to donors who developed clinical vCJD within 6 months of their donations. One hundred and fifty-one (25%) had received their first dose when under 10 years of age. By 1st January 2009, none of these patients had developed clinical vCJD. The absence of clinical vCJD cases in this cohort to date suggests that either plasma fraction infectivity estimates are overly precautionary, or the incubation period is longer for this cohort than for implicated cellular blood product recipients. Further follow-up of this cohort is needed.

http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2516.2011.02508.x/abstract

Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate

Emmanuel E. Comoy1*, Cristina Casalone2, Nathalie Lescoutra-Etchegaray1, Gianluigi Zanusso3, Sophie Freire1, Dominique Marcé1, Frédéric Auvré1, Marie-Magdeleine Ruchoux1, Sergio Ferrari3, Salvatore Monaco3, Nicole Salès4, Maria Caramelli2, Philippe Leboulch1,5, Paul Brown1, Corinne I. Lasmézas4, Jean-Philippe Deslys1

1 Institute of Emerging Diseases and Innovative Therapies, CEA, Fontenay-aux-Roses, France, 2 Istituto Zooprofilattico Sperimentale del Piemonte, Turin, Italy, 3 Policlinico G.B. Rossi, Verona, Italy, 4 Scripps Florida, Jupiter, Florida, United States of America, 5 Genetics Division, Brigham & Women's Hospital, Harvard Medical School, Boston, Massachusetts, United States of America

Abstract

Background Human variant Creutzfeldt-Jakob Disease (vCJD) results from foodborne transmission of prions from slaughtered cattle with classical Bovine Spongiform Encephalopathy (cBSE). Atypical forms of BSE, which remain mostly asymptomatic in aging cattle, were recently identified at slaughterhouses throughout Europe and North America, raising a question about human susceptibility to these new prion strains.

Methodology/Principal

Findings Brain homogenates from cattle with classical BSE and atypical (BASE) infections were inoculated intracerebrally into cynomolgus monkeys (Macacca fascicularis), a non-human primate model previously demonstrated to be susceptible to the original strain of cBSE. The resulting diseases were compared in terms of clinical signs, histology and biochemistry of the abnormal prion protein (PrPres). The single monkey infected with BASE had a shorter survival, and a different clinical evolution, histopathology, and prion protein (PrPres) pattern than was observed for either classical BSE or vCJD-inoculated animals. Also, the biochemical signature of PrPres in the BASE-inoculated animal was found to have a higher proteinase K sensitivity of the octa-repeat region. We found the same biochemical signature in three of four human patients with sporadic CJD and an MM type 2 PrP genotype who lived in the same country as the infected bovine.

Conclusion/Significance

Our results point to a possibly higher degree of pathogenicity of BASE than classical BSE in primates and also raise a question about a possible link to one uncommon subset of cases of apparently sporadic CJD. Thus, despite the waning epidemic of classical BSE, the occurrence of atypical strains should temper the urge to relax measures currently in place to protect public health from accidental contamination by BSE-contaminated products.

Citation: Comoy EE, Casalone C, Lescoutra-Etchegaray N, Zanusso G, Freire S, et al. (2008) Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate. PLoS ONE 3(8): e3017. doi:10.1371/journal.pone.0003017

Editor: Neil Mabbott, University of Edinburgh, United Kingdom

Received: April 24, 2008; Accepted: August 1, 2008; Published: August 20, 2008

Copyright: © 2008 Comoy et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Funding: This work has been supported by the Network of Excellence NeuroPrion.

Competing interests: CEA owns a patent covering the BSE diagnostic tests commercialized by the company Bio-Rad.

* E-mail: emmanuel.comoy@cea.fr

http://www.plosone.org/article/info:doi/10.1371/journal.pone.0003017

Session I - Prions: Structure, Strain and Detection (II)

Searching for BASE Strain Signature in Sporadic Creutzfedlt-Jakob Disease

Gianluigi Zanusso

Department of Neurological and Visual Sciences, Section of Clinical Neurology University of Verona, Verona, Italy.

Bovine amyloidotic spongiform encephalopathy (BASE) is a newly recognized form of bovine prion disease, which was originally detected in Italy in 2004 as an effect of active surveillance. BASE or BSE L-type (L is referred to the lower electrophoretic PrPSc migration than classical BSE) has now been reported in several countries, including Japan. All field cases of BASE were older than 8 years and neurologically normal at the time of slaughtered. By experimental transmission, we defined the disease phenotype of cattle BASE, which is quite distinct from that seen in typical BSE and characterized by mental dullness and amyotrophy. Surprisingly, following intraspecies and interspecies transmission the incubation period of BASE was shorter than BSE. The relatively easy transmission of BASE isolate as well as the molecular similarity with sporadic Creutzfeldt-Jakob disease (sCJD) have raised concern regarding its potential passage to humans. Tg humanized mice Met/Met at codon 129 challenged with both BSE and BASE isolates, showed a resistance to BSE but a susceptibility to BASE at a 60% rate; in addition, BASE-inoculated Cynomolgus (129 Met/Met) had shorter incubation periods than BSE-inoculated primates. In this study we compared the biochemical properties of PrPSc in Cynomolgus and in TgHu Met/Met mice challenged with BSE and BASE strains, by conventional SDS-PAGE analysis and 2D separation. The results obtained disclose distinct conformational changes in PrPSc, which are dependent on the inoculated host but not on the codon 129 genotype.

This work was supported by Neuroprion contract n. FOOD CT 2004 -506579 (NOE)

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

P02.35

Molecular Features of the Protease-resistant Prion Protein (PrPres) in H-type BSE

Biacabe, A-G1; Jacobs, JG2; Gavier-Widén, D3; Vulin, J1; Langeveld, JPM2; Baron, TGM1 1AFSSA, France; 2CIDC-Lelystad, Netherlands; 3SVA, Sweden

Western blot analyses of PrPres accumulating in the brain of BSE-infected cattle have demonstrated 3 different molecular phenotypes regarding to the apparent molecular masses and glycoform ratios of PrPres bands. We initially described isolates (H-type BSE) essentially characterized by higher PrPres molecular mass and decreased levels of the diglycosylated PrPres band, in contrast to the classical type of BSE. This type is also distinct from another BSE phenotype named L-type BSE, or also BASE (for Bovine Amyloid Spongiform Encephalopathy), mainly characterized by a low representation of the diglycosylated PrPres band as well as a lower PrPres molecular mass. Retrospective molecular studies in France of all available BSE cases older than 8 years old and of part of the other cases identified since the beginning of the exhaustive surveillance of the disease in 20001 allowed to identify 7 H-type BSE cases, among 594 BSE cases that could be classified as classical, L- or H-type BSE. By Western blot analysis of H-type PrPres, we described a remarkable specific feature with antibodies raised against the C-terminal region of PrP that demonstrated the existence of a more C-terminal cleaved form of PrPres (named PrPres#2 ), in addition to the usual PrPres form (PrPres #1). In the unglycosylated form, PrPres #2 migrates at about 14 kDa, compared to 20 kDa for PrPres #1. The proportion of the PrPres#2 in cattle seems to by higher compared to the PrPres#1. Furthermore another PK-resistant fragment at about 7 kDa was detected by some more N-terminal antibodies and presumed to be the result of cleavages of both N- and C-terminal parts of PrP. These singular features were maintained after transmission of the disease to C57Bl/6 mice. The identification of these two additional PrPres fragments (PrPres #2 and 7kDa band) reminds features reported respectively in sporadic Creutzfeldt-Jakob disease and in Gerstmann-Sträussler-Scheinker (GSS) syndrome in humans.

http://www.neuroprion.com/pdf_docs/conferences/prion2007/abstract_book.pdf

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

PRION 2010

International Prion Congress: From agent to disease September 8–11, 2010 Salzburg, Austria

PPo3-11:

Blood Transmission Experiments in Primates: Squirrel Monkeys (the Baxter Study)

Paul Brown, James Ironside, Susan Gibson, Robert G. Will, Thomas R. Kreil and Christian Abee

Plasma and buffy coat samples from 2 sCJD and 3 vCJD cases were inoculated i.c. and i.v. into a total of 21 squirrel monkeys. Pooled brain from the 3 vCJD patients titered 106 LD50/g (i.c.). Whole blood from each of 4 monkeys inoculated with 10% vCJD brain homogenate was transfused i.v. to individual recipient monkeys at approximately 3-month intervals during the incubation and clinical stages of disease in the donor animals. Plasma, RBC’s, platelets, and purified leukocytes from 6 chimpanzees infected with either human sCJD or GSS were inoculated i.c. and i.v. into 12 monkeys. In the entire group of monkeys inoculated with blood or blood components, only a single neuropathologically-verified transmission occurred within a 5-year observation period, in an animal inoculated with leukocytes from a pair of GSS-infected chimpanzee.

