SEAC 103/2
EFFECT OF AGE ON THE PATHOGENESIS OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
ISSUE
1. Most clinical cases of variant CJD (vCJD) have occurred in young adults. The reasons behind this apparent age-related susceptibility are uncertain. A recent paper by Brown et al in the Journal of Immunology, (attached at annex A), reports the findings of a study in mice, which suggest that age related decline in the functioning of follicular dendritic cells might impair TSE pathogenesis.
STUDY DESIGN
2. The study used C57BL/Dk mice challenged with ME7 scrapie brain homongenate. The mice were assessed weekly for signs of clinical disease and culled at a standard clinical endpoint. Survival times were recorded for mice that did not develop clinical signs of disease and were culled when they showed signs of intercurrent disease.
FINDINGS
3. This study finds that early TSE agent accumulation in the spleens of aged mice was significantly impaired compared to that in young adults. Furthermore, following peripheral exposure, none of the aged mice developed clinical TSE disease during their lifespans, although most mice displayed histopathological signs of TSE disease in their brains.
4. Comparison of follicular dendritic cell networks (FDCs) in the spleens of aged and young adult mice, showed a highly significant reduction in the total number of PrPC expressing FDCs in aged mice when compared with those of young adults. 5. The data imply that the reduced status of FDCs in aged mice significantly impairs the early TSE agent accumulation in
© SEAC 2009
lymphoid tissues and subsequent neuroinvasion. Furthermore, the inefficient neuroinvasion in aged individuals may lead to significant levels of subclinical TSE disease in the population.
BACKGROUND
6. Following peripheral exposure, many TSE agents accumulate first in lymphoid tissues before spreading to the Central Nervous System, where they cause neurodegeneration. Early TSE agent accumulation upon FDCs in lymphoid follicles appears to be critical for efficient neuroinvasion.1
7. Most clinical cases of vCJD have occurred in young adults; the median age at onset of disease was 26 years and the median age at death 28 years (the corresponding ages for sporadic CJD are 67 and 67). The youngest case of vCJD was aged 12 years at onset, while the oldest case of vCJD was aged 74 years.2
ADVICE SOUGHT
8. The committee is invited to note the findings of this study.
SEAC SECRETARIAT
NOVEMBER 2009
1 Klein, M. A., R. Frigg, A. J. Raeber, E. Flechsig, I. Hegyi, R. M. Zinkernagel, C. Weissmann, and A. Aguzzi. 1998. PrP expression in B lymphocytes is not required for prion neuroinvasion. Nat. Med. 4: 1429-1433; Brown, K. L., K. Stewart, D. Ritchie, N. A. Mabbott, A. Williams, H. Fraser, W. I. Morrison, and M. E. Bruce. 1999. Scrapie replication in lymphoid tissuesdepends on PrP-expressing follicular dendritic cells. Nat. Med. 5: 1308-1312. 2 National CJD Surveillance Unit, Seventeenth Annual Report, 2008, p.13. ©
http://www.seac.gov.uk/papers/103-2.pdf
ITEM 6 - UPDATE ON CJD EPIDEMIOLOGY
22. Professor Richard Knight (National CJD Surveillance Unit) updated SEAC on the latest figures for the number of clinical vCJD and sporadic CJD (sCJD) cases. To date there had been 168 definite and probable clinical cases of vCJD in the UK - 165 from dietary infection with BSE and three from vCJD infection via blood transfusion. Four cases are still alive. The number of deaths from vCJD peaked at 28 in 2000 and had since declined with one known death in 2008. The trend in incidence of vCJD deaths fits the quadratic-exponential model. The median age of death is 28 years of age. No individuals born after 1989 have developed vCJD to date. Analysis of vCJD deaths by birth cohort supports the hypothesis that susceptibility to vCJD from dietary exposure to BSE may be age-related with a peak in susceptibility between five and 20 years of age.
