Thursday, November 27, 2008

Prion diseases are efficiently transmitted by blood transfusion in sheep

TRANSFUSION MEDICINE

Prion diseases are efficiently transmitted by blood transfusion in sheep


Fiona Houston1, Sandra McCutcheon1, Wilfred Goldmann2, Angela Chong2, James Foster2, Silvia Sisó3, Lorenzo González3, Martin Jeffrey3, and Nora Hunter2 1 Neuropathogenesis Division, Roslin Institute, Compton, United Kingdom; 2 Neuropathogenesis Division, Roslin Institute, University of Edinburgh, Edinburgh, United Kingdom; and 3 Lasswade Laboratory, Veterinary Laboratories Agency, Penicuik, United Kingdom

The emergence of variant Creutzfeld-Jakob disease, following on from the bovine spongiform encephalopathy (BSE) epidemic, led to concerns about the potential risk of iatrogenic transmission of disease by blood transfusion and the introduction of costly control measures to protect blood supplies. We previously reported preliminary data demonstrating the transmission of BSE and natural scrapie by blood transfusion in sheep. The final results of this experiment, reported here, give unexpectedly high transmission rates by transfusion of 36% for BSE and 43% for scrapie. A proportion of BSE-infected tranfusion recipients (3 of 8) survived for up to 7 years without showing clinical signs of disease. The majority of transmissions resulted from blood collected from donors at more than 50% of the estimated incubation period. The high transmission rates and relatively short and consistent incubation periods in clinically positive recipients suggest that infectivity titers in blood were substantial and/or that blood transfusion is an efficient method of transmission. This experiment has established the value of using sheep as a model for studying transmission of variant Creutzfeld-Jakob disease by blood products in humans.


http://bloodjournal.hematologylibrary.org/cgi/content/abstract/112/12/4739?ct



Plasma & Serum Proteins Receive Continued FDA Approval

4/25/2008
APC, Inc. is pleased to advise our customers and industry partners that as anticipated, the Food and Drug Administration (FDA) will continue to allow the use of bovine blood, plasma and serum proteins in ruminant feeds.


In April 2008 FDA announced the publication of its Final Rule for 21 CFR Part 589.2001 - Substances Prohibited From Use in Animal Food or Feed. FDA specifically stated in their opinion that, "FDA is not prohibiting the use of blood and blood products in animal feed because we believe such a prohibition would do very little to reduce the risk of BSE transmission."


Known as a leader in developing nutritional products for the swine industry, where 95% of pig starter diets in the United States contain functional proteins, APC has more recently developed their line of colostrum replacers, supplements, feed additives and milk replacer ingredients for calves. Products include plasma, serum and immunoglobulin concentrate based Acquire®, Lifeline®, Gammulin® and Nutrapro® used to optimize the health and performance of calves.


To view the full report for Final Rule 21 CFR Part 589.2001 visit:

http://www.fda.gov/OHRMS/DOCKETS/98fr/FDA-2002-N-0031-nfr.pdf


To view the complete Feed Rule 21 CFR Part 589 visit:

http://www.accessdata.fda.gov/scripts/cdrh/cfdocs/cfcfr/CFRSearch.cfm?CFRPart=589&showFR=1



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

From: Terry S. Singeltary Sr.
To: FREAS@CBER.FDA.GOVCc: william.freas@fda.hhs.gov ; rosanna.harvey@fda.hhs.gov
Sent: Wednesday, November 29, 2006 1:24 PM
Subject: TSE advisory committee for the meeting December 15, 2006 [TSSSUBMISSION]

November 29, 2006

Greetings FDA, DHH, Dr. Freas, and Dr. Harvey et al, a kind and warm Holiday Greetings to you all. i kindly wish to submit the following to the TSE advisory committee for the meeting December 15, 2006, about the assessment for potential exposure to vCJD in human plasma-derived antihemophilic factor (FVIII) products manufactured from U.S. plasma donors and related communication material ;

http://a257.g.akamaitech.net/7/257/2422/01jan20061800/edocket.access.gpo.gov/2006/E6-20251.htm


i see the media picked up on this as a 'low risk', from what the gov. agency perceived to be to them;

http://www.newsday.com/news/health/ats-ap_health14nov27,0,7955259.story?coll=ny-leadhealthnews-headlines

however, i seem to disagree. from my primitive ciphering, i see it another way. this is a huge catastrophic risk. 3 in 160 is 1.9%. so call that 2% which is 1 in 50 or twenty per thousand or 20,000 per million. also, what about the mixed genotypes/mixed susceptibility? what about the silent carriers that donated tainted blood? what about the sporadic CJDs of UNKNOWN strain or phenotype? this risk assessment is just more BSe to me. just another in a long line of industry fed crap. i pray that my assessment is the one that is wrong. but it is THEY who roll the dice with your life. it is THEY who refuse to regulate an industry that has run amok. just from a recall aspect of potentially tainted blood, and these are just recent recalls ;


PRODUCT


Source Plasma, Recall # B-0054-7CODEUnits: 03MMNC5465, 03MMNC6361, 03MMNC6801, 03MMNC7510, 03MMNC7891,03MMNC8252, 03MMNC8801, 03MMNC9144, 03MMND1122, 03MMND1478, 03MMND1969,03MMND2350, 03MMND2825, 03MMND3211, 03MMND3708, 03MMND4072, 03MMND4588,03MMND4831, 03MMND5320, 03MMND5719, 03MMND6268, 03MMND6683, 03MMND7228,03MMND7656, 03MMND8211, 03MMND8652, 03MMND9195, 03MMND9618, 03MMNE0628,03MMNE0884, 03MMNE1597, 03MMNE1979, 03MMNE2644, 03MMNE3064, 03MMNE3707,03MMNE4122, 03MMNE4750, 03MMNE5080, 03MMNE5876, 03MMNE6218, 03MMNE7189,03MMNE7587, 03MMNE8027, 03MMNE8645, 03MMNE9029, 03MMNE9641, 03MMNE9979,03MMNF0491, 03MMNF0685, 03MMNF0937, 03MMNF1260, 04MMNA0351, 04MMNA0707,04MMNA1241, 04MMNA1650, 04MMNA2291, 04MMNA2646, 04MMNA3340, 04MMNA3719,04MMNA4312, 04MMNA4683, 04MMNA5298, 04MMNA5750, 04MMNA6407, 04MMNA6816,04MMNA7482, 04MMNA7915, 04MMNA8632, 04MMNA9076, 04MMNA9723, 04MMNB0063,04MMNB0696, 04MMNB1100, 04MMNB1845, 04MMNB2285, 04MMNB3035, 04MMNB3485,04MMNB4213, 04MMNB4672, 04MMNB5841, 04MMNB6652, 04MMNB7162, 04MMNB7930,04MMNB8453, 04MMNB9239, 04MMNB9747, 04MMNC0456, 04MMNC0931, 04MMNC1578RECALLING FIRM/MANUFACTURERBioLife Plasma Services, L.P., Mankato, MN, by facsimile on June 4, 2004.Firm initiated recall is complete.REASONBlood products, collected from a donor who was at increased risk for newvariant Creutzfeldt-Jakob Disease (nvCJD), were distributed.

VOLUME OF PRODUCT IN COMMERCE

89 unitsDISTRIBUTIONCA and Austria

END OF ENFORCEMENT REPORT FOR October 25, 2006###


http://www.fda.gov/bbs/topics/enforce/2006/ENF00975.html


USA FDA MAD COW BLOOD HUMANS RECALL (these are dime a dozen)

RECALLS AND FIELD CORRECTIONS: BIOLOGICS -- CLASS II______________________________PRODUCTSource Plasma, Recall # B-1708-6CODEUnits: MI180733, MI180927, MI181625, MI181780, MI182337, MI182519, MI183140,MI183311, MI183955, MI185006, MI185278, MI185822, MI186081, MI186855,MI187183, MI187903, MI188273, MI188695, MI189257, MI189553, MI190136,MI190473, MI191073, MI191395, MI191972, MI192303, MI193473, MI194343,04MINA0377, 04MINA0801, 05MINA7147, 05MINA7451, 05MINA8094, 05MINA8504,05MINA9548, 05MINA9883, 05MINB0489, 05MINB0875, 05MINB1469, 05MINB1874,05MINB3116, 05MINB7192, 05MINB7529, 05MINB8246, 05MINB8612, 05MINB9236,05MINB9366, 05MINB9475, 05MINB9641, 05MINC0031, 05MINC0237, 05MINC0336,05MINC0894, 05MINC0964, 05MINC1138, 05MINC1619, 05MINC1750, 05MINC1907,05MINC1977, 05MINC2375, 05MINC2774, 05MINC3113, 05MINC3484, 05MINC4277,05MINC4623, 05MINC5623, 05MINC5914, 05MINC7545, 05MINC7870, 05MINC8355,05MINC8689, 05MINC9437, 05MINC9775, 05MIND0067, 05MIND0393, 05MIND0892,05MIND0951, 05MIND1836, 05MIND2183 and 05MIND2962RECALLING FIRM/MANUFACTURERBioLife Plasma Services L.P., Muncie, IN, by facsimile on November 22, 2005.Firm initiated recall is complete.REASONBlood products, collected from unsuitable donors based on risk factors forCreutzfeldt-Jakob Disease (CJD), were distributed.VOLUME OF PRODUCT IN COMMERCE80 unitsDISTRIBUTIONCA, NC, and MD______________________________

PRODUCTa) Red Blood Cells, Leukocytes Reduced, Recall # B-1714-6;b) Fresh Frozen Plasma, Recall # B-1715-6;c) Platelets, Recall # B-1716-6CODEa), b), and c) Unit: 2443732

RECALLING FIRM/MANUFACTURERSouth Texas Blood and Tissue Center, San Antonio, TX, by letters datedNovember 11, 2003 and December 18, 2003. Firm initiated recall is complete.REASONBlood products, co lected from a donor who was at increased risk for newvariant Creutzfeldt-Jakob Disease (nvCJD), were distributed.

VOLUME OF PRODUCT IN COMMERCE3 unitsDISTRIBUTIONTX and WIEND OF ENFORCEMENT REPORT FOR SEPTEMBER 13, 2006###


http://www.fda.gov/bbs/topics/enforce/2006/ENF00969.html


PRODUCT

Fresh Frozen Plasma, Recall # B-1751-6CODEUnit: 4936623RECALLING FIRM/MANUFACTURERGulf Coast Regional Blood Center, Houston, TX, by facsimile dated September16, 2005. Firm initiated recall is complete.REASONBlood product, which was collected from an unsuitable donor based on riskfactors for variant Creutzfeldt-Jakob Disease (vCJD), was distributed.

VOLUME OF PRODUCT IN COMMERCE

1 unit DISTRIBUTION TX

END OF ENFORCEMENT REPORT FOR SEPTEMBER 6, 2006###


http://www.fda.gov/bbs/topics/enforce/2006/ENF00968.html


Mon Aug 7, 2006 10:2471.248.132.189
PRODUCTa) Red Blood Cells, Recall # B-1587-6;b) Cryoprecipitated AHF, Recall # B-1588-6;c) Recovered Plasma, Recal # B-1589-6CODEa), b) and c) Unit: 2016719RECALLING FIRM/MANUFACTURERWalter Shepeard Community Blood Center, Inc., Augusta, GA, by facsimile onMarch 13, 2003. Firm initiated recall is complete.REASONBlood products, which were collected from a donor who may be at increasedrisk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.

VOLUME OF PRODUCT IN COMMERCE 3 units DISTRIBUTION GA and Germany______________________________

PRODUCT

a) Red Blood Cells Leukocytes Reduced, Recall # B-1590-6;b) Fresh Frozen Plasma, Recall # B-1591-6CODEa) and b) Unit: 2443595RECALLING FIRM/MANUFACTURERSouth Texas Blood and Tissue Center, San Antonio, TX, by facsimile on June30, 2004. Firm initiated recall is complete.

REASON

Blood products, which were collected from a donor who may be at increasedrisk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.

VOLUME OF PRODUCT IN COMMERCE 2 units DISTRIBUTION TX______________________________

PRODUCT


a) Red Blood Cells Leukocytes Reduced, Recall # B-1592-6;b) Fresh Frozen Plasma, Recall # B-1593-6CODEa) and b) Unit: 2545596RECALLING FIRM/MANUFACTURERSouth Texas Blood and Tissue Center, San Antonio, TX, by facsimile onDecember 14, 2004 and January 3, 2005. Firm initiated recall is complete.REASONBlood products, which were collected from a donor who may be at increasedrisk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.