Conclusions. In a primate model highly susceptible to TSE (the squirrel monkey), infectivity was not detected (<10 i.c. LD50/ml) in plasma or buffy coat from 2 sCJD and 3 vCJD patients, or in whole blood from 1st passage vCJD in monkeys. We did, however, detect infectivity in one monkey inoculated with purified leukocytes from chimpanzee-passaged GSS, and observed what appeared to be a ‘triggering’ of symptomatic disease by physiological stress in chimpanzees subjected to plasmapheresis under general anaesthesia.

PPo4-20:

All Clinically Relevant Components, from Prion Infected Blood Donors, can Cause Disease Following a Single Transfusion

Sandra McCutcheon,1 Fiona E. Houston,2 Anthony R. Alejo-Blanco,1 Christopher de Wolf,1 Boon Chin Tan,1 Anthony Smith,3 Nora Hunter,1 Valerie S. Hornsey,4 Ian R. MacGregor,4 Christopher V. Prowse,4 Marc Turner5 and Jean C. Manson1

1The Roslin Institute; Roslin, Edinburgh UK; 2The University of Glasgow; Glasgow, UK; 3The Institute for Animal Health; Compton, Berkshire UK; 4National Science Laboratory; Scottish National Blood Transfusion Service (SNBTS); Edinburgh, UK; 5University of Edinburgh and SNBTS; Edinburgh, UK

Key words: blood, prion, BSE, transfusion

Introduction. To date, there have been over 220 cases of vCJD worldwide, likely acquired directly from bovine sources. There is concern that human to human transmission from individuals sub-clinically infected with vCJD may amplify/prolong a vCJD epidemic. The area of greatest concern in this respect is blood transfusion, of which there have been several reported cases. Here we examined which blood components are likely to pose the greatest risk of transmitting vCJD via blood transfusion using our sheep BSE model.

Results. 67% of donors have been confirmed as having BSE. We have recorded 25 positive transmissions of BSE following transfusion of non-leucodepleted blood components and 2 transmissions resulting from the transfusion of leucoreduced red cells and leucoreduced plasma.

Conclusion. We show that all components, prepared to the same criteria as used in human medicine, contain sufficient levels of infectivity to cause disease in recipients following a single blood transfusion. Leucoreduction of plasma and red cell concentrates does not remove infectivity. These data indicate the importance of devising appropriate control measures to minimise the risk of human to human transmission of vCJD by blood transfusion. Department of Health, UK (007/0162).

Methods. Sheep were orally infected with bovine BSE brain homogenate. We collected two full-sized donations of whole blood, before the onset of clinical signs. The following components were transfused into naive recipients: whole blood, red cell concentrates buffy coat, plasma and platelet units. We also transfused leucoreduced plasma, platelets and red cells. We collected a unit of whole blood from selected primary recipients for transfusion into secondary recipients.

PPo4-21:

The Risk of Variant Creutzfeldt-Jakob Disease (vCJD) Among UK Patients with Bleeding Disorders, Known to Have Received Clotting Factors Linked to Donors who Subsequently Developed vCJD

Syed M.A. Zaman,1 Nicky Connor,1 Noel Gill,1 Carolyn M. Millar,2,6 Mike Makris,3,6 Benedict Palmer4 and Frank G.H. Hill5,6

1CJD Section, Health Protection Agency Centre for Infections; London, UK; 2Department of Haematology; Imperial College; London, UK; 3University of Sheffield; Royal Hallamshire Hospital; Sheffield, UK; 4National Haemophilia Database; Manchester, UK; 5The Children’s Hospital NHS Foundation Trust; Birmingham, UK; 6Members of the Transfusion Transmitted Infection Working Party of the UK; Haemophilia Centre Doctors’ Organisation (UKHCDO); Sheffield, South Yorkshire UK

The risk of Creutzfeldt-Jakob Disease (vCJD) from potentially infected plasma products remains un-quantified. This risk has been assessed for 787 UK bleeding disorder patients prospectively followed-up for 10–20 years through the UK Haemophilia Centre Doctors’ Organisation (UKHCDO) Surveillance Study. These patients were treated with any of 25 ‘implicated’ clotting factor batches from 1987–1999, which included in their manufacture plasma from eight donors who subsequently developed vCJD. VCJD infectivity of these batches was estimated using plasma fraction infectivity estimates and batch manufacturing data. The quantity of implicated batches received by these patients was obtained. Total vCJD infectivity received by each patient has been estimated by cumulating infectivity from all doses received in their lifetime. Of 787 patients, 604 (77%) were followed-up for over 13 years since exposure to an implicated batch. By end 2008, none of these patients had developed vCJD. For these 604 patients, the estimated vCJD risk is <1% for 595, <50% for 164, and 100% for 51. This is additional to the background UK population risk due to dietary exposure. Of 604 patients, 94 (16%) received implicated batches linked to donors who developed vCJD within six months of their donations. 151 (25%) had received their first dose under 10 years of age. The absence of clinical vCJD cases in this cohort to date suggests that either plasma fraction infectivity estimates are overly precautionary, or the incubation period is longer for this cohort than for implicated cellular blood product recipients. Further follow up of this cohort is needed to answer these questions.

http://www.prion2010.org/bilder/prion_2010_program_latest_w_posters_4_.pdf?139&PHPSESSID=a30a38202cfec579000b77af81be3099

FC5.1.1

Transmission Results in Squirrel Monkeys Inoculated with Human sCJD, vCJD, and GSS Blood Specimens: the Baxter Study

Brown, P1; Gibson, S2; Williams, L3; Ironside, J4; Will, R4; Kreil, T5; Abee, C3 1Fondation Alliance BioSecure, France; 2University of South Alabama, USA; 3University of Texas MD Anderson Cancer Center, USA; 4Western General Hospital, UK; 5Baxter BioSience, Austria

Background: Rodent and sheep models of Transmissible Spongiform Encephalopathy (TSE) have documented blood infectivity in both the pre-clinical and clinical phases of disease. Results in a (presumably more appropriate) non-human primate model have not been reported.

Objective: To determine if blood components (red cells, white cells, platelets, and plasma) from various forms of human TSE are infectious.

Methods: Blood components were inoculated intra-cerebrally (0.1 ml) and intravenously (0.5 ml) into squirrel monkeys from 2 patients with sporadic Creutzfeldt- Jakob disease (sCJD) and 3 patients with variant Creutzfeldt-Jakob disease (vCJD). Additional monkeys were inoculated with buffy coat or plasma samples from chimpanzees infected with either sCJD or Gerstmann-StrÃ¤ussler-Scheinker disease (GSS). Animals were monitored for a period of 5 years, and all dying or sacrificed animals had post-mortem neuropathological examinations and Western blots to determine the presence or absence of the misfolded prion protein (PrPTSE).

Results: No transmissions occurred in any of the animals inoculated with blood components from patients with sporadic or variant CJD. All donor chimpanzees (sCJD and GSS) became symptomatic within 6 weeks of their pre-clinical phase plasmapheresis, several months earlier than the expected onset of illness. One monkey inoculated with purified leukocytes from a pre-clinical GSS chimpanzee developed disease after 36 months.

Conclusion: No infectivity was found in small volumes of blood components from 4 patients with sporadic CJD and 3 patients with variant CJD. ***However, a single transmission from a chimpanzee-passaged strain of GSS shows that infectivity may be present in leukocytes, and the shock of general anaesthesia and plasmspheresis appears to have triggered the onset of illness in pre-clinical donor chimpanzees.