23. Professor Knight explained that all the clinical vCJD cases genotyped to date were of the MM genotype with the exception of one case of the MV genotype recently classified as possible vCJD. This patient had died. Although the clinical features in life suggested this was a case of vCJD, it had not been possible to undertake a tonsil biopsy in life or neuropathological examination post mortem so the diagnosis could not be confirmed. The patient was born in 1978, with disease onset in 2007 and death in 2009. The clinical profile of this MV case was consistent with that observed for MM cases suggesting that the neuropathological profile of vCJD in MV and MM cases may be similar.
24. Professor Knight noted that four vCJD patients had been treated with intra-ventricular pentosan polysulphate (PPS) in the UK, in addition to one sCJD, two Gerstmann-Sträussler-Scheinker (GSS) disease and one human growth hormone (hGH) case. There is no evidence of benefit from the use of PPS for the sCJD, GSS and hGH cases. However, it appears PPS may have significantly prolonged the clinical phase of the illness in the vCJD cases treated, although no significant improvement in the clinical condition of these patients had been observed.
25. Professor Knight explained that elsewhere in the world 44 clinical vCJD cases had been reported with 23 in France, five in Spain, four in the Republic of Ireland, three in each of the USA and the Netherlands, two in Portugal and single cases in Canada, Saudi Arabia, Italy and Japan. Infection was presumed to have occurred in the UK in respect of two Irish and two USA cases, one French case, one Japanese case and one Canadian case. The time of the peak of onset of vCJD was five years later in non-UK countries than in the UK.
26. Professor Knight summarised studies to examine potential bloodborne exposures to vCJD. The Transfusion Medicine Epidemiology Review (TMER) identified 66 patients as recipients of labile blood components from donors whom later developed vCJD. Forty three of those patients had died due to non-vCJD related illnesses but three recipients developed clinical vCJD and one subclinical vCJD infections. The reverse TMER study identified three vCJD cases as receiving blood from vCJD infected donors. A study of plasma donations prepared from 1986 to 1998 plasma had identified 25 units of plasma prepared from donations from 11 individuals who later developed vCJD.
27. Professor Knight summarised data on sCJD cases. From May 1990 to January 2009, 1027 cases of sCJD had been identified in the UK with a mean age at death of 67 years and genotype distribution of 63% MM, 19% MV and 18% VV at codon 129 of the prion protein gene.
28. Members asked in what circumstances an autopsy is legally required. Professor Knight explained that autopsy may be legally required when the cause of death is considered not to be from natural causes or is unknown. However, the wishes of the family of the deceased are also considered.
29. Dr Elaine Gadd (Department of Health (DH)) asked about the clinical state of the vCJD cases treated with PPS. Professor Knight explained that the neurological impairment of the patients when treatment began was so advanced that any subtle changes in clinical state would be difficult to assess objectively. The condition of two patients is considered to have significantly deteriorated, whilst one patient may have improved slightly.
ITEM 7 - COMPARING THE RELATIVE RISK OF vCJD TRANSMISSION VIA SINGLE UNIT AND POOLED PLASMA FROM UK AND NON-UK SOURCES (SEAC 102/3)
30. Mr Stephen Dobra (DH) presented an overview of the risk assessment that had been developed by the Department of Health. He explained that there are three Fresh Frozen Plasma (FFP) products in use in the UK: (i) single unit FFP from UK donors given to recipients over 16 years of age, (ii) single unit methylene-blue treated FFP sourced from donors in the United States of America and given to patients under 16 years of age, and (iii) solvent detergent treated FFP (SD FFP) manufactured from pooled plasma currently sourced from countries with no known vCJD cases (although some, such as Germany, have a known BSE risk) given to patients with Thrombotic Thrombocytopenic Purpura (TTP). As most FFP is sourced from UK donors, there is a potential risk of vCJD transmission from its use. Prion reduction technologies may be available in the future for pooled SD FFP.
31. Mr Dobra explained that the risk assessment had been prepared to support decision making by the Advisory Committee on the Safety of Blood, Tissues and Organs (SaBTO) which is considering options for extending the use of imported plasma to all recipients and National Health Service Blood and Transplant which is considering the procurement of plasma from alternative source countries. The risk assessment examines the relative residual risk from use of plasma taking into account the source country, whether it is single unit or pooled, and the method of processing. SEAC was being asked to review the methodology used for the sourcing and pooling elements of the risk assessment. DH will convene an expert group to assess the impact of processing taking into account previous SEAC advice.