VOLUME OF PRODUCT IN COMMERCE 2 units DISTRIBUTION TX______________________________


http://www.fda.gov/bbs/topics/enforce/2006/ENF00963.html


SNIP...FULL TEXT ;

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


http://creutzfeldt-jakob-disease.blogspot.com/2008/10/cber-2007-annual-report-assessing.html


http://www.fda.gov/OHRMS/DOCKETS/ac/06/transcripts/2006-4271t1.pdf



Friday, October 24, 2008

CBER 2007 Annual Report Assessing the Potential Risk of variant Creutzfeldt-Jakob Disease from Blood Products

http://creutzfeldt-jakob-disease.blogspot.com/2008/10/cber-2007-annual-report-assessing.html



Docket APHIS-2006-0041 Docket Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived from Bovines Commodities Docket Type Rulemaking Document APHIS-2006-0041-0001 Document Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived From Bovines Public Submission APHIS-2006-0041-0006.1 Public Submission Title Attachment to Singletary comment



http://www.regulations.gov/fdmspublic/ContentViewer?objectId=09000064801f3412&disposition=attachment&contentType=crtext




http://www.regulations.gov/fdmspublic/component/main?main=DocumentDetail&d=APHIS-2006-0041-0006.1




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




TSS


Tuesday, November 11, 2008

SaBTO Summary of 1st Public Meeting - variant CJD and blood Tuesday 21st October 2008, 2pm-4pm

http://vcjdblood.blogspot.com/2008/11/sabto-summary-of-1st-public-meeting.html



Friday, November 21, 2008
Amarillo-area (suspect sporadic CJD) case linked to mad cow disease Rumor in Texas

http://cjdtexas.blogspot.com/2008/11/amarillo-area-suspect-sporadic-cjd-case.html




ONE HUNDRED AND FIRST MEETING OF THE SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE

Oct 23, 2008 at 9:00 AM

http://seac992007.blogspot.com/2008/10/one-hundred-and-first-meeting-of_23.html


http://flounder068.vox.com/library/post/one-hundred-and-first-meeting-of-the-spongiform-encephalopathy-advisory-committee.html


Wednesday, June 11, 2008

OIE Recognition of the BSE Status of Members RESOLUTION No. XXI (Adopted by the International Committee of the OIE on 27 May 2008)

snip...SEE FULL TEXT with facts and sources @ ;

http://usdavskorea.blogspot.com/2008/06/oie-recognition-of-bse-status-of.html


http://organicconsumers.org/forum/index.php?showtopic=1566


Friday, April 25, 2008

Substances Prohibited From Use in Animal Food or Feed [Docket No. 2002N-0273] (Formerly Docket No. 02N-0273) RIN 0910-AF46

http://madcowfeed.blogspot.com/2008/04/substances-prohibited-from-use-in.html


Review on the epidemiology and dynamics of BSE epidemics

Cases of atypical BSE have only been found in countries having implemented large active surveillance programs. As of 1st September 2007, 36 cases (16 H, 20 L) have been described all over the world in cattle: Belgium (1 L) [23], Canada (1 H)15, Denmark (1 L)16, France (8 H, 6 L)17, Germany (1 H, 1 L) [13], Italy (3 L)18, Japan (1 L) [71], Netherlands (1 H, 2 L)19, Poland (1 H, 6 L)20, Sweden (1 H)21, United Kingdom (1 H)22, and USA (2 H)23. Another H-type case has been found in a 19 year old miniature zebu in a zoological park in Switzerland [56]. It is noteworthy that atypical cases have been found in countries that did not experience classical BSE so far, like Sweden, or in which only few cases of classical BSE have been found, like Canada or the USA.

And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.

full text 18 pages ;

http://www.vetres.org/index.php?option=article&access=standard&Itemid=129&url=/articles/vetres/pdf/2008/04/v07232.pdf


USA BSE ACTIVE SURVEILLANCE ???

http://bse-atypical.blogspot.com/2008/06/review-on-epidemiology-and-dynamics-of.html


Please remember, the last two mad cows documented in the USA i.e. Alabama and Texas, both were of the 'atypical' BSE strain, and immediately after that, the USDA shut down the testing from 470,000 to 40,000 in the U.S. in 2007 out of about 35 million cattle slaughtered. also, science is showing that some of these atypical cases are more virulent to humans than the typical UK BSE strain ;

***Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.***

Progress Report from the National Prion Disease Pathology Surveillance Center

An Update from Stephen M. Sergay, MB, BCh & Pierluigi Gambetti, MD

April 3, 2008

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


In this context, a word is in order about the US testing program. After the discovery of the first (imported) cow in 2003, the magnitude of testing was much increased, reaching a level of >400,000 tests in 2005 (Figure 4). Neither of the 2 more recently indigenously infected older animals with nonspecific clinical features would have been detected without such testing, and neither would have been identified as atypical without confirmatory Western blots. Despite these facts, surveillance has now been decimated to 40,000 annual tests (USDA news release no. 0255.06, July 20, 2006) and invites the accusation that the United States will never know the true status of its involvement with BSE.

In short, a great deal of further work will need to be done before the phenotypic features and prevalence of atypical BSE are understood. More than a single strain may have been present from the beginning of the epidemic, but this possibility has been overlooked by virtue of the absence of widespread Western blot confirmatory testing of positive screening test results; or these new phenotypes may be found, at least in part, to result from infections at an older age by a typical BSE agent, rather than neonatal infections with new "strains" of BSE. Neither alternative has yet been investigated.

http://www.cdc.gov/ncidod/EID/vol12no12/06-0965.htm


Tuesday, June 3, 2008

SCRAPIE USA UPDATE JUNE 2008 NOR-98 REPORTED PA

http://nor-98.blogspot.com/2008/06/scrapie-usa-update-june-2008-nor-98.html


Friday, August 29, 2008 CREEKSTONE VS USDA COURT OF APPEALS, BUSH SAYS, NO WAY, NO HOW

http://madcowtesting.blogspot.com/2008/08/creekstone-vs-usda-court-of-appeals.html



Friday, November 21, 2008
Plasma & Serum Proteins Receive Continued FDA Approval

http://madcowfeed.blogspot.com/2008/11/plasma-serum-proteins-receive-continued.html


http://madcowfeed.blogspot.com/



PRION October 8th - 10th 2008 Book of Abstracts

snip...

0C3.01

Transmission of atypical BSE to Microcebus murinus, a non-human primate: Development of clinical symptoms and tissue distribution of PrPres

Background: Atypical BSE cases have been observed in Europe, Japan and North America. They differ in their PrPres profiles from those found in classical BSE. These atypical cases fall into 2 types, depending on the molecular mass of the unglycosylated PrPres band observed by Western blot: the L -type (lower molecular mass than the typical BSE cases) and H-type (higher molecular mass than the typical BSE cases).

Objectives and Methods: In order to see if the atypical BSE cases were transmissible to primates, either animals (were intracerebrally inoculated with 50 ul of a 10% brain homogenates of two atypical French BSE case, a H-type (2 males and 2 females) and a L-type (2 males and 2 females).

Results: Only one of the four lemurs challenged with H-type BSE died without clinical signs after 19 months post inoculation (mpi), whereas all the 4 animals inoculated with L -type BSE died at 19 mpi (2 males) and 22 mpi (2 females). Three months before their sacrifice, they developed blindness, tremor, abnormal posture, incoordinated movements, balance loss. Symptoms got worse according to the disease progression, until severe ataxia. The brain tissue were biochemically and immunocytochemically investigated for PrPres. For the H-type, spongiform changes without PrPres accumulation were observed in the brainstem. However Western blot analysis did not allow to detect PrPres into the brain. For the L-type, severe spongiosis was evidenced into the thalamus, the striatum, the mesencephalon, and the brainstem. whereas into the cortex the spongiosis was evidenced, but the Vacuolisation was weaker. Strong deposits of PrPres were detected by western blot, PET-blot and immunocytochemistry in the CNS: dense accumulation was observed into the thalamus, the striatum, and the hippocampus whereas in the cerebral cortex, PrPres was prominently accumulated in plaques. Western blot analysis also readily confirmed the presence of protease-resistant prion protein.

Conclusions: L-type infected lemurs showed survival times considerably shorter than for classical BSE strain, indicating that the disease is caused by a very virulent distinct prion strain in a model of non human primate.

http://www.neuroprion.org/resources/pdf_docs/conferences/prion2008/abstract-book-prion2008.pdf


P7.09

Biochemical screening for identification of atypical bse in belgium, 1999-present

Authors

Alexandre DobIy: Caroline Rodeghiero, Riet Geeroms; Stephanie Durand, Jessica De Sloovere, Emanuel Yanopdenbosch, Stefan Roels,

Content

Background: Recently atypical forms of BSE have been described. Western blot analyses showed that, in comparison to the classic BSE (C-type), they are demonstrable by a higher or lower molecular weight of the unglycosylated PrPres. They Viere thus named H-type and L-type BSE (L-type is also called BASE). In addition they show a lower proportion of diglycosylated PrPres than C-type. These emerging types represent different strains of BSE. They show unique incubation periods and histological lesions. Such types have been described on different continents. Indeed they might correspond to "sporadic" forms of BSE. In 2004 we already described one L-type in Belgium.

Objective: We retrospectively analysed the bovines at least 7-year-old in the Belgian archive of BSE ­diagnosed cattle in order to determine the prevalence of the two types of atypical BSE in Belgium.

Methods: We analysed homogenates from 39 bovines of 93 months old in median (min: 84, max: 181 months). The most recent one was diagnosed in 2006. We used Western blot with a panel of anti-PrP antibodies (Ab). They detect different regions of the PrP protein, from N-terminal to C-terminal: 12B2, 9A2, Sha31. SAFB4, 94B4. Their combination is aimed at an efficient typing diagnostic. We detected bound Ab with SuperSignal West Dura (Pierce) and analysed PrPres, signals with an image-analysis software (Quantity One, Bio-Rad).

Results: The results are still under analysis. We will detail the most crucial characteristics for typing PrPres. These include 1) the apparent molecular mass of the an-, mono- and diglycosylated bands, 2) the binding affinity to the five Ab (e.g.12B2 for H-type), 3) the presence of a fourth (unglycosylated) band and 4) the glycoprofile based on the relative proportions of the visible bands.

Discussion: The emergence of atypical types of BSE is partially due to a better knowledge of prion strains and more efficient diagnostic techniques. As the area in the brain where the PrPres is deposited can differ drastically between the types, it is essential to ascertain that the sampling techniques and analyses are adapted to these new types. As these new strains seem more virulent than classic types, they represent one of the next challenges in the field of prions.

http://www.neuroprion.org/resources/pdf_docs/conferences/prion2008/abstract-book-prion2008.pdf


http://www.prion2008.com/


Tuesday, November 11, 2008

Transmission of atypical bovine prions to mice transgenic for human prion protein

DOI: 10.3201/eid1412.080941

http://bse-atypical.blogspot.com/2008/11/transmission-of-atypical-bovine-prions.html


Wednesday, August 20, 2008

Bovine Spongiform Encephalopathy Mad Cow Disease typical and atypical strains, was there a cover-up ?

http://bse-atypical.blogspot.com/2008/08/bovine-spongiform-encephalopathy-mad.html


Tuesday, June 3, 2008 SCRAPIE USA UPDATE JUNE 2008 NOR-98 REPORTED PA

http://nor-98.blogspot.com/2008/06/scrapie-usa-update-june-2008-nor-98.html


SCRAPIE USA

http://scrapie-usa.blogspot.com/


Sunday, September 07, 2008

CWD LIVE TEST, and the political aspects or fallout of live testing for BSE in cattle in the USA

http://chronic-wasting-disease.blogspot.com/2008/09/cwd-live-test-and-political-aspects-or.html


Saturday, October 18, 2008 WYOMING STAR VALLEY MOOSE TESTS POSITIVE FOR CWD

http://chronic-wasting-disease.blogspot.com/2008/10/wyoming-star-valley-moose-tests.html


http://chronic-wasting-disease.blogspot.com/


Saturday, December 01, 2007 Phenotypic Similarity of Transmissible Mink Encephalopathy in Cattle and L-type Bovine Spongiform Encephalopathy in a Mouse Model Volume 13, Number 12-December 2007 Research

snip...see full text ;

http://transmissible-mink-encephalopathy.blogspot.com/2007/12/phenotypic-similarity-of-transmissible.html


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, April 20, 2008 Progress Report from the National Prion Disease Pathology Surveillance Center April 3, 2008

Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.

see full text ;

http://prionunitusaupdate2008.blogspot.com/2008/04/progress-report-from-national-prion.html


ONE HUNDRED AND FIRST MEETING OF THE SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Oct 23, 2008 at 9:00 AM

http://seac992007.blogspot.com/2008/10/one-hundred-and-first-meeting-of_23.html


http://flounder068.vox.com/library/post/one-hundred-and-first-meeting-of-the-spongiform-encephalopathy-advisory-committee.html


Friday, November 21, 2008

Plasma & Serum Proteins Receive Continued FDA Approval

http://madcowfeed.blogspot.com/2008/11/plasma-serum-proteins-receive-continued.html


http://madcowfeed.blogspot.com/


Sunday, November 23, 2008 PRION October 8th - 10th 2008 Book of Abstracts

http://bse-atypical.blogspot.com/2008/11/prion-october-8th-10th-2008-book-of.html


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

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Tuesday, November 11, 2008

SaBTO Summary of 1st Public Meeting – variant CJD and blood Tuesday 21st October 2008, 2pm-4pm

Advisory Committee on the Safety of Blood, Tissues and Organs (SaBTO)

Summary of 1st Public Meeting – variant CJD and blood

Tuesday 21st October 2008, 2pm-4pm

New King’s Beam House, London SE1

Introduction


Mr John Forsythe – University of Edinburgh/SaBTO Chair

Mr Forsythe welcomed the audience, and outlined the background of SaBTO and the work of the committee. He explained that the committee feels that it is vital to share information used to make decisions and to invite comments from interested parties and the public. This explains the committee’s decision to hold an annual public meeting on selected topic.

1st Presentation: Introduction to variant CJD

Dr Hester Ward – National CJD Surveillance Unit/SaBTO vCJD expert

Dr Ward gave an introductory talk on the cause and epidemiology of variant CJD.

1. There are several different types of prion diseases in humans, which can be divided into idiopathic (e.g. sporadic CJD), acquired (e.g. variant CJD) and genetic (e.g. familial CJD). There is no evidence that sporadic CJD can be transmitted via transfusion.

2. Variant CJD emerged in 1996 with features distinct from those seen in sporadic CJD. It affects younger people, who survive longer, and there are also distinct clinical and neuropathological features.

3. 18 of those affected by vCJD are known to have been blood donors. 66 recipients of their blood have been identified, of whom 23 survive.

4. vCJD is infective in blood but there remain a large number of uncertainties. A self-sustaining secondary epidemic through blood transfusion may be possible.

5. Key to determining the size of a secondary epidemic is measuring how may people in the population may be carriers of vCJD (the ‘population prevalence’).

6. A study on stored appendix samples and one on tonsils have given estimates for carriers of between 1 in 1, 500 and 1 in 20, 000 of the UK population.

7. Possible further studies of this type could involve more appendix samples, a post-mortem archive or blood tests, which are not yet available but are in development.

2nd Presentation: Variant CJD and Blood: current safety measures and future options

Professor Marc Turner – University of Edinburgh and Scottish National Blood Transfusion Service/SaBTO Haematologist

Professor Turner gave a presentation on the issues around variant CJD and blood, the current safety measures in place, and options that may be considered in the future.