FC5.1.2

Interim Transmission Results in Cynomolgus Macaques Inoculated with BSE and vCJD Blood Specimens

Lasmezas, C1; Lescoutra, N2; Comoy, E2; Holznagel, E3; Loewer, J3; Motzkus, D4; Hunsmann, G4; Ingrosso, L5; Bierke, P6; Pocchiari, M5; Ironside, J7; Will, R7; Deslys, JP2 1Scripps Florida, Infectology, USA; 2CEA, France; 3PEI, Germany; 4DPZ, Germany; 5Istituto Superiore di Sanita, Italy; 6SMI, Sweden; 7CJD Surveillance Unit, UK

BSE and vCJD transmitted to cynomolgus macaques reproduce many features of human vCJD, including clinical symptoms, neuropathological hallmarks of vCJD, PrPres electrophoretical pattern and, most importantly, the wide distribution of infectivity in peripheral organs. The latter characteristic distinguishes vCJD from sCJD in both humans and cynomolgus macaques, and prompted us to use this non-human primate model for further investigations of vCJD and its risk for human health. The occurrence of four vCJD infections in humans transfused with blood from patients who later developed vCJD has raised concern about blood transfusion safety in countries with vCJD. In this collaborative European study, we investigated the infectivity of blood components and whole blood administered by intracerebral (ic) and intravenous (iv) routes. Buffy-coat and whole blood was inoculated by ic and iv route, respectively, from two vCJD patients and from two clinical vCJD-inoculated macaques. Transfusions were also performed from whole blood and blood leucodepleted according to hospital practice standards from two clinical BSE inoculated macaques. Blood infectivity during the preclinical phase is being examined in orally infected macaques. Whole blood was collected and transfused from one such animal two years after oral challenge, whereas buffy-coat and plasma from two animals at 2 and 4.5 years post-challenge, respectively, have been inoculated by the ic route. This is an ongoing study in which recipient animals continue to be observed at various times post-inoculation. So far, we have had one positive transmission in one animal transfused 65 months earlier with 40 ml of whole blood from a vCJD macaque (the characteristics of the disease in this animal will be shown in a separate poster by E. Comoy). This positive transmission reproduces transfusion transmission of vCJD in humans, with an incubation of 5.5 years compatible with incubation periods observed in humans.

http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf

Saturday, September 5, 2009

TSEAC MEETING FEBRUARY 12, 2004 THE BAXTER STUDY GSS

snip...

But the first thing is our own study, and as I mentioned, it's a Baxter primate study, and those are the major participants. And the goal was twofold, and here is the first one: to see whether CJD, either sporadic or familial -- actually it turns out to be the familial CJD is incorrect. It really should be the Fukuoka strain of Gerstmann-Straussler-Scheinker disease. So it's really GSS instead of familial CJD -- when passaged through chimps into squirrel monkeys using purified blood components, very pure blood components.

So this addresses the question that was raised just recently about whether or not red cell infectivity that's been found in rodents is really in the red cells or is it contaminated.

We prepared these samples with exquisite care, and they are ultra-ultra-ultra purified. There's virtually no contamination of any of the components that we looked at ? platelets, red cells, plasma, white cells -- with any other component.

These are a sort of new set of slides, and what I've tried to do is make them less complicated and more clear, but I'm afraid I haven't included the build. So you'll just have to try and follow what I explain with this little red pointer.

There were three initial patients. Two of them had sporadic CJD. One of them had Gerstmann-Straussler-Scheinker syndrome. Brain tissue from each individual patient was inoculated intracerebrally into a pair of chimpanzees. All right?

From those chimps, either plasma or ultra purified -- in fact, everything is ultra-purified. I'll just talk about purified plasma, purified white cells -- were inoculated intracerebrally and intravenously to get the maximum amount of infective load into a pair of squirrel monkeys.

The same thing was done for each of these three sets. This monkey died from non-CJD causes at 34 months post inoculation.

Let me go back for a second. I didn't point out the fact that these were not sacrificed at this point. These chimpanzees were apheresed at 27 weeks when they were still asymptomatic. In this instance, we apheresed them terminally when they were symptomatic.

And before I forget, I want to mention just a little sidelight of this. Chimpanzees in our experience -- and I think we may be the only people that have ever inoculated chimpanzees, and that's no longer a possibility, so this was 20, 30 years ago -- the shortest incubation period of any chimpanzee that we have ever seen with direct intracerebral inoculation is 13 months.

So we chose 27 weeks, which is about seven months, and incidentally typically the incubation period is more like 16 or 18 months. The shortest was 13 months. We chose the 27th week, which is about six and a half months, thinking that this would be about halfway through the incubation period, which we wanted to check for the presence or absence of infectivity.

But within four weeks after the apheresis, which was conducted under general anesthesia for three or four hours apiece, every single one of the six chimpanzees became symptomatic. That is another experiment that I would love to conclude, perhaps because this is simply not heard of, and it very much smells like we triggered clinical illness. We didn't trigger the disease, but it certainly looks like we triggered symptomatic disease at a point that was much earlier than one would have possibly expected.

Maybe it will never be done because it would probably open the floodgates of litigation. There's no end of little things that you can find out from CJD patients after the fact. For example, the neighbor's dog comes over, barks at a patient, makes him fall down, and three weeks later he gets CJD. So you have a lawsuit against the neighbor.

I mean, this is not an unheard of matter, but I do think that physical stress in the form of anesthesia and four hours of whatever goes on with anesthesia, low blood pressure, sometimes a little hypoxemia looks like it's a bad thing.

So here we have the 31st week. All of the chimps are symptomatic, and here what we did was in order to make the most use of the fewest monkeys, which is always a problem in primate research, we took these same three patients and these six chimps. Only now we pooled these components; that is to say, we pooled the plasma from all six chimps. We pooled ultra-purified white cells from all six chimps because here we wanted to see whether or not we could distinguish a difference between intracerebral route of infection and intravenous route of infection.

With respect to platelets and red blood cells, we did not follow that. We inoculated both intracerebral and intravenously, as we had done earlier because nobody has any information on whether or not platelets and red cells are infectious, and so we wanted again to get the maximum.

This is an IV versus IC goal. This one, again, is just getting the maximum load in to see whether there is, in fact, any infectivity in pure platelets, in pure red cells.

And of all of the above, the only transmission of disease related to the inoculation was in a squirrel monkey that received pure leukocytes from the presymptomatic apheresis. So that goes some way to address the question as to whether or not it's a matter of contamination. To date the red cells have not been -- the monkeys that receive red cells have not been observed for more than a year because that was a later experiment.

So we still can't say about red cells, but we're about four and a half years down the road now, and we have a single transmission from purified leukocytes, nothing from plasma and nothing from platelets.

That was the first part of the experiment. The second part was undertaken with the cooperation of Bob Will and others supplying material to us. These were a couple of human, sporadic cases of CJD and three variant cases of CJD from which we obtained buffy coat and plasma separated in a normal way. That is, these are not purified components.

The two cases of sporadic CJD, the plasma was pooled from both patients. The buffy coat was pooled from both patients, and then inoculated intracerebrally and intravenously into three squirrel monkeys each. This is a non-CJD death five years after inoculation. The other animals are still alive.

For variant CJD we decided not to pool. It was more important to eliminate the possibility that there was just a little bit of infectivity in one patient that would have been diluted to extinction, if you like, by mixing them if it were to so occur with two patients, for example, who did not have infectivity. So each one of these was done individually, but the principle was the same: plasma and buffy coat for each patient was inoculated into either two or three squirrel monkeys. This is, again, a non-CJD related death.

In addition to that, we inoculated rain as a positive control from the two sporadic disease cases of human -- from the two human sporadic cases at ten to the minus one and ten to the minus three dilutions. We have done this many, many times in the past with other sporadic patients. So we knew what to expect, and we got exactly what we did expect, namely, after an incubation period not quite two years, all four monkeys developed disease at this dilution and at the minus three dilution, not a whole lot of difference between the two.

Now, these are the crucial monkeys because each one of these monkeys every three to four months was bled and the blood transfused into a new healthy monkey, but the same monkey all the time. So this monkey, for example, would have received in the course of 21 months about six different transfusions of blood from this monkey into this monkey, similarly with this pair, this pair, and this pair. So you can call these buddies. This is sort of the term that was used. These monkeys are still alive.