32. Members suggested that the presentation of the risk assessment could be improved to provide greater clarity on the reasoning behind some of the assumptions made.
33. A member asked about the estimation of the probability that infectivity would be carried by a plasma product from pooled plasma donations that had been contaminated by a donor infected with vCJD. Mr Dobra suggested that in low infectivity scenarios, infectivity might not be present in all of the plasma product units produced from a contaminated pool as there may be an uneven distribution of infectivity throughout the pool. Members noted that the physico-chemical nature of infectivity in plasma is not known. It may be homogeneously spread within the pool or remain in the form of discrete entities spread unevenly, or something between these two extremes. There may be no low dose threshold for infection.
34. Members asked what confidence there may be in the results from the risk assessment given the large number of variables with large uncertainties. It was noted that as the relative risks (as opposed to absolute risks) posed by plasma products were being estimated, assumptions around the timing, level and distribution of infectivity in blood where there is much uncertainty would not appreciably affect the estimations made. The best way to manage other assumptions where there is large uncertainty, such as around the prevalence of vCJD in the UK and other countries, would be to develop a range of scenarios incorporating reasonable high and low value estimates for such parameters. It was noted that some patients received a large number of transfusions of plasma and plasma products. It may be possible to rule out some scenarios on the basis of observations made on these groups of patients. Members reiterated the importance SEAC placed on obtaining better estimates for the prevalence of subclinical vCJD through a post mortem tissue archive.
35. A member suggested that experiments to examine the infectivity of unused batches of plasma products might provide useful data. It was considered that such experiments may be difficult to conduct as there may be a small number of contaminated batches and they would be difficult to identify.
36. A member asked why prion reduction technologies would only be applied to pooled plasma. Mr Dobra explained that the only proposal of which he was aware had been developed by a manufacturer of pooled plasma and involved filtering a large volume of plasma through a column. The Chair remarked on the lack of independent validation of the efficacy of prion reduction filters to date.
37. A member asked whether the risk assessment could take into account the measures taken in different countries to prevent dietary exposure to BSE and the movement of people from other countries to the UK during the BSE epidemic. Mr Dobra explained that there are no consistent data available to assess differences in dietary exposure to BSE in different European countries. Most countries excluded from donating blood anyone who had visited or been resident in the UK for a significant period of time.
38. A member noted that the risk of vCJD transmission alters depending on the age of the blood donor and asked whether restrictions on the age of donation could be introduced to manage the vCJD transmission risks. Mr Dobra explained that this was possible but would require clear advice from SEAC on the difference in risk and was complicated by the need to ensure sufficient supplies of blood.
39. The Chair summarised the discussion, noting that the committee felt that the best way of handling the uncertainties around key assumptions made in the risk assessment is to use reasonable high and low values for each parameter to derive a range of scenarios. Scenarios could be validated against the number of infections observed in populations that had received large numbers of transfusions of plasma products.
snip...
http://www.seac.gov.uk/papers/103-1.pdf
WHO WILL WATCH THE CHILDREN for CJD over the next 5 decades ?
FOR 4 years, the USDA fed dead stock downer cows, the most high risk cattle for mad cow disease and other dangerous pathogens to children all across the USA via the USDA certified dead stock downer cow school lunch program...
SCHOOL LUNCH PROGRAM FROM DOWNER CATTLE UPDATE
http://downercattle.blogspot.com/2009/0 ... ldren.html
http://downercattle.blogspot.com/
FNS All Regions Affected School Food Authorities By State United States Department of Agriculture Food and Nutrition Service National School Lunch Program March 24, 2008 School Food Authorities Affected by Hallmark/Westland Meat Packing Co. Beef Recall February 2006 - February 2008
http://www.fns.usda.gov/fns/safety/Hall ... yState.pdf
Approximately 50.3 million pounds of the beef recalled by HallmarkNVestland went to federal nutrition programs, including the National School Lunch Program, and of those 50.3 million pounds, about 19.6 million pounds had already been consumed at the time the recall was issued. Release No. 0054.08, USDA, Transcript of Technical Briefing - HallmarldWestland Meat Packing Company (Feb. 21, 2008).