1. The prevalence of sub-clinical infection amongst the population of blood donors was discussed. The best estimate for incidence of further clinical cases is 70 .

2. This represents a discrepancy with the retrospective tonsil and appendix study data which indicates that the prevalence of sub-clinical infection of vCJD is 1/4000 (range 1/1000 to 1/20000). This suggests that up to 3,000 members of the UK population may be infected but remain sub clinical (symptom free) in the longer term.

3. It is known that in rodent models the potential level of peripheral infection is around 10 infectious doses per ml of blood (range 1-300 ID / ml).

4. These data taken together with the known transmission of variant CJD by blood suggest the possibility of an ongoing risk of transmission of variant CJD through blood, tissues and organs.

5. In the face of uncertainty, the best risk management approaches are those that give at least some control of risk over a wide range of plausible scenarios. In the context of variant CJD and blood, there are four potential approaches; donor selection, donor screening, component processing and minimising exposure.

6. Although a number of donor deferral criteria have been introduced both in the UK and internationally, they represent a relatively blunt risk management approach and can undermine the supply of blood and tissues.

7. A future test for vCJD may detect the presence of the abnormal protein in blood which would help to control the risk of secondary transmission , but will not give definitive information on the likelihood of development of clinical disease and will pose problems around sensitivity and specificity, validation and the management of those who have tested positive. There are particular concerns around the impact of a ‘poor’ test on donors and the blood supply.

8. Blood component processing is used to further reduce risk from transfusion. Red cells are re-suspended in optimal additive solution rather than plasma, and all red blood cells undergo leucodepletion. Using prion reduction filters to filter out prions from leucodepleted red blood cells may further reduce infectivity and these are currently under evaluation.

9. Risk reduction by reducing exposure to blood represents an immediately available, low cost measure that is relatively straightforward to implement.

3rd Presentation – A Patient’s Perspective

Mr Elwyn Nicol – SaBTO Patient Representative

Mr Nicol, explained that some 5 years ago he underwent a heart transplant, hence his interest in becoming the SaBTO patient representative.With no family connections or experience in, or background of, the medical profession. Mr Nicol represents the patient. He emphasised that his views are independent and well placed to express an opinion, based on his own experience.To lay members in this audience he confirmed that the well-being of patients, on this committee, seems paramount.There are 2.4m blood donors in the UK and it is claimed that the vCJD testing programmes being developed may be 99% effective. Mr Nicol said he considers that to be very good, but it could mean 1%, i.e. 24,000 donations or donors would be falsely told that they had tested positive for vCJD.

Until testing for vCJD in blood is 100% reliable, potentially 24000 blood donors, who 'do something special' without reward could be falsely told they have the disease every year. They could not know if they are truly threatened, not know if they will ever be able to get a life insurance policy, ever be able to get a mortgage or ever be able to buy a pension.
As a heavily transfused patient, Mr Nicol was and remains, content with the range of precautionary measures that exist. Until a 100% test exists Mr Nicol would prefer to rely on those existing measures rather than see potential distress and harm being caused to individual blood donors.

Those who will make the final decision have a very difficult choice to make – Nr Nicol said that we ask a lot from donors and asked if we can ask a society that their generosity be extended to submit to an unreliable test that could result in such anguish for so many?

Statements from family members of those affected by variant CJD

Two relatives of individuals affected by vCJD spoke.

Mr Peter Buckland spoke on behalf of his family. His son died after contracting vCJD from contaminated blood. Mr Buckland made the following points:

• The devastating effect of vCJD on the families of those affected was huge.

• The tragic consequences of the disease have been made more acute by a lack of information available to his family at the time from government institutions.

• The processes around management of vCJD should be open and honest, and those responsible should be prepared to explain their decisions.

• Delays in notifying those at risk of the disease did those who went on to develop vCJD a great disservice. Lives would have been led differently if the future implications had been made clear.

• An earlier notification would at the very least have allowed families to explore potential treatments for the disease in the early stages.

• Any test for vCJD should be made available. It is not acceptable that “at-risk” individuals be kept in the dark, however low that risk may be.

Ms Christine Lord is the mother of Andrew Black, who died from vCJD in 2007 aged 24, and made the following points:

• Those making the decisions around management of vCJD should be supervised by independent sources that are not government funded or backed.

• The terrible risk and legacy of vCJD that the UK population now face should lay clearly at the door of those who Ms Lord considers responsible for her son’s death. Ms Lord believes that if those ministers and officials in the 1980s and 1990s had not put profit before lives vCJD would have never existed and SaBTO would not be faced with the terrible dilemmas it now has to wrestle with.

• Testing should be made available as soon as it is developed. Ms Lord believes that it may not be in the Government’s best interest to make such a test available. The doubts about prevalence mean that the likely number of positives is not known; ministers may be reluctant to allow the wider public to be made aware of the true prevalence of vCJD in the population, particularly if the situation was worse than is currently forecast.

• Testing for vCJD should be a personal choice allowing people to live their lives accordingly. Ms Lord believes that officials in the 1980s and 1990s played god with the UK populations lives resulting in vCJD and too many innocent people dying needlessly and she is mindful that government may very well be playing god again by refusing the UK population the choice to test or not.

• As a mother who has lost her son to vCJD Ms Lord would take a test tomorrow and would like the opportunity to be able to do this in the near future.

• The devastation vCJD has wrecked on families, careers, futures and relationships cannot be described in mere words. As a bereaved mother, a qualified journalist and psychological counsellor Ms Lord is acutely aware of the huge impact and life scarring event that nursing a child through the most horrific disease has had on hundreds of family members and thousands of colleagues and friends. Ms Lord’s website is www.justice4andy.com.

Summary of questions/open forum

Q. What developments have there been since 2004 aimed at reducing the risk of vCJD transmission by blood transfusion?

SaBTO is considering other potential options for mitigating the risk of vCJD, including importation of red cells for the children, double-dose red cell collection, extension of platelet apheresis and importation of plasma for all recipients.Several manufacturers are developing filters aiming to remove prion protein from red cells for transfusion. One of these filters is CE marked which means that it can legally be used in the UK. This filter is under active assessment by the UK and Irish Blood Services. It is important that any new technology used to produce blood components is assessed to make sure that it is effective and will not cause any harm to patients. A pathway for assessing prion removal filters has been established by the UK Blood Services and endorsed by SaBTO. The Spongiform Encephalopathy Advisory Committee (SEAC) have advised that the UK Blood Services should obtain an independent assessment of the ability of these filters to remove prion protein. The first of these studies is well underway with early results expected to be reported in 2009. The UK Blood Services are also undertaking a clinical study of prion-filtered red cells in surgical patients and then transfusion dependent patients, designed to assess whether the filter results in an increase in adverse events to patients. These clinical studies were endorsed by SaBTO’s predecessor, the Advisory Committee on the Microbiological Safety of Blood, Tissues and Organs (MSBTO). SaBTO will review this subject further in spring/summer 2009 when data from the independent evaluation and clinical studies of prion-filters are likely to become available.

Q. What are the panel’s views on the potential for embryonic stem cells to be used to produce blood components for transfusion?

Although there has been considerable progress in this area, we still have a lot to learn about how the growth of stem cells is controlled, and also how to produce specific cell types from stem cells. There are significant challenges in being able to produce sufficient amounts of cells for transfusion or transplantation from stem cells and it is likely to be some years before such cells can be used clinically.

Q. How long is the incubation period for vCJD transmitted by blood transfusion?

We do not know, but in the 4 cases where vCJD infection is thought to have been transmitted by blood transfusion, the incubation period between receiving the implicated transfusion and development of symptoms of disease was 6-8 years in the 3 symptomatic cases. In animals, the incubation period following infection can be influenced by genetic factors and in other human prion diseases, such as Kuru, the incubation period can be up to 40 years. It is possible, therefore, that some people have been infected but have not yet (and maybe will never) develop clinical disease.

Q. When is a test for vCJD in blood likely to be available?

There is currently no validated diagnostic test that can be used to determine whether blood is infected with vCJD. Several companies are developing tests for vCJD in blood and are making good progress. As for prion removal filters the UK Blood Services have developed a pathway for assessing tests that may be applied to blood donors. We currently do not know how accurate these tests will prove to be. There are concerns around telling asymptomatic people, for example blood donors, that they may be infected with vCJD when the significance of the test result is uncertain, when it is unknown whether infection would necessarily result in disease, and when there is no proven treatment.The impact of a screening test on the blood and tissue supply could be profound depending on how accurate the test is due to the direct loss of donations due to false positive results and the indirect impact in deterring people from donating., In addition there would be a need to carry out lookback studies and both donors and past recipients may need to be designated as “at risk of vCJD for public health purposes” leading to significant impact on the wider NHS.It was noted that the development of a diagnostic test for vCJD was highly desirable for groups of patients who have been identified as ‘at risk from vCJD for public health purposes’. However, SaBTO’s remit is restricted to consideration of tests in the context of blood, tissue and organ donation.

Q. With regards to screening tests for vCJD in blood, what level of specificity and sensitivity do SaBTO regard as acceptable?

Currently the UK Blood Services screen blood for several viruses. Donors that have a positive result then undergo further testing with one or more confirmatory tests before being informed that they are positive. The acceptable performance of a vCJD screening test would depend, in part therefore, on whether a secondary screening test or confirmatory test was available.

Q. Why is blood labelled "Risk of adverse reaction/infection, including vCJD" but other infectious agents such as HIV not included on the label? Why is plasma that is imported not labelled the same way?

The labelling of blood components has been changed to bring it in line with labelling of tissue products. Other infectious agents such as HIV do not appear on the label as blood donations are currently tested for these; there is no screening test for vCJD available. Plasma is imported from a country with low risk of vCJD and therefore not labelled in the same way as blood components from the UK. SaBTO are currently examining the possibility of recommending full written informed consent for transfusion, which would include information on the risks involved and whether there are any suitable alternatives.

Q. Why aren’t platelets imported? Is importation of red cells feasible?

Plasma components have a 2 year shelf-life and can be transported frozen. Platelets have a shelf-life of 5 days and must also be transported and stored in a very specific way to preserve them. It would therefore be highly unlikely that sufficient platelets could be imported from outside the UK with these limitations. NHS Blood & Transplant are currently conducting a feasibility study to assess options for importing red cells. Importing red cells for all patients in the UK will not be possible (over 2 million units of red cells per year are required). It may be possible to import red cells for selected patient groups, for example for children. Importation of special red cell products with a short-shelf life of 5 days or less is not likely to be feasible however. It is important to also consider other risks that may be increased in possible source countries, such as viral risk, since systems are not available to treat red cells to kill viruses.
Q. If a test for vCJD is available, should it be used to screen egg and sperm donors?

So far SaBTO have only considered the possible use of a vCJD screening test for blood donors. In due course the committee will also have to consider the application of such a test to donors of tissues, organs and gametes.

Q. Would prion filtration prevent the need to import red cells? And unlike screening for vCJD, prion-filtration would not have any negative impact on the donor.

Prion-filtration or importation of red cells would reduce the cost-effectiveness of the other. SaBTO are considering a number of possible options to reduce the risk of vCJD transmission by blood. The cost-effectiveness and advantages/disadvantages of each option have been considered at the April and July 2008 meetings of SaBTO. The committee will be considering these measures further in 2009 when further data on prion filtration and testing are available.

Q. Has there been a case of vCJD transmission by blood transfusion of leucocyte depleted blood?

No. So far all 4 possible transmissions of infected prion protein have all been from non-leucocyte depleted red cells and there have been no known infections from blood since this time. Leucocyte depletion was implemented in the UK in 1998/1999. Animal studies suggest that leucocyte depletion only removes about 40-50% of infectivity in blood. We also do not know what the maximum period of incubation between transfusion of infected blood and development of vCJD could be, and therefore leucocyte-depleted blood could be capable of transmitting infection but there may not have been sufficient time for any of the recipients to develop clinical disease as yet.

Q. Have SaBTO sought advice from the Association of British Insurers regarding vCJD tests?
The Association of British Insurers have been consulted previously with regard to patients who have developed CJD following treatment with growth hormones. Their response indicated that such recipients would not have any issues relating to insurance policies. SaBTO will obtain further information from the Association of British Insurers when more information is available on the performance of vCJD screening tests.

Q. SaBTO requested a feasibility study on the use of double-dose red cells at their July meeting. When will this be available?

NHS Blood & Transplant have been asked to perform a feasibility study on the use of double dose red cells for selected patient groups. This will be presented to the committee in Spring 2009 along side other options for risk-reduction of vCJD by transfusion.

Q. Why does SaBTO still use estimates of prevalence from the Hilton study published in 2004 and not the later National Anonymised Tonsil Archive (NATA) study?

This has been reviewed by the Spongiform Encephalopathy Advisory Committee (SEAC) not SaBTO. The Hilton study was on both appendices and tonsils (but mainly appendices). There are differences between the studies in terms of the tissue studied, the period in time the study was performed since the initial BSE epidemic and the sensitivity of test methods used. SEAC therefore do not consider it to be appropriate to combine data from the two studies. The estimates of prevalence from the two studies are currently consistent with each other, and the confidence intervals of the NATA study are within those of the Hilton study.

At the end of the meeting it was generally agreed that this exercise had been very useful – both to spread important information on this issue and also to open dialogue with all those involved. SaBTO intend to hold a similar public meeting next year.


http://www.advisorybodies.doh.gov.uk/acsbto/SaBTOPublicMeeting2008-Summary.pdf



Worries over test for mad cow disease

Published: Tuesday, 21-Oct-2008 Printer Friendly Email to a Friend

Disease/Infection News

A new test to screen blood for the incurable human form of mad cow disease could be available within 18 months, but it has raised concerns. The breakthrough blood test which will be able to diagnose variant Creutzfeldt-Jakob diseases (vCJD), is currently undergoing clinical trials but experts are worried it will reduce the number of people prepared to donate blood - research suggests 1 in every 4,000 people might harbour vCJD in their blood, though 95% of them may never actually develop the full blown disease.