In the same way, the three human variant CJD specimens, brain, were inoculated into four monkeys, and again, each one of these monkeys has been repeatedly bled at three to four month intervals and that blood transfused into a squirrel monkey, the same one each time. Ideally we would love to have taken bleeding at three months and inoculated a monkey and then let him go, watch him, and then done the same thing at six months. It would have increased the number of monkeys eightfold and just unacceptably expensive. So we did the best we could.

That, again, is a non-CJD death, as is this.

This was of interest mainly to show that the titer of infectivity in brain from variant CJD is just about the same as it from sporadic. We didn't do a minus five and a minus seven in sporadic because we have an enormous experience already with sporadic disease in squirrel monkeys, and we know that this is exactly what happens. It disappears at about ten to the minus five. So the brain titer in monkeys receiving human vCJD is identical to the brain titer in monkeys that have been inoculated with sporadic CJD.

That's the experiment. All of the monkeys in aqua are still alive. They are approaching a five-year observation period, and I think the termination of this experiment will now need to be discussed very seriously in view of a probable six-year incubation period in the U.K. case. The original plan was to terminate the experiment after five years of observation with the understanding that ideally you would keep these animals for their entire life span, which is what we used to do when had unlimited space, money, and facilities. We can't do that anymore.

It's not cheap, but I think in view of the U.K. case, it will be very important to think very seriously about allowing at least these buddies and the buddies from the sporadic CJD to go on for several more years because although you might think that the U.K. case has made experimental work redundant, in point of fact, anything that bears on the risk of this disease in humans is worthwhile knowing, and one of the things we don't know is frequency of infection. We don't know whether this case in the U.K. is going to be unique and never happen again or whether all 13 or 14 patients have received blood components are ultimately going to die. Let's hope not.

The French primate study is primarily directed now by Corinne Lasmezas. As you know, the late Dominique Dromont was the original, originally initiated this work, and they have very active primate laboratory in France, and I'm only going to show two very simple slides to summarize what they did.

The first one is simply to show you the basis of their statement that the IV route of infection looks to be pretty efficient because we all know that the intracerebral route of infection is the most efficient, and if you look at this where they inoculated the same infective load either intracerebrally or intravenously, the incubation periods were not substantially different, which suggests but doesn't prove, but doesn't prove that the route of infection is pretty efficient.

Lower doses of brain material given IV did extend the incubation period and presumably it's because of the usual dose response phenomenon that you see in any infectious disease.

With a whopping dose of brain orally, the incubation period was even lower. Again, just one more example of inefficiency of the route of infection and the necessity to use more infective material to get transmissions.

And they also have blood inoculated IV which is on test, and the final slide or at least the penultimate slide shows you what they have on test and the time of observation, that taken human vCJD and like us inoculated buffy coat, they've also inoculated whole blood which we did not do.

So to a great extent their studies are complementary to ours and makes it all worthwhile.

We have about -- oh, I don't know -- a one to two-year lead time on the French, but they're still getting into pretty good observation periods. Here's three-plus years.

They have variant CJD adapted to the macaque. That is to say this one was passaged in macaque monkeys, the cynomolgus, and they did the same thing. Again, we're talking about a study here in which like ours there are no transmissions. I mean, we have that one transmission from leukocytes, and that's it.

Here is a BSE adapted to the macaque. Whole blood, and then they chose to inoculate leukodepleted whole blood, in both instances IV. Here they are out to five years without a transmission.

And then finally oral dosing of the macaque, which had been infected with -- which was infected with BSE, but a macaque passaged BSE, whole blood buffy coat and plasma, all by the IC route, and they're out to three years.

So with the single exception of the leukocyte transmission from our chimp that was inoculated with a sporadic case of CJD or -- excuse me -- with a GSS, Gerstmann-Straussler, in neither our study nor the French study, which are not yet completed have we yet seen a transmission.

And I will just close with a little cartoon that appeared in the Washington Post that I modified slightly lest you get too wound up with these questions of the risk from blood. This should be a "corrective."

(Laughter.)

DR. BROWN: Thanks.

Questions?

CHAIRPERSON PRIOLA: Yes. Any questions for Dr. Brown? Dr. Linden.

DR. LINDEN: I just want to make sure I understand your study design correctly. When you mention the monkeys that have the IV and IC inoculations, the individual monkeys had both or --

DR. BROWN: Yes, yes, yes. That's exactly right.

DR. LINDEN: So an individual monkey had both of those as opposed to some monkeys had one and some had the other?

DR. BROWN: Correct, correct. Where IC and IV are put down together was IC plus IV into a given monkey.

DR. LINDEN: Into a given monkey. Okay.

And the IC inoculations, where were those given?

DR. BROWN: Right parietal cortex, Southern Alabama.

(Laughter.)

DR. BROWN: Oh, it can't be that clear. Yeah, here, Pierluigi.

CHAIRPERSON PRIOLA: Dr. Epstein.

DR. BROWN: Pierluigi always damns me with feint praise. He always says that's a very interesting study, but. I'm waiting for that, Pierluigi.

I think Jay Epstein --

DR. GAMBETTI: I will say that there's an interesting study and will say, but I just --

(Laughter.)

DR. GAMBETTI: -- I just point of review. You talk about a point of information. You say that -- you mention GSS, I guess, and the what, Fukuowa (phonetic) --

DR. BROWN: Yes, Fukuoka 1.

DR. GAMBETTI: Fukuowa, and is that from the 102, if I remember correctly, of the --

DR. BROWN: Yes, that is correct.

DR. GAMBETTI: Because that is the only one that also --

DR. BROWN: No, it's not 102. It's 101. It's the standard. It's a classical GSS. Oh, excuse me. You're right. One, oh, two is classical GSS. It's been so long since I've done genetics. You're right.

DR. GAMBETTI: Because that is the only one I know, I think, that I can remember that has both the seven kv fragment that is characteristic of GSS, but also the PrPsc 2730. So in a sense, it can be stretching a little bit compared to the sporadic CJD.

DR. BROWN: Yeah, I think that's right. That's why I want to be sure that I made you aware on the very first slide that that was not accurate, that it truly was GSS.

There's a GSS strain that has been adapted to mice, and it's a hot strain, and therefore, it may not be translatable to sporadic disease, correct. All we can say for sure is that it is a human TSE, and it is not variant. I think that's about it.

DR. GAMBETTI: I agree, but this is also not perhaps the best --

DR. BROWN: No, it is not the best. We understand --

DR. GAMBETTI: -- of GSS either.

DR. BROWN: Yeah. If we had to do it over again, we'd look around for a -- well, I don't know. We'd probably do it the same way because we have two sporadics already on test they haven't transmitted, and so you can take your pick of what you want to pay attention to.

Jay?

DR. EPSTEIN: Yes, Paul. Could you just comment? If I understood you correctly, when you did the pooled apheresis plasma from the six chimps when they were symptomatic at 31 weeks, you also put leukocytes into squirrel monkeys in that case separately IV and IC, but in that instance you have not seen an infection come down in squirrel monkey, and the question is whether it's puzzling that you got transmission from the 27-week asymptomatic sampling, whereas you did not see transmission from the 31-week sampling in symptomatic animals.

DR. BROWN: Yes, I think there are two or three possible explanations, and I don't know if any of them are important. The pre-symptomatic animal was almost symptomatic as it turned out so that we were pretty close to the period at which symptoms would being, and whether you can, you know, make much money on saying one was incubation period and the other was symptomatic in this particular case because both bleedings were so close together. That's one possibility.

The other possibility is we're dealing with a very irregular phenomenon and you're not surprised at all by surprises, so to speak so that a single animal, you could see it almost anywhere.

The third is that we, in fact, did just what I suggested we didn't want to do for the preclinical, namely, by pooling we got under the threshold. See?

You can again take that for what it's worth. It is a possible explanation, and again, until we know what the levels of infectivity are and whether by pooling we get under the threshold of transmission, we simply cannot make pronouncements.

CHAIRPERSON PRIOLA: Dr. DeArmond.

DR. DeARMOND: Yeah, it was very interesting data, but the --

(Laughter.)

DR. BROWN: I just love it. Go ahead.

DR. DeARMOND: Two comments. The first one was that the GSS cases, as I remember from reading your publications -- I think Gibbs was involved with them -- when you transmitted the GSS into animals, into monkeys, perhaps I think it was chimps, the transmission was more typical of CJD rather than GSS. There were no amyloid plaques. It was vacuolar degeneration so that you may be transmitting a peculiar form, as I criticized once in Bali and then you jumped all over me about.