9. HSUS members that consume meat products, including beef products, are concerned about eating adulterated meat products and the health risks associated with such adulterated meat. Specifically, they are concerned that downed cattle are at an increased risk for harboring and transmitting BSE prions and other pathogens. The consumption of meat products derived from BSE-infected cattle is believed to cause a human neurological disease known as variant Creutzfeldt-Jakob disease ("vCJD"). The disease is progressive, invariably fatal, and there is no known effective treatment or cure. Downed cattle may also be at higher risk for harboring other foodborne transmissible pathogens, including E. coli 0157:H7, Salmonella, and anthrax. By allowing downed cattle to enter the food supply, USDA's regulatory loophole injures members of The HSUS by placing them at an increased risk of contracting these food-borne illnesses each time they eat beef. 10. Members of The HSUS are also concerned about the meat products provided to their children through the National School Lunch Program. More than 31 million school children receive lunches through the program each school day. To assist states in providing healthful, low-cost or free meals, USDA provides states with various commodities including ground beef. As evidenced by the HallmarkNVestland investigation and recall, the potential for downed animals to make their way into the National School Lunch Program is neither speculative nor hypothetical.
http://biotech.law.lsu.edu/cases/FDA/hs ... plaint.pdf
United States of America Ex rel. The Humane Society of the United States v. Hallmark Meat Packing Company, Westland Meat Company Inc. (Downed animal abuse/government fraud)
Court or Agency: United States District Court for the Central District of California Plaintiff(s): United States of America Ex rel. The Humane Society of the United States Defendant: Hallmark Meat Packing Company, Westland Meat Company Inc. HSUS Counsel: Peter J. Petersan, Leana Stormont Outside Counsel: Milbank, Tweed, Hadley & McCloy LLP Status: In Briefing
Federal court action under the False Claims Act alleging that Westland/Hallmark defrauded the federal government by violating the terms of its school lunch program contracts requiring the humane handling of animals. The lawsuit was the result of an investigation by The HSUS which exposed the facility's mistreatment of animals too sick or injured to walk and led to the largest meat recall in the nation's history.
http://www.hsus.org/in_the_courts/docke ... lmark.html
http://www.law.gmu.edu/assets/files/aca ... 090818.pdf
http://www.hsus.org/farm/news/ournews/d ... 50409.html
FOIA
http://www.fsis.usda.gov/PDF/FOIA_Requests_0308.pdf
"California Firm Recalls Beef Products". USDA. February 17, 2008.
http://www.fsis.usda.gov/PDF/Recall_005 ... elease.pdf
AP (February 17, 2008). "USDA recalls 143 million pounds of beef". MSNBC.
http://www.msnbc.msn.com/id/23212514/
"Statement by Secretary of Agriculture Ed Schafer Regarding Animal Cruelty Charges Filed Against Employees at Hallmark/Westland Meat Packing Company". USDA. February 15, 2008.
http://www.usda.gov/wps/portal/!ut/p/_s ... 2/0044.xml
"USDA Q&A". USDA. February 19, 2008.
http://www.usda.gov/wps/portal/usdahome ... mation.xml
The Case is captioned as United States of America ex rel. The Humane Society of the United States v. Hallmark Meat Packing Company; Westland Meat Company, Inc.
http://www.hsus.org/farm/news/ournews/d ... 50409.html
http://www.econ.iastate.edu/classes/eco ... erCows.pdf
Thursday, September 24, 2009
(09-24) 15:00 PDT LOS ANGELES (AP) --
A Southern California meatpacking plant that supplied beef to the nation's school lunch program slaughtered stumbling, potentially contaminated cows for four years before undercover video of animal abuse prompted a massive beef recall, federal court filings say...