Variant CJD is a rare and fatal human neurodegenerative condition and is a Transmissible Spongiform Encephalopathy (TSE) or prion disease - because of the characteristic spongy degeneration of the brain - it is strongly linked to exposure, probably through food, to a TSE of cattle called Bovine Spongiform Encephalopathy (BSE).

In the early stages patients usually experience psychiatric symptoms such as depression or, less often, a schizophrenia-like psychosis.

Unusual sensory symptoms, such as "stickiness" of the skin, have been experienced by half of the cases early in the illness and neurological signs, including unsteadiness, difficulty walking and involuntary movements, which develop as the illness progresses; by the time of death, patients become completely immobile and mute.

There are at present no available, completely reliable diagnostic tests for use before the onset of clinical symptoms, but magnetic resonance scans, tonsillar biopsy and cerebrospinal fluid tests are useful for detection.

The highest incidence of vCJD is in the UK, the country with the largest potential exposure to BSE. A statutory ban on the feeding of protein derived from ruminants (e.g. cattle, sheep and goats) to any ruminant exists.

The use in the food chain of bovine offals thought to pose a potential risk to humans was also banned in the UK in 1989.

According to advisers to the British government, though the test is undoubtedly a significant step towards eliminating the incurable disease and preventing it from becoming endemic in society, it could result in a reduction in the number of blood donors and there are also fears it could increase insurance premiums.

Experts suspect that donors will be reluctant to give blood if they risk being told that they have the possibility of developing the disease which causes a horrible and agonising death.

Dr. John Forsythe, chair of the Advisory Committee on the Safety of Blood Tissues and Organs (SABTO) and a transplant surgeon at the Royal Infirmary of Edinburgh says the test does have significant downsides, despite concerns that the disease could become widespread in the UK.

However only 4 of the 167 people who have died from vCJD contacted it through infected blood - but the knowledge of having the disease would be terrifying prospect.

The problem is compounded because around 1% of the positive tests could be wrong and with two million people donating blood every year in Britain that could amount to 250 people being told they had the infection, and up to four of them being falsely diagnosed.

Experts say from both a legal and ethical standpoint, those incubating the disease would have to be told even though only a few could develop it fully.

In 2004 vCJD had an impact on blood supplies when the number of blood donors dropped by 52,000 when those who had received blood transfusions in the previous two decades was banned from donating blood.

Currently donated blood is screened for HIV, syphilis and hepatitis B and C, but because vCJD is neither curable or treatable, many donors may prefer to not know they could develop the progressive and fatal degenerative brain disease.

Presently vCJD is tested for by performing post mortem biopsies on the brain.

http://www.news-medical.net/?id=42079



SEAC

ONE HUNDRED AND FIRST MEETING OF THE SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE

The Spongiform Encephalopathy Advisory Committee held its 101st meeting in London on 15th October 2008, and discussed the following:

CURRENT ISSUES SEAC was informed about:

. A mother and son in Spain who had died of variant Creutzfeldt-Jakob Disease (vCJD). This is the first recorded instance of more than one case of vCJD within one family. Both the mother and son lived in a region of Spain with a history of BSE and had frequently shared meals of cattle brain. As no other risk factor has been identified, it seems most likely that both infections were acquired from dietary exposure.

. Results of tests on a single goat from a culled UK dairy herd with a large classical scrapie outbreak. On the basis of the results the presence of Bovine Spongiform Encephalopathy (BSE) cannot be excluded. Further testing by mouse bioassays, which may take at least two, if not more, years to complete, is required to make a definitive diagnosis.

UPDATE ON vCJD PREVALENCE STUDIES

SEAC was updated by the Health Protection Agency (HPA) about the progress of the National Anonymous Tonsil Archive (NATA), a proposed second retrospective survey of 30 000 stored appendix samples and a proposed post mortem tissue archive. These studies would provide data to estimate the prevalence of subclinical vCJD (vCJD infections that have yet to develop, or may never develop, into clinical disease).

Around 62 500 tonsil samples collected by NATA have been tested with no positive samples found. An application for the second retrospective survey of appendix samples is currently under consideration by a Research Ethics Committee. SEAC learned that the establishment of a post mortem tissue archive, which is dependent on the collection of samples from Coroners' autopsies, does not have the support of Coroners needed to take it forward. SEAC is extremely disappointed about the lack of support from Coroners for the post mortem tissue archive. As SEAC has repeatedly stated, the archive is key to obtaining better estimates of the prevalence of subclinical vCJD. These estimates are vital to make meaningful assessments of the risks to public health from vCJD and of the effectiveness of current, and the need for further, very costly public health protection measures. SEAC acknowledged the strenuous efforts made by the HPA, the Department of Health (DH) and National Health Service Blood and Tissue to devise a system to collect samples that would have the least impact on the work of Coroners. SEAC remains strongly in favour of establishing the archive.

snip... see;

http://www.seac.gov.uk/papers/101-1.pdf



PROTEASE SENSITIVE PRIONOPATHY

SEAC discussed with Dr Pierluigi Gambetti (US National Prion Disease Pathology Surveillance Center) his recently published report5 on the identification in the United States of America of a new human prion disease. SEAC agreed that there is considerable work to be done to characterise fully this new disease, its cause and whether it is infectious or not. As preliminary unpublished data were also presented, this issue was discussed in a reserved business session in accordance with the SEAC Code of Practice.

RESULTS ON HUMAN SCLERA

SEAC considered preliminary results provided by the HPA and National CJD Surveillance Unit from tests on eye tissue (sclera) from a vCJD case. The results suggest the presence of infectivity and, in contrast with previous testing of samples from other vCJD cases, abnormal prion protein in this tissue. However, as the sclera is very difficult to remove from surrounding eye tissues, which are themselves known to carry vCJD infectivity, the findings may have arisen as a result of contamination at autopsy. Nevertheless, even if the data are reliable, they indicate that there may only be a relatively low level of infectivity present in sclera. As preliminary unpublished data were considered, this issue was discussed in a reserved business session in accordance with the SEAC Code of Practice.

5 Gambetti et al. (2008) A novel human disease with abnormal prion protein sensitive to protease. Ann. Neurol. 63, 697-708. 5 © SEAC 2008

SEE FULL TEXT ;

http://www.seac.gov.uk/papers/101-summary.pdf


http://www.mad-cow.org/dec99_news.html#bbb


http://creutzfeldt-jakob-disease.blogspot.com/2008/01/cjd-hgh-body-snatchers.html


5.5 There is now convincing evidence of human to human transmission of vCJD via blood transfusion with 3 clinical cases of the disease and one of sub-clinical infection believed to have been transmitted via this route. However, in humans little is known about the level, distribution and temporal development of infectivity in blood. Estimates of prevalence of asymptomatic infection in the UK population remain uncertain, as does the susceptibility of recipients to infection. 5.6 To assess the cost-effectiveness of future measures to reduce the risk of vCJD by blood components 8 scenarios relating to prevalence, susceptibility and infectivity were modelled: a prevalence of 1:20,000 (LOW) and1:4000 (HIGH), infectivity of 0.1 ID/ml (LOW) and 30 ID/ml (HIGH), and susceptibility of recipients to development of clinical disease of 10% (LOW) and 100% (HIGH). It was noted that the high susceptibility scenario is not consistent with the observed number of clinical cases. It was noted that SEAC reviewed data available to date from The National Anonymised Tonsil Archive (NATA) Study at their meeting on 25th April 2008 and has not revised its estimate of prevalence of sub-clinical infection as a result.

http://www.advisorybodies.doh.gov.uk/acsbto/2nd_meeting_minutes_290408.pdf


these minutes are not available yet ;

1st Public Meeting of SaBTO (Advisory Committee on the Safety of Blood, Tissues and Organs)

Tuesday 21st October 2008, 2pm-4pm

http://www.advisorybodies.doh.gov.uk/acsbto/Public_Meeting-21_October_2008.htm


vCJD case study highlights blood transfusion risk Friday 8th December 2006

http://www.mrc.ac.uk/consumption/groups/public/documents/content/mrc003431.pdf


Public release date: 29-Aug-2008

Contact: Tara Womersley mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000179/!x-usc:mailto:tara.womersley@ed.ac.uk 44-131-650-9836 University of Edinburgh

Study confirms vCJD could be transmitted by blood transfusion A 9-year study in sheep has added to the evidence that vCJD can be transmitted through blood transfusion in humans The findings underline the importance of precautions against vCJD transmission, such as the Government decision in 2004 to ban blood donations from anyone who had received a blood transfusion since 1980.

The study published in Blood, the journal of the American Society of Hematology, looked at BSE transmission between sheep through infected blood with the aim of quantifying how vCJD - the human form of BSE - could be spread through transfusions.

Researchers (Fiona Houston, Nora Hunter and colleagues) at the Neuropathogenesis Unit at the Institute of Animal Health, which is now part of The Roslin Institute, University of Edinburgh, found that the likelihood of BSE being transmitted between sheep through transfusion of infected sheep blood was 36 per cent, with rates of 43 per cent found for scrapie.

Fiona Houston, now at the University of Glasgow, who led the research, said: "It is apparent that the stage of disease incubation in infected donors played a large role in the likelihood of transmission. The longer that BSE or scrapie had been carried by donors, the greater the likelihood of the disease being transmitted with transfusions of infected blood."

While cases of vCJD are tailing off there are concerns that up to 4,000 people could be carrying the disease in the UK, which could then be transmitted through infected blood causing further infections.

Scientists are working to develop a test for vCJD that can be used before symptoms develop and a filter is also being trialled to remove prions – infective proteins – from donated blood.

Dr Houston said: "The study shows that, for sheep infected with BSE or scrapie, transmission rates via blood transfusion can be high, particularly when donors are in the later stages of infection. This suggests that blood transfusion represents an efficient route of transmission for these diseases and justifies the current control measures put in place to safeguard human blood supplies.

"While it may not correlate directly to what happens in the human population, due to factors such as species differences in genetic susceptibility to disease, it provides greater insight into the role of how vCJD may be carried through infected blood. By understanding how vCJD can be transmitted through blood transfusions, we can ensure the most effective control measures to minimise human to human infection."

BSE is one of a group of rare neurodegenerative disorders called transmissible spongiform encephalopathies (TSEs), which include scrapie and vCJD. Of 22 sheep that received BSE infected blood, eight showed evidence of infection. Nine out of 21 sheep receiving scrapie-infected blood developed the disease.

To date 167 cases of vCJD have been recorded in the United Kingdom, of which three patients are thought to have received vCJD through infected blood.

### Tara Womersley, Press and PR office, University of Edinburgh, Tel 0131 650 9836 or 07791 355 804 Email: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000179/!x-usc:mailto:Tara.womersley@ed.ac.uk


The University of Edinburgh is a charitable body, registered in Scotland, with registration number SC005336.

http://www.eurekalert.org/pub_releases/2008-08/uoe-scv082908.php


PLEASE REMEMBER ALSO ;

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://creutzfeldt-jakob-disease.blogspot.com/2008/10/cjd-foundation-update.html



----- Original Message -----
From: Terry S. Singeltary Sr.
To: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000179/!x-usc:mailto:FREAS@CBER.FDA.GOVCc: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000179/!x-usc:mailto:william.freas@fda.hhs.gov ; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000179/!x-usc:mailto:rosanna.harvey@fda.hhs.gov
Sent: Wednesday, November 29, 2006 1:24 PM
Subject: TSE advisory committee for the meeting December 15, 2006 [TSSSUBMISSION]

November 29, 2006

Greetings FDA, DHH, Dr. Freas, and Dr. Harvey et al, a kind and warm Holiday Greetings to you all. i kindly wish to submit the following to the TSE advisory committee for the meeting December 15, 2006, about the assessment for potential exposure to vCJD in human plasma-derived antihemophilic factor (FVIII) products manufactured from U.S. plasma donors and related communication material ;


http://a257.g.akamaitech.net/7/257/2422/01jan20061800/edocket.access.gpo.gov/2006/E6-20251.htm



i see the media picked up on this as a 'low risk', from what the gov. agency perceived to be to them;


http://www.newsday.com/news/health/ats-ap_health14nov27,0,7955259.story?coll=ny-leadhealthnews-headlines



however, i seem to disagree. from my primitive ciphering, i see it another way. this is a huge catastrophic risk. 3 in 160 is 1.9%. so call that 2% which is 1 in 50 or twenty per thousand or 20,000 per million. also, what about the mixed genotypes/mixed susceptibility? what about the silent carriers that donated tainted blood? what about the sporadic CJDs of UNKNOWN strain or phenotype? this risk assessment is just more BSe to me. just another in a long line of industry fed crap. i pray that my assessment is the one that is wrong. but it is THEY who roll the dice with your life. it is THEY who refuse to regulate an industry that has run amok. just from a recall aspect of potentially tainted blood, and these are just recent recalls ;