DR. BROWN: I may do it again.

DR. DeARMOND: Calling me a bigot and some other few things like that.

(Laughter.)

DR. BROWN: Surely not. I wouldn't have said that.

DR. DeARMOND: So there could be something strange about that particular --

DR. BROWN: Yeah. I think you and Pierluigi are on the same page here. This may be an unusual strain from a number of points of view.

DR. DeARMOND: The other question though has to do with species barrier because the data you're showing is kind of very reassuring to us that it's hard to transmit from blood, but the data from the sheep and from the hamsters and some of the work, I think, that has been done by others, that it's easy in some other animals to transmit, hamster to hamster, mouse to mouse.

Could you comment on the --

DR. BROWN: That's exactly why we went to primates. That's exactly it, because a primate is closer to a human than a mouse is, and that's just common sense.

And so to try and get a little closer to the human situation and not totally depend on rodents for transferrable data, that is why you would use a primate. Otherwise you wouldn't use them. They're too expensive and they cause grief to animal care study people and protocol makers and the whole thing.

Primate studies are a real pain.

DR. DeARMOND: But right now it's inconclusive and you need more time on it.

DR. BROWN: I believe that's true. I think if we cut it off at six years you could still say it was inconclusive, and cutting it off at all will be to some degree inconclusive, and that's just the way it is.

DR. DeARMOND: So what has to be done? Who do you have to convince, or who do we all have to convince to keep that going?

DR. BROWN: Thomas?

Without trying to be flip at all, the people that would be the first people to try to convince would be the funders of the original study. If that fails, and it might for purely practical reasons of finance, then we will have to look elsewhere because I really don't want to see those animals sacrificed, not those eight buddies. Those are crucial animals, and they don't cost a whole lot to maintain. You can maintain eight -- well, they cost a lot from my point of view, but 15 to $20,000 a year would keep them going year after year.

CHAIRPERSON PRIOLA: Dr. Johnson.

DR. JOHNSON: Yeah, Paul, I'm intrigued as you are by the shortening of the incubation period. Have you in all of the other years of handling these animals when they were transfused, when they were flown out to Louisiana at night -- a lot of the stressful things have happened to some of these chimps. Have you ever noticed that before or is this a new observation?

DR. BROWN: Brand new.

MR. JOHNSON: Brand new. Okay.

CHAIRPERSON PRIOLA: Bob, did you want to say something? Dr. Rohwer.

DR. ROHWER: The Frederick fire, wasn't that correlated with a lot of --

DR. BROWN: Not that I k now of, but you may --

DR. ROHWER: Well, that occurred shortly after I came to NIH, and what I remember is that there were a whole bunch of conversions that occurred within the few months following the fire. That was fire that occurred adjacent to the NINDS facility, but in order to protect it, they moved the monkeys out onto the tarmac because they weren't sure it wouldn't burn as well.

DR. BROWN: Well, if you're right, then it's not brand new, but I mean, I'm not sure how we'll ever know because if I call Carlton and ask him, I'm not sure but what I would trust the answer that he gives me, short of records.

You know, Carlot is a very enthusiastic person, and he might say, "Oh, yeah, my God, the whole floor died within three days," but I would want to verify that.

On the other hand, it may be verifiable. There possibly are records that are still extant.

DR. ROHWER: Actually I thought I heard the story from you.

(Laughter.)

DR. BROWN: You didn't because it's brand new for me. I mean, either that or I'm on the way

(Laughter.)

CHAIRPERSON PRIOLA: Dr. Bracey.

DR. BRACEY: I was wondering if some of the variability in terms of the intravenous infection route may be related to intraspecies barriers, that is, the genetic differences, the way the cells, the white leukocytes are processed, whether or not microchimerism is established, et cetera.

DR. BROWN: I don't think that processing is at fault, but the question, the point that you raise is a very good one, and needless to say, we have material with which we can analyze genetically all of the animals, and should it turn out that we get, for example, -- I don't know -- a transmission in one variant monkey and no transmissions in another and a transmission in three sporadic monkeys, we will at that point genetically analyze every single animal that has been used in this study, but we wanted to wait until we could see what would be most useful to analyze.

but the material is there, and if need be, we'll do it.

CHAIRPERSON PRIOLA: Okay. Thank you very much, Dr. Brown.

I think we'll move on to the open public hearing section of the morning.

snip...

http://www.fda.gov/ohrms/dockets/ac/04/transcripts/4019t1.DOC

snip...

see full text ;

http://tseac.blogspot.com/2009/09/tseac-meeting-february-12-2004-baxter.html

Thursday, August 12, 2010

USA Blood products, collected from a donor who was at risk for vCJD, were distributed July-August 2010

http://creutzfeldt-jakob-disease.blogspot.com/2010/08/usa-blood-products-collected-from-donor.html

Sunday, August 01, 2010

Blood product, collected from a donors possibly at increased risk for vCJD only, was distributed USA JULY 2010

http://vcjdtransfusion.blogspot.com/2010/08/blood-product-collected-from-donors.html

14th ICID International Scientific Exchange Brochure -

Final Abstract Number: ISE.114

Session: International Scientific Exchange

Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009

T. Singeltary

Bacliff, TX, USA

Background:

An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.

Methods:

12 years independent research of available data

Results:

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 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.

Conclusion:

I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.

page 114 ;

http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf

International Society for Infectious Diseases Web: http://www.isid.org/

please see full text ;

http://transmissiblespongiformencephalopathy.blogspot.com/

Wednesday, September 08, 2010

Emerging Infectious Diseases: CJD, BSE, SCRAPIE, CWD, PRION, TSE Evaluation to Implementation for Transfusion and Transplantation September 2010

http://vcjdtransfusion.blogspot.com/2010/09/emerging-infectious-diseases-cjd-bse.html

Wednesday, February 2, 2011

Detection of prion infection in variant Creutzfeldt-Jakob disease: a blood-based assay

http://transmissiblespongiformencephalopathy.blogspot.com/2011/02/detection-of-prion-infection-in-variant.html

Monday, February 7, 2011

FDA's Currently-Recommended Policies to Reduce the Possible Risk of Transmission of CJD and vCJD by Blood and Blood Products 2011 ???

http://tseac.blogspot.com/2011/02/fdas-currently-recommended-policies-to.html

Monday, September 13, 2010

atypical BSE strains and sporadic CJD strains, is there a connection and why shouldn't there be $

http://bse-atypical.blogspot.com/2010/09/atypical-bse-strains-and-sporadic-cjd.html

Saturday, January 29, 2011

Atypical L-Type Bovine Spongiform Encephalopathy (L-BSE) Transmission to Cynomolgus Macaques, a Non-Human Primate

Jpn. J. Infect. Dis., 64 (1), 81-84, 2011

http://transmissiblespongiformencephalopathy.blogspot.com/2011/01/atypical-l-type-bovine-spongiform.html

Tuesday, September 14, 2010

Transmissible Spongiform Encephalopathies Advisory Committee; Notice of Meeting October 28 and 29, 2010 (COMMENT SUBMISSION)

http://tseac.blogspot.com/2010/09/transmissible-spongiform_14.html

Sunday, May 10, 2009

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

TO : william.freas@fda.hhs.gov

May 8, 2009

Greetings again Dr. Freas, TSEAC et al,

I would kindly, once again, wish to comment at this meeting about the urgent actions that need to be taken asap, to the Meeting of the Transmissible Spongiform Encephalopathies Committee On June 12, 2009. Due to my disability from my neck injury, I will not be attending this meeting either, however I hope for my submission to be read and submitted. ...

IN reply to ;

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

Friday, February 11, 2011

Creutzfeldt-Jakob disease (CJD) biannual update (2010/1) Emerging infections/CJD

http://creutzfeldt-jakob-disease.blogspot.com/2011/02/creutzfeldt-jakob-disease-cjd-biannual.html

TSS

Variant CJD: where has it gone, or has it?

2010-09-28T08:09:00.000-07:00

Pract Neurol 2010; 10: 250–251

Variant CJD: where has it gone, or has it?