http://downercattle.blogspot.com/2009/0 ... for-4.html
also ;
http://www.usda.gov/oig/webdocs/24601-07-KC.pdf
USDA officials cited three other interlocking safeguards that protect the public even if other safeguards, such as ante-mortem inspection, should fail; these safeguards are the removal of Specified Risk Materials (SRM),5 BSE surveillance testing, and the feed ban.6 Under the Federal Meat Inspection Act (FMIA),7<<<
Thursday, November 12, 2009
BSE FEED RECALL Misbranding of product by partial label removal to hide original source of materials 2009
http://madcowfeed.blogspot.com/2009/11/ ... oduct.html
Friday, September 4, 2009
FOIA REQUEST ON FEED RECALL PRODUCT 429,128 lbs. feed for ruminant animals may have been contaminated with prohibited material Recall # V-258-2009
http://madcowfeed.blogspot.com/2009/09/ ... oduct.html
Saturday, August 29, 2009
FOIA REQUEST FEED RECALL 2009 Product may have contained prohibited materials Bulk Whole Barley, Recall # V-256-2009
http://madcowfeed.blogspot.com/2009/08/ ... oduct.html
2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006
http://bse-atypical.blogspot.com/2006/0 ... pdate.html
"Everything they did on the Texas cow makes everything USDA did before 2005 suspect," Brown said. ...snip...end
http://www.upi.com/ConsumerHealthDaily/ ... 5557-1284r
PAUL BROWN COMMENT TO ME ON THIS ISSUE
Tuesday, September 12, 2006 11:10 AM
"Actually, Terry, I have been critical of the USDA handling of the mad cow issue for some years, and with Linda Detwiler and others sent lengthy detailed critiques and recommendations to both the USDA and the Canadian Food Agency."
http://madcowtesting.blogspot.com/
see full text sporadic CJD the big lie;
http://lists.ifas.ufl.edu/cgi-bin/wa.ex ... =&P=135027
PLUS
http://cjdmadcowbaseoct2007.blogspot.co ... prion.html
Sunday, August 10, 2008
A New Prionopathy OR more of the same old BSe and sporadic CJD
http://creutzfeldt-jakob-disease.blogsp ... d-bse.html
HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory
http://www.regulations.gov/search/Regs/ ... ntType=pdf
AS I SAID BEFORE, WHO WATCH THE CHILDREN FOR CJD FOR THE NEXT 5 DECADES ???
Thursday, October 15, 2009
Cell Study Explains Why Younger People More At Risk Of Variant Creutzfeldt-Jakob Disease (vCJD)
http://downercattle.blogspot.com/2009/1 ... eople.html
Tuesday, November 17, 2009
SEAC NEW RESULTS ON IDIOPATHIC BRAINSTEM NEURONAL CHROMATOLYSIS (IBNC) FROM THE VETERINARY LABORATORIES AGENCY (VLA) SEAC 103/1
http://bse-atypical.blogspot.com/2009/1 ... athic.html
TSS
Tuesday, November 17, 2009
SEAC EFFECT OF AGE ON THE PATHOGENESIS OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES SEAC 103/2
http://downercattle.blogspot.com/2009/1 ... is-of.html
EFFECT OF AGE ON THE PATHOGENESIS OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
ISSUE
1. Most clinical cases of variant CJD (vCJD) have occurred in young adults. The reasons behind this apparent age-related susceptibility are uncertain. A recent paper by Brown et al in the Journal of Immunology, (attached at annex A), reports the findings of a study in mice, which suggest that age related decline in the functioning of follicular dendritic cells might impair TSE pathogenesis.
STUDY DESIGN
2. The study used C57BL/Dk mice challenged with ME7 scrapie brain homongenate. The mice were assessed weekly for signs of clinical disease and culled at a standard clinical endpoint. Survival times were recorded for mice that did not develop clinical signs of disease and were culled when they showed signs of intercurrent disease.
FINDINGS
3. This study finds that early TSE agent accumulation in the spleens of aged mice was significantly impaired compared to that in young adults. Furthermore, following peripheral exposure, none of the aged mice developed clinical TSE disease during their lifespans, although most mice displayed histopathological signs of TSE disease in their brains.