PRODUCTSource Plasma, Recall # B-0054-7CODEUnits: 03MMNC5465, 03MMNC6361, 03MMNC6801, 03MMNC7510, 03MMNC7891,03MMNC8252, 03MMNC8801, 03MMNC9144, 03MMND1122, 03MMND1478, 03MMND1969,03MMND2350, 03MMND2825, 03MMND3211, 03MMND3708, 03MMND4072, 03MMND4588,03MMND4831, 03MMND5320, 03MMND5719, 03MMND6268, 03MMND6683, 03MMND7228,03MMND7656, 03MMND8211, 03MMND8652, 03MMND9195, 03MMND9618, 03MMNE0628,03MMNE0884, 03MMNE1597, 03MMNE1979, 03MMNE2644, 03MMNE3064, 03MMNE3707,03MMNE4122, 03MMNE4750, 03MMNE5080, 03MMNE5876, 03MMNE6218, 03MMNE7189,03MMNE7587, 03MMNE8027, 03MMNE8645, 03MMNE9029, 03MMNE9641, 03MMNE9979,03MMNF0491, 03MMNF0685, 03MMNF0937, 03MMNF1260, 04MMNA0351, 04MMNA0707,04MMNA1241, 04MMNA1650, 04MMNA2291, 04MMNA2646, 04MMNA3340, 04MMNA3719,04MMNA4312, 04MMNA4683, 04MMNA5298, 04MMNA5750, 04MMNA6407, 04MMNA6816,04MMNA7482, 04MMNA7915, 04MMNA8632, 04MMNA9076, 04MMNA9723, 04MMNB0063,04MMNB0696, 04MMNB1100, 04MMNB1845, 04MMNB2285, 04MMNB3035, 04MMNB3485,04MMNB4213, 04MMNB4672, 04MMNB5841, 04MMNB6652, 04MMNB7162, 04MMNB7930,04MMNB8453, 04MMNB9239, 04MMNB9747, 04MMNC0456, 04MMNC0931, 04MMNC1578RECALLING FIRM/MANUFACTURERBioLife Plasma Services, L.P., Mankato, MN, by facsimile on June 4, 2004.Firm initiated recall is complete.REASONBlood products, collected from a donor who was at increased risk for newvariant Creutzfeldt-Jakob Disease (nvCJD), were distributed.VOLUME OF PRODUCT IN COMMERCE89 unitsDISTRIBUTIONCA and AustriaEND OF ENFORCEMENT REPORT FOR October 25, 2006###http://www.fda.gov/bbs/topics/enforce/2006/ENF00975.htmlUSA FDA MAD COW BLOOD HUMANS RECALL (these are dime a dozen)RECALLS AND FIELD CORRECTIONS: BIOLOGICS -- CLASS II______________________________PRODUCTSource Plasma, Recall # B-1708-6CODEUnits: MI180733, MI180927, MI181625, MI181780, MI182337, MI182519, MI183140,MI183311, MI183955, MI185006, MI185278, MI185822, MI186081, MI186855,MI187183, MI187903, MI188273, MI188695, MI189257, MI189553, MI190136,MI190473, MI191073, MI191395, MI191972, MI192303, MI193473, MI194343,04MINA0377, 04MINA0801, 05MINA7147, 05MINA7451, 05MINA8094, 05MINA8504,05MINA9548, 05MINA9883, 05MINB0489, 05MINB0875, 05MINB1469, 05MINB1874,05MINB3116, 05MINB7192, 05MINB7529, 05MINB8246, 05MINB8612, 05MINB9236,05MINB9366, 05MINB9475, 05MINB9641, 05MINC0031, 05MINC0237, 05MINC0336,05MINC0894, 05MINC0964, 05MINC1138, 05MINC1619, 05MINC1750, 05MINC1907,05MINC1977, 05MINC2375, 05MINC2774, 05MINC3113, 05MINC3484, 05MINC4277,05MINC4623, 05MINC5623, 05MINC5914, 05MINC7545, 05MINC7870, 05MINC8355,05MINC8689, 05MINC9437, 05MINC9775, 05MIND0067, 05MIND0393, 05MIND0892,05MIND0951, 05MIND1836, 05MIND2183 and 05MIND2962RECALLING FIRM/MANUFACTURERBioLife Plasma Services L.P., Muncie, IN, by facsimile on November 22, 2005.Firm initiated recall is complete.REASONBlood products, collected from unsuitable donors based on risk factors forCreutzfeldt-Jakob Disease (CJD), were distributed.VOLUME OF PRODUCT IN COMMERCE80 unitsDISTRIBUTIONCA, NC, and MD______________________________PRODUCTa) Red Blood Cells, Leukocytes Reduced, Recall # B-1714-6;b) Fresh Frozen Plasma, Recall # B-1715-6;c) Platelets, Recall # B-1716-6CODEa), b), and c) Unit: 2443732RECALLING FIRM/MANUFACTURERSouth Texas Blood and Tissue Center, San Antonio, TX, by letters datedNovember 11, 2003 and December 18, 2003. Firm initiated recall is complete.REASONBlood products, co lected from a donor who was at increased risk for newvariant Creutzfeldt-Jakob Disease (nvCJD), were distributed.VOLUME OF PRODUCT IN COMMERCE3 unitsDISTRIBUTIONTX and WIEND OF ENFORCEMENT REPORT FOR SEPTEMBER 13, 2006###http://www.fda.gov/bbs/topics/enforce/2006/ENF00969.htmlPRODUCTFresh Frozen Plasma, Recall # B-1751-6CODEUnit: 4936623RECALLING FIRM/MANUFACTURERGulf Coast Regional Blood Center, Houston, TX, by facsimile dated September16, 2005. Firm initiated recall is complete.REASONBlood product, which was collected from an unsuitable donor based on riskfactors for variant Creutzfeldt-Jakob Disease (vCJD), was distributed.VOLUME OF PRODUCT IN COMMERCE1 unitDISTRIBUTIONTXEND OF ENFORCEMENT REPORT FOR SEPTEMBER 6, 2006###http://ww .fda.gov/bbs/topics/enforce/2006/ENF00968.htmlMon Aug 7, 2006 10:2471.248.132.189PRODUCTa) Red Blood Cells, Recall # B-1587-6;b) Cryoprecipitated AHF, Recall # B-1588-6;c) Recovered Plasma, Recal # B-1589-6CODEa), b) and c) Unit: 2016719RECALLING FIRM/MANUFACTURERWalter Shepeard Community Blood Center, Inc., Augusta, GA, by facsimile onMarch 13, 2003. Firm initiated recall is complete.REASONBlood products, which were collected from a donor who may be at increasedrisk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.VOLUME OF PRODUCT IN COMMERCE3 unitsDISTRIBUTIONGA and Germany______________________________PRODUCTa) Red Blood Cells Leukocytes Reduced, Recall # B-1590-6;b) Fresh Frozen Plasma, Recall # B-1591-6CODEa) and b) Unit: 2443595RECALLING FIRM/MANUFACTURERSouth Texas Blood and Tissue Center, San Antonio, TX, by facsimile on June30, 2004. Firm initiated recall is complete.REASONBlood products, which were collected from a donor who may be at increasedrisk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.VOLUME OF PRODUCT IN COMMERCE2 unitsDISTRIBUTIONTX______________________________PRODUCTa) Red Blood Cells Leukocytes Reduced, Recall # B-1592-6;b) Fresh Frozen Plasma, Recall # B-1593-6CODEa) and b) Unit: 2545596RECALLING FIRM/MANUFACTURERSouth Texas Blood and Tissue Center, San Antonio, TX, by facsimile onDecember 14, 2004 and January 3, 2005. Firm initiated recall is complete.REASONBlood products, which were collected from a donor who may be at increasedrisk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.VOLUME OF PRODUCT IN COMMERCE2 unitsDISTRIBUTIONTX______________________________http://www.fda.gov/bbs/topics/enforce/2006/ENF00963.html

SNIP...FULL TEXT ;


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



http://creutzfeldt-jakob-disease.blogspot.com/2008/10/cber-2007-annual-report-assessing.html



http://www.fda.gov/OHRMS/DOCKETS/ac/06/transcripts/2006-4271t1.pdf



Friday, October 24, 2008

CBER 2007 Annual Report Assessing the Potential Risk of variant Creutzfeldt-Jakob Disease from Blood Products

http://creutzfeldt-jakob-disease.blogspot.com/2008/10/cber-2007-annual-report-assessing.html



Docket APHIS-2006-0041 Docket Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived from Bovines Commodities Docket Type Rulemaking Document APHIS-2006-0041-0001 Document Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived From Bovines Public Submission APHIS-2006-0041-0006.1 Public Submission Title Attachment to Singletary comment



http://www.regulations.gov/fdmspublic/ContentViewer?objectId=09000064801f3412&disposition=attachment&contentType=crtext





http://www.regulations.gov/fdmspublic/component/main?main=DocumentDetail&d=APHIS-2006-0041-0006.1





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





TSS

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Sunday, August 24, 2008

Blood scandal victims speak out Infected with HIV, about 5,700 in Britain were 'used as lab rats'

Blood scandal victims speak out Infected with HIV, about 5,700 in Britain were 'used as lab rats'

By GREGORY KATZ Associated Press Aug. 23, 2008, 7:17PM


PEEBLES, SCOTLAND — Robert Mackie trembles with rage when he describes how he and his wife were kept in the dark about his HIV infection — and how doctors published his medical data in journals years before they gave him the devastating news.

Mackie is one of about 5,700 British hemophiliacs who received tainted blood and were infected with HIV, hepatitis or both, in what has been viewed as one of the worst treatment disasters in the history of Britain's heath care system. Nearly a third have since died.

Tainted blood scandals have been investigated throughout the world, leading to some convictions of health officials and many compensation packages for infected hemophiliacs, but there has been no detailed probe in Britain until now. One inquiry under way will likely end in a nonbinding report, while the other is an official investigation by the Scottish government that could lead to charges filed against individuals.

"They used me as a guinea pig," said Mackie, 58, in his house in Scotland. "It's just a miracle my wife wasn't infected."

An inherited disorder Hemophiliacs suffer from an inherited disorder that prevents blood from clotting. Mackie — an active sportsman who had hoped to become a salmon fishing guide — had controlled his hemophilia with a treatment called cryoprecipitate when he switched in 1980 to a new product. Called Factor VIII, it was supposed to be more effective in helping his blood clot.

In 1983, he heard hemophiliacs were developing AIDS, then a mysterious disease that usually claimed its victims in two or three years.

He said he asked his doctors if he could be exposed to the killer virus through his use of Factor VIII, a relatively new blood plasma product made from blood collected from thousands of donors.

They told him not to worry. A year later, he was infected by a contaminated batch.

"We could have had more of a family," says Alice Mackie, who had a son with Robert before he became infected. "The two of us had plans for what we were going to do. But you could say our whole lives stopped."

The tainted blood led to the deaths of Mackie's cousin, two uncles and friends, who were part of a close-knit community of hemophiliacs in Scotland.

"From '87, all we saw was people dying," said Alice, her hair white at 51. "And believe me, when you see someone dying of AIDS, it's really bad."

No consent for study Mackie said he was told of his infection in 1987. But he told an independent inquiry commission that when he finally obtained his medical records, he learned he had been used for an AIDS study that began several years before then.

Mackie said that in 1985 — when he was already infected but didn't know it — his physician, Dr. Christopher Ludlam, wrote to government authorities seeking ethics approval to study the immune system of infected patients and claimed that his patients knew about the research and had agreed to participate.

"If, as the ethics application form states, consent was obtained from all subjects ... how is it that I did not know about my AIDS status until 1987?" he said at the hearing. "I did not know anything about his studies or research."

Ludlam, who practices at the Royal Infirmary of Edinburgh, declined to talk with The Associated Press about the case.

Brian Montgomery, a National Health Service executive who oversees the hospital, said it would be "inappropriate" to comment while the inquiries are ongoing.

New medicine helps Surprisingly, Mackie stayed relatively healthy for a decade. He thought he had escaped a death sentence, but in 1997 his appetite began to wane. By 2000, he had advanced symptoms of AIDS.

He became too weak to climb stairs. The smell of food sickened him. Doctors said he had a few weeks left, but he was too stubborn, and too suspicious about doctors, to take the new anti-retroviral drugs that were by then extending the lives of many AIDS patients.

For days he sat, feverish, in his kitchen, believing death was imminent.

Then, drawing on reserves he did not know he possessed, his fighting spirit returned. He gave in to Alice's pleas and started to take the new drugs after she convinced him they were not poison.

The drugs worked. Mackie said they at first caused a dangerous reaction that left him "out of his head" but eventually gave him more energy and confidence.

Despite being weak from AIDS and Hepatitis C, which he found out he had in 2000, Mackie insisted on giving evidence to the Archer committee last year. The hearings were closed to the public but a report is expected next month.

Alice read most of his statement, and he spoke quietly when he spoke at all, but he did raise his voice at one point to tell the committee that doctors had endangered the safety of his wife and son by holding back his HIV status.

"I believe nonconsensual research was conducted by doctors of hemophilia in this country," he said, voice booming again. "We were all used as lab rats."


http://www.chron.com/disp/story.mpl/world/5962519.html



4th case of variant CJD infection associated with blood transfusion 18 January 2007

A new case of variant-Creutzfeldt-Jakob disease (vCJD ) associated with a blood transfusion has recently been diagnosed.

This latest patient has been diagnosed with vCJD about nine years after receiving a blood transfusion from a donor who later went on to develop vCJD. A transfusion from the same blood donor was also associated with one of the previously identified cases. The patient is still alive and is under specialist care.

This fourth case of vCJD infection associated with blood transfusion increases the concern about the risk of vCJD transmission between humans via blood transfusion. All four cases relate to the transfusion of blood components: no cases have been reported relating to treatment with plasma products.

The patient is one of a small number (less than 30) of living individuals who are known to have received a blood transfusion in the UK from a donor who later developed vCJD. All these individuals have previously been informed of their potential exposure to vCJD and asked to take certain precautions to reduce the chance of passing on vCJD to other people via healthcare procedures, such as surgery.

The Health Protection Agency has been in contact with doctors caring for the other patients who have been exposed to blood transfusions from donors who later developed vCJD. This is to ensure that they are informed of this new development and provide access to the latest information and specialist advice about their risk due to blood transfusion.

Professor Peter Borriello, Director of the HPA's Centre for Infections said, "This new case of vCJD infection increases our concern about the risk to the small group of people who had blood transfusions from donors who unknowingly at the time of donation must have had vCJD infection. However, this new case does not change our understanding of the risk for other people in any specific way. It does however reinforce the importance of the precautions that have already been taken to reduce the risk of transmission of vCJD infection by blood."

Dr Angela Robinson, Medical Director of NHS Blood and Transplant said, "Blood transfusions are often given to save or prolong the life of patients who are very ill and the benefit of receiving a transfusion when needed must always be balanced against any possible risk. Nonetheless, our primary concern is the safety of our patients through maintaining the quality of blood used for medical treatment. Since 1997, the NBS has introduced a range of precautionary measures against the risk of vCJD."

vCJD is a rare disease, and less than 2% of the vCJD cases reported to date in the UK have been associated with blood transfusion.