Bob Will

The feared large scale epidemic of variant Creutzfeldt–Jakob disease (vCJD) has thankfully not materialised. The number of cases identifi ed annually in the UK has been in decline since 1999 although there could still be a tail to the outbreak lasting for many years (fi gure). Internationally, the trend in the number of vCJD cases is also in decline and bovine spongiform encephalopathy (BSE) is now a rare disease, even in the UK. One explanation is that the measures introduced to control these diseases were effective; indeed, it is of interest that, to date, no case of vCJD in the UK was born after 1989 when the specifi ed bovine offal ban was introduced whereas there have been three cases born after this date in other European countries where legislative measures to minimise human exposure to BSE were introduced some years later. However, BSE and vCJD control measures are very costly and there will be pressure in the coming years to withdraw or amend relevant legislation and guidance. An important question is whether there are continuing realistic concerns about public health in relation to vCJD and not simply, as has been suggested in the press, scaremongering by some researchers keen to maintain funding.

All probable and definite cases of vCJD internationally, in which genetic testing has been carried out (199/219), have been methionine (MM) homozygous at codon 129 of the prion protein gene. This is clearly a susceptibility factor for the development of clinical disease. However, two out of three prion protein positive appendices in a screening study of 12 674 routine appendix specimens were valine/valine (VV), and a clinically unaffected recipient of a vCJD implicated blood transfusion with disease associated prion protein in their spleen was a heterozygote (MV). This implies that there may be a population of individuals who are subclinically infected but who may never develop clinical disease within their lifespan, perhaps explaining, in part, the mismatch between the extensive human exposure to BSE in the food chain and the relatively limited number of clinical cases so far observed.

This has important implications for the risk of secondary transmission from person to person—for example, through contaminated surgical instruments or blood transfusion. According to one estimate, extrapolating from the estimated prevalence of subclinical infection, 1 in 10 000 blood donations have been derived from individuals who are infected with vCJD. The fact of transfusion transmission of vCJD is now established with three cases of vCJD developing symptoms 5–8 years after having received a blood transfusion from people who donated the blood 1.5–3.5 years before themselves developing the condition (in addition to the subclinical infection referred to above).1 Concerns have been heightened by the discovery of disease associated prion protein in the spleen of a person with haemophilia who had received factor VIII derived from a pool containing a vCJD donation.2 Furthermore, there is a signifi cant population who have been exposed to plasma products potentially contaminated with the vCJD agent but, to date, there is no evidence that clinical cases of vCJD have been caused by exposure to such treatments. One unresolved question is why there have not been more cases of vCJD linked to blood transfusion or plasma products and there is a pressing need to obtain more precise information on the prevalence of infection in the general population.

The recent identifi cation of a possible clinical case of vCJD in an individual with an MV genotype3 reinforces the long held concern that there may be further waves of vCJD cases in individuals with a non-MM codon 129 genotype. Mathematical models suggest that the number of MV and VV cases will be limited and not exceed the primary MM outbreak, but predicting infectious outbreaks is an imprecise science, as may be inferred from the recent swine fl u epidemic which never materialised, at least not to the extent predicted. The adage that ‘Essentially, all models are wrong but some are useful’ (George Edwin Pelham Box, 2007), reinforces the need for caution in predicting the future course of the vCJD outbreak. There is also the possibility that the phenotype of vCJD may be infl uenced by the genetic background. It is reassuring therefore that the recent possible MV case was identifi ed on the basis of the clinical features as this may indicate that any further such cases will also be recognised as vCJD.

However, there is clearly still a continuing need to look for new phenotypes of human prion disease. Novel forms of animal prion diseases have been identifi ed in recent years, including atypical scrapie and the rare H and L forms of atypical BSE, probably as a result of the extensive abattoir testing of animal populations. Atypical BSE has been transmitted to a range of laboratory animals, and in a primate model the incubation period was shorter than with conventional BSE and the clinical and pathological phenotype different.4

The incubation period in human prion disease can extend to decades and there are continuing concerns and uncertainties that indicate that there are good reasons to continue research and surveillance in vCJD, despite the clear decline in the primary outbreak of vCJD.

Competing interests BW holds research grants in relation to CJD research and surveillance. Provenance and peer review Commissioned; not externally peer reviewed.

REFERENCES

1. Hewitt PE, Llewelyn CA, Mackenzie J, et al. Creutzfeldt–Jakob disease and blood transfusion: results of the UK Transfusion Medicine Epidemiological Review study. Vox Sang 2006;91:221–30.

2. Peden A, McCardle L, Head MW, et al. Variant CJD infection in the spleen of a neurologically asymptomatic UK adult patient with haemophilia. Haemophilia 2010;16:296–304.

3. Kaski D, Mead S, Hyare H, et al. Variant CJD in an individual heterozygous for PRNP codon 129. Lancet 2009;374:2128.

4. Comoy EE, Casalone C, Lescoutra-Etchegaray N, et al. Atypical BSE (BASE) transmitted from asymptomatic aging cattle to a primate. PLoS One 2008;3:e3017.

http://pn.bmj.com/content/10/5/250.extract

Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America

14th ICID International Scientific Exchange Brochure -

Final Abstract Number: ISE.114

Session: International Scientific Exchange

Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America

update October 2009

T. Singeltary

Bacliff, TX, USA

Background:

An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.

Methods:

12 years independent research of available data

Results:

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 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.

Conclusion:

I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.

page 114 ;

http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf

International Society for Infectious Diseases Web: http://www.isid.org/

please see full text ;

http://transmissiblespongiformencephalopathy.blogspot.com/

To date the OIE/WAHO assumes that the human and animal health standards set out in the BSE chapter for classical BSE (C-Type) applies to all forms of BSE which include the H-type and L-type atypical forms. This assumption is scientifically not completely justified and accumulating evidence suggests that this may in fact not be the case. Molecular characterization and the spatial distribution pattern of histopathologic lesions and immunohistochemistry (IHC) signals are used to identify and characterize atypical BSE. Both the L-type and H-type atypical cases display significant differences in the conformation and spatial accumulation of the disease associated prion protein (PrPSc) in brains of afflicted cattle. Transmission studies in bovine transgenic and wild type mouse models support that the atypical BSE types might be unique strains because they have different incubation times and lesion profiles when compared to C-type BSE. When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE. In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.

http://www.prionetcanada.ca/detail.aspx?menu=5&dt=293380&app=93&cat1=387&tp=20&lk=no&cat2

please see full text ;

Wednesday, March 31, 2010

Atypical BSE in Cattle

http://bse-atypical.blogspot.com/2010/03/atypical-bse-in-cattle-position-post.html

let's take a closer look at this new prionpathy or prionopathy, and then let's look at the g-h-BSEalabama mad cow.

This new prionopathy in humans? the genetic makeup is IDENTICAL to the g-h-BSEalabama mad cow, the only _documented_ mad cow in the world to date like this, ......wait, it get's better. this new prionpathy is killing young and old humans, with LONG DURATION from onset of symptoms to death, and the symptoms are very similar to nvCJD victims, OH, and the plaques are very similar in some cases too, bbbut, it's not related to the g-h-BSEalabama cow, WAIT NOW, it gets even better, the new human prionpathy that they claim is a genetic TSE, has no relation to any gene mutation in that family. daaa, ya think it could be related to that mad cow with the same genetic make-up ??? there were literally tons and tons of banned mad cow protein in Alabama in commerce, and none of it transmitted to cows, and the cows to humans there from ??? r i g h t $$$

ALABAMA MAD COW g-h-BSEalabama

In this study, we identified a novel mutation in the bovine prion protein gene (Prnp), called E211K, of a confirmed BSE positive cow from Alabama, United States of America. This mutation is identical to the E200K pathogenic mutation found in humans with a genetic form of CJD. This finding represents the first report of a confirmed case of BSE with a potential pathogenic mutation within the bovine Prnp gene. We hypothesize that the bovine Prnp E211K mutation most likely has caused BSE in "the approximately 10-year-old cow" carrying the E221K mutation.

http://www.plospathogens.org/article/info%3Adoi%2F10.1371%2Fjournal.ppat.1000156

http://www.plospathogens.org/article/fetchObjectAttachment.action?uri=info%3Adoi%2F10.1371%2Fjournal.ppat.1000156&representation=PDF