4. Comparison of follicular dendritic cell networks (FDCs) in the spleens of aged and young adult mice, showed a highly significant reduction in the total number of PrPC expressing FDCs in aged mice when compared with those of young adults. 5. The data imply that the reduced status of FDCs in aged mice significantly impairs the early TSE agent accumulation in
© SEAC 2009
lymphoid tissues and subsequent neuroinvasion. Furthermore, the inefficient neuroinvasion in aged individuals may lead to significant levels of subclinical TSE disease in the population.
BACKGROUND
6. Following peripheral exposure, many TSE agents accumulate first in lymphoid tissues before spreading to the Central Nervous System, where they cause neurodegeneration. Early TSE agent accumulation upon FDCs in lymphoid follicles appears to be critical for efficient neuroinvasion.1
7. Most clinical cases of vCJD have occurred in young adults; the median age at onset of disease was 26 years and the median age at death 28 years (the corresponding ages for sporadic CJD are 67 and 67). The youngest case of vCJD was aged 12 years at onset, while the oldest case of vCJD was aged 74 years.2
ADVICE SOUGHT
8. The committee is invited to note the findings of this study.
SEAC SECRETARIAT
NOVEMBER 2009
1 Klein, M. A., R. Frigg, A. J. Raeber, E. Flechsig, I. Hegyi, R. M. Zinkernagel, C. Weissmann, and A. Aguzzi. 1998. PrP expression in B lymphocytes is not required for prion neuroinvasion. Nat. Med. 4: 1429-1433; Brown, K. L., K. Stewart, D. Ritchie, N. A. Mabbott, A. Williams, H. Fraser, W. I. Morrison, and M. E. Bruce. 1999. Scrapie replication in lymphoid tissuesdepends on PrP-expressing follicular dendritic cells. Nat. Med. 5: 1308-1312. 2 National CJD Surveillance Unit, Seventeenth Annual Report, 2008, p.13. ©
http://www.seac.gov.uk/papers/103-2.pdf
ITEM 6 - UPDATE ON CJD EPIDEMIOLOGY
22. Professor Richard Knight (National CJD Surveillance Unit) updated SEAC on the latest figures for the number of clinical vCJD and sporadic CJD (sCJD) cases. To date there had been 168 definite and probable clinical cases of vCJD in the UK - 165 from dietary infection with BSE and three from vCJD infection via blood transfusion. Four cases are still alive. The number of deaths from vCJD peaked at 28 in 2000 and had since declined with one known death in 2008. The trend in incidence of vCJD deaths fits the quadratic-exponential model. The median age of death is 28 years of age. No individuals born after 1989 have developed vCJD to date. Analysis of vCJD deaths by birth cohort supports the hypothesis that susceptibility to vCJD from dietary exposure to BSE may be age-related with a peak in susceptibility between five and 20 years of age.
23. Professor Knight explained that all the clinical vCJD cases genotyped to date were of the MM genotype with the exception of one case of the MV genotype recently classified as possible vCJD. This patient had died. Although the clinical features in life suggested this was a case of vCJD, it had not been possible to undertake a tonsil biopsy in life or neuropathological examination post mortem so the diagnosis could not be confirmed. The patient was born in 1978, with disease onset in 2007 and death in 2009. The clinical profile of this MV case was consistent with that observed for MM cases suggesting that the neuropathological profile of vCJD in MV and MM cases may be similar.
24. Professor Knight noted that four vCJD patients had been treated with intra-ventricular pentosan polysulphate (PPS) in the UK, in addition to one sCJD, two Gerstmann-Sträussler-Scheinker (GSS) disease and one human growth hormone (hGH) case. There is no evidence of benefit from the use of PPS for the sCJD, GSS and hGH cases. However, it appears PPS may have significantly prolonged the clinical phase of the illness in the vCJD cases treated, although no significant improvement in the clinical condition of these patients had been observed.
25. Professor Knight explained that elsewhere in the world 44 clinical vCJD cases had been reported with 23 in France, five in Spain, four in the Republic of Ireland, three in each of the USA and the Netherlands, two in Portugal and single cases in Canada, Saudi Arabia, Italy and Japan. Infection was presumed to have occurred in the UK in respect of two Irish and two USA cases, one French case, one Japanese case and one Canadian case. The time of the peak of onset of vCJD was five years later in non-UK countries than in the UK.