Notes to Editors:

To date, there have been 66 people identified in the UK who have received vCJD implicated blood transfusions. The transfusions received by these 66 individuals were donated by eighteen different donors who were diagnosed with vCJD after their blood donation. Of these 66 people, 40 have died of illnesses other than vCJD, including one patient who was found to have evidence of vCJD in parts of their body after their death. Including the new (4th) case, 3 of these people who have received vCJD implicated blood transfusions have developed symptoms of vCJD. There are 23 people who have received vCJD implicated blood transfusions who are alive and have not been diagnosed with vCJD.

The identification of cases of variant-CJD associated with blood transfusion has depended on the Transfusion Medicine Epidemiology Review, a collaborative study between the National Blood Services, the National CJD Surveillance Unit and the Office of National Statistics. For further information about this study see Hewitt et al Creutzfeldt-Jakob disease and blood transfusion: results of the UK Transfusion Epidemiology Review study. Vox Sanguinis 2006 91:221-230.

'Blood Transfusion' means transfusion with labile blood components (e.g. red cells, platelets, fresh frozen plasma). This latest case (and the previous three referred to) relate to transfusion of blood components and not treatment with plasma products (i.e. products that are manufactured from plasma). To date, no case of vCJD has been associated with treatment with plasma-products (e.g. clotting factors used to treat individuals with bleeding disorders such as haemophilia).

This fourth case has been classified by the National CJD Surveillance Unit ( www.cjd.ed.ac.uk ) as a 'probable' case of vCJD. Of the 158 vCJD cases that have died (data to 5 Jan 2007), all 112 that have undergone post-mortem (46 have not) have been 'confirmed' by neuropathological examination (examination of brain tissue).

The first clinical case of vCJD associated with transfusion was identified in December 2003. A case of vCJD 'infection' associated with transfusion was identified a few months later. The patient had no symptoms but evidence of infection (abnormal prion proteins) was identified in a post mortem investigation. The individual died from causes unrelated to vCJD.

Following the first case of vCJD associated with a blood transfusion in 2003, the Department of Health asked all recipients of blood transfusions not to donate blood as a precautionary measure to protect the blood supply from vCJD.

Patients who are informed that they are considered to be 'at risk' of vCJD for public health purposes are asked to take the following precautions to reduce the chance of passing on vCJD to other people: Not to donate blood, tissues or organs and To inform their healthcare providers of their 'at-risk' status so that special procedures may be arranged for certain instruments used in their healthcare (NB. Their GPs are also asked to do this.)

A range of measures have been put in place by the Department of Health to minimise the possible risk of vCJD being passed through blood:

Since 1997 all cases of vCJD that are reported to the National CJD Surveillance Unit and diagnosed as having 'probable' vCJD, result in a search of the UK Blood Services blood donor records. If the patient has donated blood, any unused parts of that blood are immediately removed from stock. The fate of all used components of blood from the donor is traced, and surviving recipients informed of their risk. In July 1998, the Department of Health announced that plasma for the manufacture of blood products, such as clotting factors, would be obtained from non-UK sources. Since October 1999, white blood cells (which may carry the greatest risk of transmitting vCJD) have been removed from all blood used for transfusion. In August 2002 the Department of Health announced that fresh frozen plasma for treating babies and young children born after 1 January 1996 would be obtained from the USA, extended to all children under 16 years of age (Summer 2005). In December 2002, the Department of Health completed its purchase of the largest remaining independent US plasma collector, Life Resources Incorporated. This secures long-term supplies of non-UK blood plasma for the benefit of NHS patients. Since April 2004, blood donations have not been accepted from people who have themselves received a blood transfusion in the UK since 1980. This has been extended to include apheresis donors and donors who are unsure if they had previously had a blood transfusion (August 2004). Since late 2005, blood donations have not been accepted from donors whose blood was transfused to patients who later developed vCJD. The UK Blood Services continue to promote the appropriate use of blood and tissues and alternatives throughout the NHS.

The likelihood of a person who may be infected with vCJD going onto develop symptoms of the disease is uncertain, and may depend on individual susceptibility. It is possible that infected individuals may never develop symptoms.

For further information contact the HPA press office on 0208 327 7098/7097/6055

Specialist care for vCJD is available from The NHS National Prion Clinic, based at The Hospital for Neurology and Neurosurgery, Queen Square, London

http://www.nationalprionclinic.org/


The National CJD Surveillance Unit is based at the Western General Hospital Edinburgh:

www.cjd.ed.ac.uk


For further information about vCJD go to:


http://www.hpa.org.uk/infections/topics_az/cjd/menu.htm


http://www.dh.gov.uk/PolicyAndGuidance/HealthAndSocialCareTopics/CJD/fs/en


http://www.blood.co.uk/ http://www.cjd.ed.ac.uk http://www.nationalprionclinic.org/



Last reviewed: 13 December 2007


http://www.hpa.org.uk/webw/HPAweb&HPAwebStandard/HPAweb_C/1195733711457?p=1171991026241



TRANSFUSION MEDICINE

EPIDEMIOLOGY REVIEW (TMER)

The Transfusion Medicine Epidemiology Review (TMER) is a collaborative project between the UK NCJDSU and the UK Blood Services. The main purpose is to investigate whether there is any evidence that Creutzfeldt-Jakob disease (CJD) or variant Creutzfeldt-Jakob disease (vCJD) may have been transmitted via the blood supply.

This website contains data on vCJD donors and cases of vCJD who have received transfusions in the past but does not contain data on the ongoing study of sporadic CJD.

This site was last updated on 11 March 2008

Methods

Results

vCJD donor summary

Use of blood donations from vCJD cases

Fate of recipients

vCJD cases who received blood tranfusion(s) in the past

Relevant Publications

Back to NCJDSU Home Page


http://www.cjd.ed.ac.uk/TMER/TMER.htm



Thursday, July 24, 2008

Prion diseases are efficiently transmitted by blood transfusion in sheep


http://vcjdblood.blogspot.com/2008/07/prion-diseases-are-efficiently.html



4th CASE VCJD VIA BLOOD TRANSFUSION, BSE, BASE, AND SPORADIC CJD

By Terry S Singeltary

Bacliff, Texas USA Jan 24, 07


http://www.bloodindex.com/view_news_zone.php?id=206




----- Original Message ----- From: Terry S. Singeltary Sr. To: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000071/!x-usc:mailto:FREAS@CBER.FDA.GOV Cc: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000071/!x-usc:mailto:william.freas@fda.hhs.gov ; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000071/!x-usc:mailto:rosanna.harvey@fda.hhs.gov Sent: Wednesday, November 29, 2006 1:24 PM Subject: TSE advisory committee for the meeting December 15, 2006 [TSS SUBMISSION]

November 29, 2006

Greetings FDA, DHH, Dr. Freas, and Dr. Harvey et al,

a kind and warm Holiday Greetings to you all.

i kindly wish to submit the following to the TSE advisory committee for the meeting December 15, 2006, about the assessment for potential exposure to vCJD in human plasma-derived antihemophilic factor (FVIII) products manufactured from U.S. plasma donors and related communication material ;


http://a257.g.akamaitech.net/7/257/2422/01jan20061800/edocket.access.gpo.gov/2006/E6-20251.htm



i see the media picked up on this as a 'low risk', from what the gov. agency perceived to be to them;


http://www.newsday.com/news/health/ats-ap_health14nov27,0,7955259.story?coll=ny-leadhealthnews-headlines



however, i seem to disagree. from my primitive ciphering, i see it another way. this is a huge catastrophic risk. 3 in 160 is 1.9%. so call that 2% which is 1 in 50 or twenty per thousand or 20,000 per million. also, what about the mixed genotypes/mixed susceptibility? what about the silent carriers that donated tainted blood? what about the sporadic CJDs of UNKNOWN strain or phenotype? this risk assessment is just more BSe to me. Just another in a long line of industry fed crap. i pray that my assessment is the one that is wrong. but it is THEY who roll the dice with your life. It is THEY who refuse to regulate an industry that has run amok. just from a recall aspect of potentially tainted blood, and these are just recent recalls ;

PRODUCT Source Plasma, Recall # B-0054-7 CODE Units: 03MMNC5465, 03MMNC6361, 03MMNC6801, 03MMNC7510, 03MMNC7891, 03MMNC8252, 03MMNC8801, 03MMNC9144, 03MMND1122, 03MMND1478, 03MMND1969, 03MMND2350, 03MMND2825, 03MMND3211, 03MMND3708, 03MMND4072, 03MMND4588, 03MMND4831, 03MMND5320, 03MMND5719, 03MMND6268, 03MMND6683, 03MMND7228, 03MMND7656, 03MMND8211, 03MMND8652, 03MMND9195, 03MMND9618, 03MMNE0628, 03MMNE0884, 03MMNE1597, 03MMNE1979, 03MMNE2644, 03MMNE3064, 03MMNE3707, 03MMNE4122, 03MMNE4750, 03MMNE5080, 03MMNE5876, 03MMNE6218, 03MMNE7189, 03MMNE7587, 03MMNE8027, 03MMNE8645, 03MMNE9029, 03MMNE9641, 03MMNE9979, 03MMNF0491, 03MMNF0685, 03MMNF0937, 03MMNF1260, 04MMNA0351, 04MMNA0707, 04MMNA1241, 04MMNA1650, 04MMNA2291, 04MMNA2646, 04MMNA3340, 04MMNA3719, 04MMNA4312, 04MMNA4683, 04MMNA5298, 04MMNA5750, 04MMNA6407, 04MMNA6816, 04MMNA7482, 04MMNA7915, 04MMNA8632, 04MMNA9076, 04MMNA9723, 04MMNB0063, 04MMNB0696, 04MMNB1100, 04MMNB1845, 04MMNB2285, 04MMNB3035, 04MMNB3485, 04MMNB4213, 04MMNB4672, 04MMNB5841, 04MMNB6652, 04MMNB7162, 04MMNB7930, 04MMNB8453, 04MMNB9239, 04MMNB9747, 04MMNC0456, 04MMNC0931, 04MMNC1578 RECALLING FIRM/MANUFACTURER BioLife Plasma Services, L.P., Mankato, MN, by facsimile on June 4, 2004. Firm initiated recall is complete. REASON Blood products, collected from a donor who was at increased risk for new variant Creutzfeldt-Jakob Disease (nvCJD), were distributed. VOLUME OF PRODUCT IN COMMERCE 89 units DISTRIBUTION CA and Austria

END OF ENFORCEMENT REPORT FOR October 25, 2006

###


http://www.fda.gov/bbs/topics/enforce/2006/ENF00975.html



USA FDA MAD COW BLOOD HUMANS RECALL (these are dime a dozen)

RECALLS AND FIELD CORRECTIONS: BIOLOGICS -- CLASS II ______________________________ PRODUCT Source Plasma, Recall # B-1708-6 CODE Units: MI180733, MI180927, MI181625, MI181780, MI182337, MI182519, MI183140, MI183311, MI183955, MI185006, MI185278, MI185822, MI186081, MI186855, MI187183, MI187903, MI188273, MI188695, MI189257, MI189553, MI190136, MI190473, MI191073, MI191395, MI191972, MI192303, MI193473, MI194343, 04MINA0377, 04MINA0801, 05MINA7147, 05MINA7451, 05MINA8094, 05MINA8504, 05MINA9548, 05MINA9883, 05MINB0489, 05MINB0875, 05MINB1469, 05MINB1874, 05MINB3116, 05MINB7192, 05MINB7529, 05MINB8246, 05MINB8612, 05MINB9236, 05MINB9366, 05MINB9475, 05MINB9641, 05MINC0031, 05MINC0237, 05MINC0336, 05MINC0894, 05MINC0964, 05MINC1138, 05MINC1619, 05MINC1750, 05MINC1907, 05MINC1977, 05MINC2375, 05MINC2774, 05MINC3113, 05MINC3484, 05MINC4277, 05MINC4623, 05MINC5623, 05MINC5914, 05MINC7545, 05MINC7870, 05MINC8355, 05MINC8689, 05MINC9437, 05MINC9775, 05MIND0067, 05MIND0393, 05MIND0892, 05MIND0951, 05MIND1836, 05MIND2183 and 05MIND2962 RECALLING FIRM/MANUFACTURER BioLife Plasma Services L.P., Muncie, IN, by facsimile on November 22, 2005. Firm initiated recall is complete. REASON Blood products, collected from unsuitable donors based on risk factors for Creutzfeldt-Jakob Disease (CJD), were distributed. VOLUME OF PRODUCT IN COMMERCE 80 units DISTRIBUTION CA, NC, and MD

______________________________

PRODUCT a) Red Blood Cells, Leukocytes Reduced, Recall # B-1714-6; b) Fresh Frozen Plasma, Recall # B-1715-6; c) Platelets, Recall # B-1716-6 CODE a), b), and c) Unit: 2443732 RECALLING FIRM/MANUFACTURER South Texas Blood and Tissue Center, San Antonio, TX, by letters dated November 11, 2003 and December 18, 2003. Firm initiated recall is complete. REASON Blood products, collected from a donor who was at increased risk for new variant Creutzfeldt-Jakob Disease (nvCJD), were distributed. VOLUME OF PRODUCT IN COMMERCE 3 units DISTRIBUTION TX and WI

END OF ENFORCEMENT REPORT FOR SEPTEMBER 13, 2006

###


http://www.fda.gov/bbs/topics/enforce/2006/ENF00969.html



PRODUCT Fresh Frozen Plasma, Recall # B-1751-6 CODE Unit: 4936623 RECALLING FIRM/MANUFACTURER Gulf Coast Regional Blood Center, Houston, TX, by facsimile dated September 16, 2005. Firm initiated recall is complete. REASON Blood product, which was collected from an unsuitable donor based on risk factors for variant Creutzfeldt-Jakob Disease (vCJD), was distributed. VOLUME OF PRODUCT IN COMMERCE 1 unit DISTRIBUTION TX