Saturday, August 14, 2010

BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY

(see mad cow feed in COMMERCE IN ALABAMA...TSS)

http://prionpathy.blogspot.com/2010/08/bse-case-associated-with-prion-protein.html

2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006

http://bse-atypical.blogspot.com/2006/08/bse-atypical-texas-and-alabama-update.html

Monday, October 19, 2009

Atypical BSE, BSE, and other human and animal TSE in North America Update October 2009

http://bse-atypical.blogspot.com/2009/10/atypical-bse-bse-and-other-human-and.html

Tuesday, September 14, 2010

Feed Safety and BSE/Ruminant Feed Ban Support Project (U18)

http://madcowfeed.blogspot.com/2010/09/feed-safety-and-bseruminant-feed-ban.html

Tuesday, August 03, 2010

Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein

http://creutzfeldt-jakob-disease.blogspot.com/2010/08/variably-protease-sensitive-prionopathy.html

Monday, August 9, 2010

Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein or just more PRIONBALONEY ?

http://prionunitusaupdate2008.blogspot.com/2010/08/variably-protease-sensitive-prionopathy.html

***+++***

Thursday, July 10, 2008

A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008 Friday, June 20, 2008

http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.html

CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER

>>> Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle. <<<

Irma Linda Andablo CJD Victim, she died at 38 years old on February 6, 2010 in Mesquite Texas

Irma Linda Andablo CJD Victim, she died at 38 years old on February 6, 2010 in Mesquite Texas. She left 6 Kids and a Husband.The Purpose of this web is to give information in Spanish to the Hispanic community, and to all the community who want's information about this terrible disease.-

Physician Discharge Summary, Parkland Hospital, Dallas Texas

Admit Date: 12/29/2009

Discharge Date: 1/20/2010

Attending Provider: Greenberg, Benjamin Morris;

General Neurology Team: General Neurology Team

Linda was a Hispanic female with no past medical history presents with 14 months of incresing/progressive altered mental status, generalized weakness, inability to walk, loss of appetite, inability to speak, tremor and bowel/blader incontinence. She was, in her usual state of health up until February, 2009, when her husbans notes that she began forgetting things like names and short term memories. He also noticed mild/vague personality changes such as increased aggression. In March, she was involved in a hit and run MVA,although she was not injured. The police tracked her down and ticketed her. At that time, her son deployed to Iraq with the Army and her husband assumed her mentation changes were due to stress over these two events. Also in March, she began to have weakness in her legs, making it difficult to walk. Over the next few months, her mentation and personality changes worsened, getting to a point where she could no longer recognized her children. She was eating less and less. She was losing more weight. In the last 2-3 months, she reached the point where she could not walk without an assist, then 1 month ago, she stopped talking, only making grunting/aggressive sounds when anyone came near her. She also became both bowel and bladder incontinent, having to wear diapers. Her '"tremor'" and body jerks worsened and her hands assumed a sort of permanent grip position, leading her family to put tennis balls in her hands to protect her fingers. The husband says that they have lived in Nebraska for the past 21 years. They had seen a doctor there during the summer time who prescribed her Seroquel and Lexapro, Thinking these were sx of a mood disorder. However, the medications did not help and she continued to deteriorate clinically. Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle. The husband says that he does not know any fellow workers with a similar illness. He also says that she did not have any preceeding illness or travel.

http://www.recordandoalinda.com/index.php?option=com_content&view=article&id=19:cjd-english-info&catid=9:cjd-ingles&Itemid=8

Terry S. Singeltary Sr. has added the following comment: "According to the World Health Organisation, the future public health threat of vCJD in the UK and Europe and potentially the rest of the world is of concern and currently unquantifiable. However, the possibility of a significant and geographically diverse vCJD epidemic occurring over the next few decades cannot be dismissed .

The key word here is diverse. What does diverse mean?

If USA scrapie transmitted to USA bovine does not produce pathology as the UK c-BSE, then why would CJD from there look like UK vCJD?"

SEE FULL TEXT ;

http://www.promedmail.org/pls/apex/f?p=2400:1001:568933508083034::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1000,82101

.57 The experiment which might have determined whether BSE and scrapie were caused by the same agent (ie, the feeding of natural scrapie to cattle) was never undertaken in the UK. It was, however, performed in the USA in 1979, when it was shown that cattle inoculated with the scrapie agent endemic in the flock of Suffolk sheep at the United States Department of Agriculture in Mission, Texas, developed a TSE quite unlike BSE. 32 The findings of the initial transmission, though not of the clinical or neurohistological examination, were communicated in October 1988 to Dr Watson, Director of the CVL, following a visit by Dr Wrathall, one of the project leaders in the Pathology Department of the CVL, to the United States Department of Agriculture. 33 The results were not published at this point, since the attempted transmission to mice from the experimental cow brain had been inconclusive. The results of the clinical and histological differences between scrapie-affected sheep and cattle were published in 1995. Similar studies in which cattle were inoculated intracerebrally with scrapie inocula derived from a number of scrapie-affected sheep of different breeds and from different States, were carried out at the US National Animal Disease Centre. 34 The results, published in 1994, showed that this source of scrapie agent, though pathogenic for cattle, did not produce the same clinical signs of brain lesions characteristic of BSE.

32 Clark, W., Hourrigan, J. and Hadlow, W. (1995) Encephalopathy in Cattle Experimentally Infected with the Scrapie Agent, American Journal of Veterinary Research, 56, 606-12

33 YB88/10.00/1.1

http://web.archive.org/web/20040823105233/www.bseinquiry.gov.uk/files/yb/1988/10/00001001.pdf

Monday, August 9, 2010

National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010)

(please watch and listen to the video and the scientist speaking about atypical BSE and sporadic CJD and listen to Professor Aguzzi)

http://prionunitusaupdate2008.blogspot.com/2010/08/national-prion-disease-pathology.html

Tuesday, September 14, 2010

Transmissible Spongiform Encephalopathies Advisory Committee; Notice of Meeting October 28 and 29, 2010 (COMMENT SUBMISSION)

http://tseac.blogspot.com/2010/09/transmissible-spongiform_14.html

Friday, September 24, 2010

USA Blood products, collected from a donor who was at risk for vCJD, were distributed SEPTEMBER 2010

http://vcjdtransfusion.blogspot.com/2010/09/usa-blood-products-collected-from-donor.html

Wednesday, September 08, 2010

Emerging Infectious Diseases: CJD, BSE, SCRAPIE, CWD, PRION, TSE Evaluation to Implementation for Transfusion and Transplantation September 2010

http://vcjdtransfusion.blogspot.com/2010/09/emerging-infectious-diseases-cjd-bse.html

layperson

Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518
flounder9@verizon.net

USA Blood products, collected from a donor who was at risk for vCJD, were distributed SEPTEMBER 2010

2010-09-24T19:04:00.000-07:00

USA Blood products, collected from a donor who was at risk for vCJD, were distributed SEPTEMBER 2010

PRODUCT
Red Blood Cells. Recall # B-2300-10
CODE
Unit: W001607702825
RECALLING FIRM/MANUFACTURER
Recalling Firm: Department of the Air Force, Wright Patterson AFB, OH, by letter dated April 8, 2008.
Manufacturer: Depart of Air Force 88th Medical Group SGQC WPAFB, Wright Patterson AFB, OH. Firm initiated recall is complete.
REASON
Blood product, collected from a donor who was at risk for variant Creutzfeldt-Jakob Disease (vCJD), was distributed.
VOLUME OF PRODUCT IN COMMERCE
1 unit
DISTRIBUTION
Japan

___________________________________

PRODUCT
Recovered Plasma. Recall # B-2302-10
CODE
Units: R08951; P90041; P90041
RECALLING FIRM/MANUFACTURER
Blood Center of Northcentral Wisconsin, Inc., Wausau, WI, by fax on January 2, 2007. Firm initiated recall is complete.
REASON
Blood products, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.
VOLUME OF PRODUCT IN COMMERCE
3 units
DISTRIBUTION
NY

___________________________________

PRODUCT
1) Red Blood Cells Leukocytes Reduced. Recall # B-2338-10;
2) Plasma Frozen. Recall # B-2339-10
CODE
1) and 2) Unit: 5039861
RECALLING FIRM/MANUFACTURER
Community Blood Center, Inc., Appleton, WI, by letter dated September 21, 2007 or by electronic notification on September 21, 2007. Firm initiated recall is complete.
REASON
Blood products, collected from a donor who was at risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.
VOLUME OF PRODUCT IN COMMERCE
2 units
DISTRIBUTION
WI, Switzerland

___________________________________

END OF ENFORCEMENT REPORT FOR SEPTEMBER 22, 2010

http://www.fda.gov/Safety/Recalls/EnforcementReports/ucm227078.htm

PRODUCT

1) Cryoprecipitated AHF, Pooled. Recall # B-2155-10;

2) Recovered Plasma. Recall # B-2156-10

CODE

1) Unit: W036309907231;

2) Unit: W036309616077

RECALLING FIRM/MANUFACTURER

BloodCenter of Wisconsin, Inc., Milwaukee, WI, by fax and internet on May 5, 2010 and May 13, 2010. Firm initiated recall is complete.