END OF ENFORCEMENT REPORT FOR SEPTEMBER 6, 2006

###


http://www.fda.gov/bbs/topics/enforce/2006/ENF00968.html



Mon Aug 7, 2006 10:24 71.248.132.189

PRODUCT a) Red Blood Cells, Recall # B-1587-6; b) Cryoprecipitated AHF, Recall # B-1588-6; c) Recovered Plasma, Recal # B-1589-6 CODE a), b) and c) Unit: 2016719 RECALLING FIRM/MANUFACTURER Walter Shepeard Community Blood Center, Inc., Augusta, GA, by facsimile on March 13, 2003. Firm initiated recall is complete. REASON Blood products, which were collected from a donor who may be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed. VOLUME OF PRODUCT IN COMMERCE 3 units DISTRIBUTION GA and Germany

______________________________ PRODUCT a) Red Blood Cells Leukocytes Reduced, Recall # B-1590-6; b) Fresh Frozen Plasma, Recall # B-1591-6 CODE a) and b) Unit: 2443595 RECALLING FIRM/MANUFACTURER South Texas Blood and Tissue Center, San Antonio, TX, by facsimile on June 30, 2004. Firm initiated recall is complete. REASON Blood products, which were collected from a donor who may be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed. VOLUME OF PRODUCT IN COMMERCE 2 units DISTRIBUTION TX

______________________________ PRODUCT a) Red Blood Cells Leukocytes Reduced, Recall # B-1592-6; b) Fresh Frozen Plasma, Recall # B-1593-6 CODE a) and b) Unit: 2545596 RECALLING FIRM/MANUFACTURER South Texas Blood and Tissue Center, San Antonio, TX, by facsimile on December 14, 2004 and January 3, 2005. Firm initiated recall is complete. REASON Blood products, which were collected from a donor who may be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed. VOLUME OF PRODUCT IN COMMERCE 2 units DISTRIBUTION TX

______________________________



http://www.fda.gov/bbs/topics/enforce/2006/ENF00963.html



PRODUCT a) Red Blood Cells Leukocytes Reduced, Recall # B-1550-6; b) Fresh Frozen Plasma, Recall # B-1551-6 CODE a) and b) Unit 2395371 RECALLING FIRM/MANUFACTURER South Texas Blood and Tissue Center, San Antonio, TX, by fax on August 20, 2003. Firm initiated recall is complete. REASON Blood products, which were collected from a donor who may be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed. VOLUME OF PRODUCT IN COMMERCE 2 units DISTRIBUTION TX ______________________________ PRODUCT a) Red Blood Cells Leukocytes Reduced, Recall # B-1552-6; b) Platelets, Recall # B-1553-6; c) Fresh Frozen Plasma, Recall # B-1554-6 CODE a), b) and c) Unit 2438702 RECALLING FIRM/MANUFACTURER South Texas Blood and Tissue Center, San Antonio, TX, by fax on May 29, 2003. Firm initiated recall is complete. REASON Blood products, which were collected from a donor who may be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed. VOLUME OF PRODUCT IN COMMERCE 3 units DISTRIBUTION TX

______________________________ PRODUCT a) Red Blood Cells Leukocytes Reduced, Recall # B-1555-6; b) Fresh Frozen Plasma, Recall # B-1556-6 CODE a) and b) Unit 2454970 RECALLING FIRM/MANUFACTURER South Texas Blood and Tissue Center, San Antonio, TX, by fax on July 23 and December 11. 2003. Firm initiated recall is complete. REASON Blood products, which were collected from a donor who may be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed. VOLUME OF PRODUCT IN COMMERCE 2 units DISTRIBUTION TX

______________________________ PRODUCT a) Red Blood Cells, Recall # B-1494-6 b) Cryoprecipitated AHF, Recall # B-1495-6 CODE a) and b) Unit 5013100 RECALLING FIRM/MANUFACTURER Walter L. Shepeard Community Blood Center, Inc., Augusta, GA, by fax on May 17, 2005. Firm initiated recall is complete. REASON Blood products, which were collected from a donor who may be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed. VOLUME OF PRODUCT IN COMMERCE 2 units DISTRIBUTION GA

______________________________ PRODUCT Source Plasma, Recall # B-1450-6 CODE Unit numbers ST0824313 and ST0824764 RECALLING FIRM/MANUFACTURER Stillwater Plasma Center LLC, Stillwater, OK, by fax on November 21, 2003. Firm initiated recall is complete. REASON Blood products, which were collected from a donor whose suitability pertaining to risk factors for Creutzfeldt-Jakob Disease (vCJD) was not adequately determined, were distributed. VOLUME OF PRODUCT IN COMMERCE 2 units DISTRIBUTION UK

______________________________ PRODUCT Plasma Frozen, Recall # B-1422-6; Recovered Plasma, Recall # B-1423-6 CODE a) Unit 03E42218; b) Unit 03E38153 RECALLING FIRM/MANUFACTURER American Red Cross Blood Services, Atlanta, GA, by telephone, e-mail or letter on February 20 or 21, 2004. Firm initiated recall is complete. REASON Blood products, which were collected from a donor who may be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed. VOLUME OF PRODUCT IN COMMERCE 2 units DISTRIBUTION GA and Switzerland

______________________________ PRODUCT a) Red Blood Cells Leukocytes Reduced, Recall # B-1374-6; b) Recovered Plasma, Recall # B-1375-6 CODE a) and b) unit 2453906 RECALLING FIRM/MANUFACTURER South Texas Blood and Tissue Center, San Antonio, TX, by fax on October 31 and November 5, 2003. Firm initiated recall is complete. REASON Blood products, which were collected from a donor who may be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed. VOLUME OF PRODUCT IN COMMERCE 2 units DISTRIBUTION TX and Austria

______________________________ PRODUCT Source Plasma. Recall # B-1295-6 CODE Units: NG0046551, NG0045950 RECALLING FIRM/MANUFACTURER DCI Biologicals Nacogdoches LLC, Nacogdoches, TX, by telephone and fax on December 20, 2002, Firm initiated recall is complete. REASON Blood products, collected from a donor who did not answer the questions on the new variant Creutzfeldt-Jacob disease (nvCJD) questionnaire appropriately, were distributed. VOLUME OF PRODUCT IN COMMERCE 2 units DISTRIBUTION KY

______________________________ PRODUCT Source Plasma. Recall # B-1296-6 CODE Unit: NG 0044520 RECALLING FIRM/MANUFACTURER DCI Biologicals Nacogdoches LLC, Nacogdoches, TX, by telephone and fax on December 12, 2002. Firm initiated recall is complete. REASON Blood product, collected from a donor who did not answer the questions on the new variant Creutzfeldt-Jacob disease (nvCJD) questionnaire, was distributed. VOLUME OF PRODUCT IN COMMERCE 1 unit DISTRIBUTION KY

______________________________ PRODUCT Source Plasma. Recall # B-1297-6 CODE Units: NG0042874, NG0043139, NG0043312, NG0043618, NG0043797, NG0044020, NG0044209, NG0044507, NG0044718, NG0044977, NG0045161, NG0045412, NG0045555 RECALLING FIRM/MANUFACTURER DCI Biologicals Nacogdoches LLC, Nacogdoches, TX, by telephone and fax on December 20, 2002. 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 13 units DISTRIBUTION KY

______________________________ PRODUCT Source Plasma, Recall # B-1298-6 CODE Units: NG 0046823, NG 0046671, NG 0045205, NG 0044635, NG 0043095, NG 0042525, NG 0042341 RECALLING FIRM/MANUFACTURER DCI Biologicals Nacogdoches LLC, Nacogdoches, TX, by telephone and fax on December 20, 2002. Firm initiated recall is complete. REASON Blood products, collected from a donor who answered questions on the variant Creutzfeldt-Jacob disease (vCJD) questionnaire inappropriately, were distributed. VOLUME OF PRODUCT IN COMMERCE 7 units DISTRIBUTION KY

______________________________ PRODUCT Recovered Plasma, Recall # B-1299-6 CODE Unit: 4357117 RECALLING FIRM/MANUFACTURER Department of the Navy, Naval Medical Center, San Diego, CA, by fax and letter on September 25, 2003. Firm initiated recall is complete. REASON Blood product, collected from a donor considered to be at risk of exposure to Creutzfeldt-Jacob Disease (CJD), was distributed. VOLUME OF PRODUCT IN COMMERCE 1 unit DISTRIBUTION Germany

END OF ENFORCEMENT REPORT FOR July 12, 2006

###



http://www.fda.gov/bbs/topics/enforce/2006/ENF00960.html



CJD WATCH MESSAGE BOARD TSS FDA mad cow nvCJD 'only' blood recalls 1ST WEEK JULY Fri Jul 7, 2006 09:37 70.110.83.160

FDA mad cow nvCJD 'only' blood recalls 1ST WEEK JULY

PRODUCT a) Red Blood Cells Leukocytes Reduced, Recall # B-1379-6; b) Platelets, Recall # B-1380-6; c) Fresh Frozen Plasma, Recall # 1381-6; d) Recovered Plasma, Recall # B-1382-6 CODE a) Unit numbers: 2343106, 2377779, and 2403533; b) and c) Unit numbers: 2377779; d) Unit numbers: 2343106 and 2403533 RECALLING FIRM/MANUFACTURER South Texas Blood and Tissue Center, San Antonio, TX, by facsimile on June 12, 2003. Firm initiated recall is complete. REASON Blood products, which were collected from a donor who may be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed. VOLUME OF PRODUCT IN COMMERCE 7 units DISTRIBUTION TX and Austria ______________________________

PRODUCT a) Red Blood Cells Leukocytes Reduced, Recall # B-1467-6; b) Recovered Plasma, Recall # B-1468-6 CODE a) and b) Unit numbers: 2329380 RECALLING FIRM/MANUFACTURER South Texas Blood and Tissue Center, San Antonio, TX, by facsimile on May 8, 2003. Firm initiated recall is complete. REASON Blood products, which were collected from a donor who may be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed. VOLUME OF PRODUCT IN COMMERCE 2 units DISTRIBUTION TX and Switzerland

______________________________

PRODUCT a) Red Blood Cells Leukocytes Reduced, Recall # B-1479-6; b) Cryoprecipitated AHF, Recall # B-1480-6; c) Recovered Plasma, Recall # B-1481-6 CODE a), b), and c) Unit numbers: 2383280 RECALLING FIRM/MANUFACTURER South Texas Blood and Tissue Center, San Antonio, TX, by facsimile on July 23 and 29, 2004. Firm initiated recall is complete. REASON Blood products, which were collected from a donor who may be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed. VOLUME OF PRODUCT IN COMMERCE 3 units DISTRIBUTION TX and Switzerland

______________________________ PRODUCT a) Red Blood Cells Leukocytes Reduced, Recall # B-1482-6; b) Fresh Frozen Plasma, Recall # B-1483-6 CODE a) and b) Unit number: 2501452 RECALLING FIRM/MANUFACTURER South Texas Blood and Tissue Center, San Antonio, TX, by facsimile on October 5, 2004. Firm initiated recall is complete. REASON Blood products, which were collected from a donor who may be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed. VOLUME OF PRODUCT IN COMMERCE 2 units DISTRIBUTION TX and NY

______________________________ PRODUCT a) Red Blood Cells Leukocytes Reduced, Recall # B-1484-6; b) Plasma Cryoprecipitated Reduced, Recall # B-1485-6; c) Recovered Plasma, Recall # B-1486-6 CODE a) and c) Unit number: 2554077; b) Unit number: 2415708 RECALLING FIRM/MANUFACTURER South Texas Blood and Tissue Center, San Antonio, TX, by facsimile on August 13, 2004. Firm initiated recall is complete. REASON Blood products, which were collected from a donor who may be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed. VOLUME OF PRODUCT IN COMMERCE 3 units DISTRIBUTION TX and Austria

_____________________________________

END OF ENFORCEMENT REPORT FOR July 5, 2006

###



http://www.fda.gov/bbs/topics/enforce/2006/ENF00959.html



Greetings again Dr. Freas et al at FDA,

WITH new atypical TSE in the bovine, in the sheep, goat, and humans, and the fact that the new BASE TSE in cattle being very very similar to sporadic CJD, rather than the nvCJD, the fact that now science showing the TSE agent of the atypical cattle in Japan showing infectivity other than CNS tissue, the fact that the latest Texas mad cow and the recent Alabama mad cow both being of the atypical strain, it would seem prudent to include all human TSE in the blood ban, in my opinion. with sporadic CJD, you have many strains and or phenotypes, some of which are 'UNKNOWN', so we do not know how this will transmit, what tissues are infectious and or if blood transmits. that's the bottom line, however it has been reported that the BASE is more virulent to humans. With this, and the fact that sporadic CJD has tripled in the past few years or so, i see it as being prudent to take serious and immediate action ;

PERSPECTIVE

On the Question of Sporadic or Atypical Bovine Spongiform Encephalopathy and Creutzfeldt-Jakob Disease

Paul Brown,* Lisa M. McShane,† Gianluigi Zanusso,‡ and Linda Detwiler§


snip... full text 48 pages ;


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





Subject: Re: Docket No. 2005N-0373 RIN number 0910-AF54 Use of Materials Derived From Cattle in Medical Products REOPENING COMMENT PERIOD Date: March 30, 2007 at 11:37 am PST

Subject: Re: Docket No. 2005N-0373 RIN number 0910-AF54 TSS SUBMISSION Date: March 30, 2007 at 10:57 am PST


http://madcowfeed.blogspot.com/2008/07/docket-no-2005n-0373-and-rin-number.html




18 January 2007 - Draft minutes of the SEAC 95 meeting (426 KB) held on 7 December 2006 are now available.

snip...

4. Members had received information about the notification by the Health Protection Agency (HPA) of recipients of four batches of plasma products that had been produced from blood donated by individuals that had later developed variant Creutzfeldt Jakob Disease (vCJD). THESE batches HAD NOT been included in a similar notification exercise in 2004, as the fate of these products COULD NOT BE TRACED at that time. The fourteenth annual report of the National CJD Surveillance Unit had been published. The European Food Safety Authority (EFSA) had issued a consultation on a revised methodology for geographical bovine spongiform encephalopathy (BSE) risk assessment. Members could submit individual responses. Submission of a SEAC response was under consideration.

snip...

ITEM 9 - ANY OTHER BUSINESS

snip...

***$$$***

64. A member noted that at the recent Neuroprion meeting, a study was presented showing that in transgenic mice BSE passaged in sheep may be more virulent and infectious to a wider range of species than bovine derived BSE. Other work presented suggested that BSE and bovine amyloidotic spongiform encephalopathy (BASE) MAY BE RELATED. A mutation had been identified in the prion protein gene in an AMERICAN BASE CASE THAT WAS SIMILAR IN NATURE TO A MUTATION FOUND IN CASES OF SPORADIC CJD. A study also demonstrated that in a mouse model it was possible to alleviate the pathological changes of prion disease by suppressing expression of the prion protein gene after infection.


http://www.seac.gov.uk/minutes/95.pdf



Molecular Mechanisms of Prion Pathogenesis

Adriano Aguzzi, Christina Sigurdson, and Mathias Heikenwaelder Institute of Neuropathology, University Hospital of Z¨ urich, CH-8091 Z¨ urich, Switzerland; email: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000071/!x-usc:mailto:adriano.aguzzi@usz.ch, mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000071/!x-usc:mailto:mathias.heikenwaelder@usz.ch, mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000071/!x-usc:mailto:christina.sigurdson@usz.ch Annu. Rev. Pathol. Mech. Dis. 2008. 3:11-40 First published online as a Review in Advance on August 8, 2007 The Annual Review of Pathology: Mechanisms of Disease is online at pathmechdis.annualreviews.org This article's doi: 10.1146/annurev.pathmechdis.3.121806.154326 Copyright c 2008 by Annual Reviews. All rights

Annu. Rev. Pathol. Mech. Dis. 2008. 3:11-40 First published online as a Review in Advance on August 8, 2007 The Annual Review of Pathology: Mechanisms of Disease is online at pathmechdis.annualreviews.org This article's doi: 10.1146/annurev.pathmechdis.3.121806.154326 Copyright c 2008 by Annual Reviews. All rights reserved 1553-4006/08/0228-0011$20.00

Abstract

Prion diseases are infectious neurodegenerative diseases occurring in humans and animals with an invariably lethal outcome. One fundamental mechanistic event in prion diseases is the aggregation of aberrantly folded prion protein into large amyloid plaques and fibrous structures associated with neurodegeneration. The cellular prion protein (PrPC) is absolutely required for disease development, and prion knockout mice are not susceptible to prion disease. Prions accumulate not only in the central nervous system but also in lymphoid organs, as shown for new variant and sporadic Creutzfeldt- Jakob patients and for some animals. To date it is largely accepted that prions consist primarily of PrPSc, a misfolded and aggregated â-sheet-rich isoform of PrPC. However, PrPSc may or may not be completely congruent with the infectious moiety. Here, we discuss the molecular mechanisms leading to neurodegeneration, the role of the immune system in prion pathogenesis, and the existence of prion strains that appear to have different tropisms and biochemical characteristics.

snip...

As an example in the field of human medicine, four cases of vCJD have been reported to be caused by blood transfusion (9- 11). This indicates that BSE prions can be recycled among humans, which has caused considerable alarm that the supply of bloodderived pharmaceuticals may be threatened (12). In particular, the report of a subclinical blood-derived vCJD infection in an individual carrying a heterozygote methionine/ valine polymorphism at codon 129 of the human PRNP gene (10) suggests that transmission of BSE prions to humans enhances their virulence and broadens the spectrum of susceptible recipients. In this respect, it has been demonstrated that polymorphisms at codon 129 of the human PRNP gene control susceptibility and incubation time in human patients (e.g., 129MM versus 129MV or 129VV drastically increases the susceptibility of humans to BSE prions). It was reported only recently that most individuals who suffered from kuru and were polymorphic at codon 129 showed incubation times longer than 50 years (13). Moreover, recent reports indicate that there is still a lot to be learned about the mechanisms of prion transmission (e.g., human to human or within scrapie-affected animal flocks) and prion tropism underlining the complex alternating distribution patterns of PrPSc (e.g., PrPSc deposition in lymphoid tissue, the CNS) and prion infectivity under varying conditions (e.g., chronic inflammation) and hosts (e.g., sheep, elk and deer, human): Chronic inflammation can alter the tropism of prion infectivity or PrPSc to organs hitherto believed prion free (e.g., liver, pancreas, kidney of mice, mammary gland of sheep, muscle of humans) (14-16). Moreover, PrPSc was reported in spleen and muscle tissue of sporadic Creutzfeldt-Jakob disease (sCJD) patients (17), and prion infectivity was demonstrated in muscle, blood, and saliva of deer suffering from CWD (18, 189). Also, prion infectivity was shown to be excreted via urine of prion-infected nephritic mice, a process defined as prionuria (19). These results emphasize the need for further assessment of possible public health risks from TSE-affected extraneural organs. It is very well possible that preexisting pathophysiological conditions of the infected host additionally contributed to unexpected distribution patterns of prion infectivity. For example, the presence of prion infectivity in the blood of sheep or deer may influence the deposition of prion infectivity in various organs previously deemed prion free. Therefore, it should be carefully reconsidered whether only organs of the CNS and the lymphoreticular system should be included in the current risk classifications of biologicals in the future. It will be important to test altered prion tropism profiles in nonlymphoid organs and body fluids (e.g., blood, urine, milk, saliva) of ruminants (e.g., sheep, goat, cattle, elk, and deer) and human patients suffering from sCJD and vCJD.

snip...

Recent in vivo evidence indicated that a similar phenomenon of conformational variants may occur in Alzheimer's disease (151). Here the existence of Aâ strains that can seed and accelerate aggregation and Aâ pathology was posited. These intriguing observations support the hypothesis that the pathogenetic mechanisms operating in Alzheimer's disease and in prion diseases have more in common than we often appreciate (152). Perhaps future studies will address whether different Aâ strains with distinct biochemical or neuropathological characteristics occur in humans. Can multiple prion strains coexist and effect prion replication? Two subtypes of sporadic CJD have recently been demonstrated to coexist in humans (62). Experimental studies have shown that when two strains infect the same host, one strain can impede the ability of the second strain to cause disease (153). Bartz and colleagues (154) recently suggested that this might be caused by the suppression of prion replication of the second strain. Strain features are useful for tracing prion infections between species. When transmitted to primates, BSE causes lesions strikingly similar to that of vCJD (155, 156). BSE is most likely transmissible to humans too, and strong circumstantial evidence (157-159) suggests that BSE is the cause of vCJD, which has claimed more than 200 lives in the United Kingdom (3, 160), as well as a much smaller number in some other countries (161).

NATURAL TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES: WHAT IS NEW? Cattle Prions More than a few surprises have come from further investigations of prion strains in field cases of TSEs. Until recently, BSE was believed to be associated with one single prion strain, classified by an exclusive and remarkably stable biochemical profile of PrPSc. However, distinct molecular signatures have recently been discovered through the large-scale screening of cattle mandated by European authorities in the context of BSE surveillance. These atypical profiles fall into either of two groups: H-type cases of protease-resistant fragments with a molecular weight higher than BSE, and bovine amyloidotic spongiform encephalopathy, or L type (lower) (162). To test whether these different biochemical and histopathological properties correspond to distinct strains, the Laude laboratory transmitted H-type-PrPSc isolates from French cattle into transgenic mice expressing bovine or ovine PrP (163). The recipient mice developed neurological signs exhibiting strain-specific features clearly distinct from that of the classical BSE agent, providing pivotal evidence that the underlying strains are distinct.

Atypical Sheep Scrapie In 1998, aberrant cases of sheep scrapie were described in Norway and the strain was newly classified as Nor98 (164). Active European Union surveillance later revealed additional cases of atypical scrapie in several other countries (165, 166). Sheep infected with Nor98, or atypical scrapie, accumulated PrPSc primarily in the cerebellum and cerebral cortex rather than in the brainstem target in the classical strain (167). Additionally, on western blot analysis of atypical scrapie cases, an additional small-molecular-weight (10-12 kDa) PrP fragment appeared afterPKdigestion and was shown by epitope mapping to lack both N and C termini of PrP (167, 168). Furthermore, atypical scrapie cases occurred not only in the classical scrapie-susceptible genotypes (A136 R154 Q171), but also in genotypes associated with high resistance to classical scrapie (A136 R154 R171) (165, 166). Were these atypical scrapie cases also infectious? In 2001, atypical scrapie cases were shown to be transmissible prion diseases after inoculated ovine PrP-expressing transgenic mice developed disease and prion aggregates (169). In the meantime, several countries appear to be reporting extremely high incidences of atypical scrapie, and in fact atypical scrapie appears to be the rule rather than the exception in some geographical areas.

Chronic Wasting Disease Among all animal prion diseases, CWD of cervids is likely the most efficiently transmitted. CWD infections occur in mule deer (Odocoileus hemionus), white-tailed deer (O. virginianus), Rocky Mountain elk (Cervus elaphus nelsoni) (170), and moose (Alces alces shirasi) (171). Prevalence can reach as high as 30% in dense, free-ranging deer populations and nearly 100% in captive animals (171). Hypotheses for CWD transmission range from spread via direct contact to exposure through grazing in areas contaminated by prion-infected secretions, excretions (saliva, urine, feces), tissues (placenta), or decomposed carcasses. Insightful experimental studies have recently revealed two key findings: (a) Saliva from CWD-infected deer can transmit disease (18), and (b) CWD-infected carcasses allowed to decay naturally in confined pastures can lead to CWD infections in captive deer (172). Additionally, the abundant CWD-prion accumulation within lymphoid tissues may also lead to CWD prion buildup in nonlymphoid organs with lymphoid follicles, as was recently shown in kidney, potentially shifting shedding routes (173). It is unknown whether other types of inflammation, such as the granulomatous inflammation in the intestine seen in Johne's disease (Mycobacterium avium subsp. paratuberculosis; affects ruminants, including deer and elk) or parasitic inflammation, could lead to or perhaps increase prion excretion by fecal routes. The environmental prion contamination in CWD underscores the difficulties of CWD disease management.Within North America,CWDinfected deer and elk have been detected in 14 states and two Canadian provinces (170, 174, 175). CWD surveillance in Europe has been more limited. However, in Germany, a total of 7300 captive and free-ranging cervids were tested forCWDwith no sign of infection (176). Reindeer or caribou (Rangifer tarandus), from North America or Northern Europe respectively, have a highly homologous prion sequence compared with mule deer and thus are likely susceptible to CWD. Other European cervids such as moose and red deer (C. elaphus) are also expected to be CWD susceptible. The deer and elk primary protein structures are highly conserved, as seen in other mammals. Interestingly, a polymorphism at codon 225S/F may influence CWD susceptibility in mule deer. When comparing the frequency of genotypes among CWDnegative and -positive deer (n = 1482), the odds that a CWD-infected animal was 225SS was 30 times greater when compared with 225SF, whereas the frequency of 225SF/FF genotypes in CWD-negative deer was 9.3%, but only 0.3% in CWD-positive deer (177).

Additionally, elk have a polymorphism at codon 132 (M/L) of Prnp, corresponding to polymorphic codon 129 (M/V) in humans. Elk expressing 132ML and 132LL Prnp were reported to be overrepresented among elk with CWD when compared with uninfected controls (178), and 132LL elk experimentally infected with CWD have resisted infection for at least four years, whereas 132MM or 132ML elk (n = 2 each) developed terminal clinical prion disease by 23 or 40 months post inoculation, respectively, confirmed by immunohistochemistry and western blotting for PrPSc (179). White-tailed deer also have Prnp polymorphisms that may affect their CWD susceptibility. A reduced susceptibility to CWD was linked to a G96S and a Q95H polymorphism in a study comparing allelic frequencies from CWD-positive and CWDnegative free-rangingWisconsin white-tailed deer (180).

snip...end



http://arjournals.annualreviews.org/doi/pdf/10.1146/annurev.pathmechdis.3.121806.154326



USA PRION UNIT BLOG



http://prionunitusaupdate2008.blogspot.com/




Sunday, April 20, 2008

Progress Report from the National Prion Disease Pathology Surveillance Center April 3, 2008

Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.

see full text ;


http://prionunitusaupdate2008.blogspot.com/2008/04/progress-report-from-national-prion.html



CJD TEXAS (cjd clusters)


http://cjdtexas.blogspot.com/



USA WRITTEN CJD QUESTIONNAIRE ???


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




"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." <<< http://www.vetres.org/index.php?option=article&access=standard&Itemid=129&url=/articles/vetres/pdf/2008/04/v07232.pdf'>http://www.vetres.org/index.php?option=article&access=standard&Itemid=129&url=/articles/vetres/pdf/2008/04/v07232.pdf">http://www.vetres.org/index.php?option=article&access=standard&Itemid=129&url=/articles/vetres/pdf/2008/04/v07232.pdf

please see full text ;


http://bse-atypical.blogspot.com/2008/06/review-on-epidemiology-and-dynamics-of.html



***Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.***

Progress Report from the National Prion Disease Pathology Surveillance Center

An Update from Stephen M. Sergay, MB, BCh & Pierluigi Gambetti, MD

April 3, 2008


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



Sunday, March 16, 2008

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


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



HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory JUNE 2008

snip...

Tissue infectivity and strain typing of the many variants Manuscript of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...

snip...


http://cjdmadcowbaseoct2007.blogspot.com/2008/06/human-and-animal-tse-classifications-ie.html



Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate



http://organicconsumers.org/forum/index.php?showtopic=1951



http://bse-atypical.blogspot.com/2008/08/atypical-bse-base-transmitted-from.html



Thursday, July 10, 2008

A New Prionopathy



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



Wednesday, August 20, 2008

Bovine Spongiform Encephalopathy Mad Cow Disease typical and atypical strains, was there a cover-up ?


http://bse-atypical.blogspot.com/2008/08/bovine-spongiform-encephalopathy-mad.html



Friday, August 22, 2008

Creutzfeldt Jakob Disease and Veterans and how they are treated at death


http://creutzfeldt-jakob-disease.blogspot.com/2008/08/creutzfeldt-jakob-disease-and-veterans.html



http://organicconsumers.org/forum/index.php?showtopic=1965



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

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