REASON

Blood products, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.

VOLUME OF PRODUCT IN COMMERCE

2 units

DISTRIBUTION

TX, Switzerland

___________________________________

PRODUCT

Red Blood Cells Leukocytes Reduced. Recall # B-2157-10

CODE

Unit: 6371718

RECALLING FIRM/MANUFACTURER

South Texas Blood & Tissue Center, San Antonio, TX, by telephone on January 23, 2010 and by fax on January 25, 2010. Firm initiated recall is complete.

REASON

Blood product, collected from a donor who failed to answer questions regarding risk for vCJD, was distributed.

VOLUME OF PRODUCT IN COMMERCE

1 unit

DISTRIBUTION

TX

___________________________________

PRODUCT

Source Plasma. Recall # B-2212-10

CODE

Units: 09FMOG6851; 09FMOG3410; 09FMOG2756; 09FMOG1418; 09FMOF6640; 09FMOF2642; 09FMOF1554; 09FMOD7746; 09FMOF0063; 09FMOF7599

RECALLING FIRM/MANUFACTURER

BioLife Plasma Service LP, Springfield, MO, by fax on April 1, 2010. Firm initiated recall is complete.

REASON

Blood products, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.

VOLUME OF PRODUCT IN COMMERCE

10 units

DISTRIBUTION

CA

___________________________________

PRODUCT

1) Red Blood Cells Leukocytes Reduced. Recall # B-2213-10;

2) Recovered Plasma. Recall # B-2214

CODE

1) and 2) Unit: 6325245

RECALLING FIRM/MANUFACTURER

South Texas Blood and Tissue Center, San Antonio, TX, by facsimile on February 8, 2010. Firm initiated recall is complete.

REASON

Blood products, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.

VOLUME OF PRODUCT IN COMMERCE

2 units

DISTRIBUTION

FL, TX

___________________________________

END OF ENFORCEMENT REPORT FOR SEPTEMBER 15, 2010

http://www.fda.gov/Safety/Recalls/EnforcementReports/ucm225990.htm

PRODUCT
Source Plasma. Recall # B-2056-10
CODE
Units: FD0500537, FD0502880, FD0503259, FD0509894, FD0515518, FD0516063, FD0517957, FD0518606, FD0522255, FD0523346, FD0523544, FD0524204, FD0524698, FD0525142, FD0525845, FD0526653, FD0526878, FD0527579, FD0527845, FD0528519, FD0528827, FD0529544, FD0529761, FD0530471, FD0530712, FD0531425, FD0531801, FD0532483, FD0532869, FD0537501, FD0537687, FD0538370, FD0543210, FD0546250, FD0546632, FD0547328, FD0547832, FD0548286, FD0548743, FD0549325, FD0549840, FD0550427, FD0551448, FD0551572, FD0552307, FD0553173, FD0553418, FD0554063, FD0554834, FD0555041, FD0559685, FD0560235, FD0560592, FD0561168, FD0561786, FD0562212, FD0562883, FD0563248, FD0564435, FD0564723, FD0565467, FD0565880, FD0566540, FD0567053, FD0567723, FD0567965, FD0568941, FD0569180, FD0570057, FD0571177, FD0571477, FD0572411, FD0572818, FD0573582, FD0573871, FD0574531, FD0576955, FD0577140, FD0579983, FD0580403, FD0581156, FD0581623, FD0582680, FD0583090, FD0584073, FD0584500, FD0585410, FD0586089, FD0586790, FD0587500, FD0588791, FD0589023, FD0590248, FD0590600, FD0591592, FD0592445, FD0593277, FD0593712, FD0594626, FD0595049, FD0596132, FD0596519, FD0597701, FD0598681, FD0599198, FD0600210, FD0600690, FD0601755, FD0602401, FD0603415, FD0603985, FD0605122, FD0608608, FD0609074, FD0609979, FD0610508, FD0611469, FD0612006, FD0612905
RECALLING FIRM/MANUFACTURER
DCI Biologicals LLC, Farmington, NM, by facsimile on September 26, 2009 and electronic mail dated January 15, 2010. Firm initiated recall is complete.
REASON
Blood products, collected from a donor who was at risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.
VOLUME OF PRODUCT IN COMMERCE
119 units
DISTRIBUTION
NY, UK

___________________________________

END OF ENFORCEMENT REPORT FOR SEPTEMBER 8, 2010

http://www.fda.gov/Safety/Recalls/EnforcementReports/ucm225223.htm

PRODUCT
Source Plasma. Recall # B-2134-10
CODE
Units: 3910020431, 3910019695, 3910018715, 3910018227, 3910017100, 3910016675, 3910015596, 3910015120, 3910014175, 3910013575, 3910012934, 3910012281, 3910010102, 3910009899, 3910007715, 3910007430
RECALLING FIRM/MANUFACTURER
Talecris Plasma Resources, Inc., N Las Vegas, NV, by fax on July 17, 2009. Firm initiated recall is complete.
REASON
Blood products, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.
VOLUME OF PRODUCT IN COMMERCE
16 units
DISTRIBUTION
NC

___________________________________

PRODUCT
1) Red Blood Cells. Recall # B-2215-10;
2) Fresh Frozen Plasma. Recall # B-2216-10
CODE
1) and 2) Unit: 0951592
RECALLING FIRM/MANUFACTURER
Memorial Blood Centers, Saint Paul, MN, by letter on November 5, 2008. Firm initiated recall is complete.
REASON
Blood products, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.
VOLUME OF PRODUCT IN COMMERCE
2 units
DISTRIBUTION
MN

___________________________________

PRODUCT
Recovered Plasma. Recall # B-2217-10
CODE
Unit: 0951592
RECALLING FIRM/MANUFACTURER
Memorial Blood Centers, Saint Paul, MN, by letter on November 5, 2008. Firm initiated recall is complete.
REASON
Blood product, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), was distributed.
VOLUME OF PRODUCT IN COMMERCE
1 unit
DISTRIBUTION
MN

___________________________________

END OF ENFORCEMENT REPORT FOR SEPTEMBER 1, 2010

http://www.fda.gov/Safety/Recalls/EnforcementReports/ucm224723.htm

Tuesday, September 14, 2010

Transmissible Spongiform Encephalopathies Advisory Committee; Notice of Meeting October 28 and 29, 2010 (COMMENT SUBMISSION)

http://tseac.blogspot.com/2010/09/transmissible-spongiform_14.html

Hi Terry,

Thank you for your comments related to the Transmissible Spongiform Encephalopathy's Advisory Committee meeting on October 28-29, 2010.

Your comments will be provided to the Committee.

Thanks

XXXX

Wednesday, September 08, 2010

Emerging Infectious Diseases: CJD, BSE, SCRAPIE, CWD, PRION, TSE Evaluation to Implementation for Transfusion and Transplantation September 2010

http://vcjdtransfusion.blogspot.com/2010/09/emerging-infectious-diseases-cjd-bse.html

Saturday, July 17, 2010

Variant Creutzfeldt-Jakob disease Ironside JW., Haemophilia.

2010 Jul;16 Suppl 5:175-80 REVIEW ARTICLE

http://vcjdtransfusion.blogspot.com/2010/07/variant-creutzfeldtjakob-disease.html

Monday, August 9, 2010

National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010)

(please watch this damning video at the bottom of this url...tss)

http://prionunitusaupdate2008.blogspot.com/2010/08/national-prion-disease-pathology.html

TSS