26. Professor Knight summarised studies to examine potential bloodborne exposures to vCJD. The Transfusion Medicine Epidemiology Review (TMER) identified 66 patients as recipients of labile blood components from donors whom later developed vCJD. Forty three of those patients had died due to non-vCJD related illnesses but three recipients developed clinical vCJD and one subclinical vCJD infections. The reverse TMER study identified three vCJD cases as receiving blood from vCJD infected donors. A study of plasma donations prepared from 1986 to 1998 plasma had identified 25 units of plasma prepared from donations from 11 individuals who later developed vCJD.
27. Professor Knight summarised data on sCJD cases. From May 1990 to January 2009, 1027 cases of sCJD had been identified in the UK with a mean age at death of 67 years and genotype distribution of 63% MM, 19% MV and 18% VV at codon 129 of the prion protein gene.
28. Members asked in what circumstances an autopsy is legally required. Professor Knight explained that autopsy may be legally required when the cause of death is considered not to be from natural causes or is unknown. However, the wishes of the family of the deceased are also considered.
29. Dr Elaine Gadd (Department of Health (DH)) asked about the clinical state of the vCJD cases treated with PPS. Professor Knight explained that the neurological impairment of the patients when treatment began was so advanced that any subtle changes in clinical state would be difficult to assess objectively. The condition of two patients is considered to have significantly deteriorated, whilst one patient may have improved slightly.
ITEM 7 - COMPARING THE RELATIVE RISK OF vCJD TRANSMISSION VIA SINGLE UNIT AND POOLED PLASMA FROM UK AND NON-UK SOURCES (SEAC 102/3)
30. Mr Stephen Dobra (DH) presented an overview of the risk assessment that had been developed by the Department of Health. He explained that there are three Fresh Frozen Plasma (FFP) products in use in the UK: (i) single unit FFP from UK donors given to recipients over 16 years of age, (ii) single unit methylene-blue treated FFP sourced from donors in the United States of America and given to patients under 16 years of age, and (iii) solvent detergent treated FFP (SD FFP) manufactured from pooled plasma currently sourced from countries with no known vCJD cases (although some, such as Germany, have a known BSE risk) given to patients with Thrombotic Thrombocytopenic Purpura (TTP). As most FFP is sourced from UK donors, there is a potential risk of vCJD transmission from its use. Prion reduction technologies may be available in the future for pooled SD FFP.
31. Mr Dobra explained that the risk assessment had been prepared to support decision making by the Advisory Committee on the Safety of Blood, Tissues and Organs (SaBTO) which is considering options for extending the use of imported plasma to all recipients and National Health Service Blood and Transplant which is considering the procurement of plasma from alternative source countries. The risk assessment examines the relative residual risk from use of plasma taking into account the source country, whether it is single unit or pooled, and the method of processing. SEAC was being asked to review the methodology used for the sourcing and pooling elements of the risk assessment. DH will convene an expert group to assess the impact of processing taking into account previous SEAC advice.
32. Members suggested that the presentation of the risk assessment could be improved to provide greater clarity on the reasoning behind some of the assumptions made.
33. A member asked about the estimation of the probability that infectivity would be carried by a plasma product from pooled plasma donations that had been contaminated by a donor infected with vCJD. Mr Dobra suggested that in low infectivity scenarios, infectivity might not be present in all of the plasma product units produced from a contaminated pool as there may be an uneven distribution of infectivity throughout the pool. Members noted that the physico-chemical nature of infectivity in plasma is not known. It may be homogeneously spread within the pool or remain in the form of discrete entities spread unevenly, or something between these two extremes. There may be no low dose threshold for infection.
34. Members asked what confidence there may be in the results from the risk assessment given the large number of variables with large uncertainties. It was noted that as the relative risks (as opposed to absolute risks) posed by plasma products were being estimated, assumptions around the timing, level and distribution of infectivity in blood where there is much uncertainty would not appreciably affect the estimations made. The best way to manage other assumptions where there is large uncertainty, such as around the prevalence of vCJD in the UK and other countries, would be to develop a range of scenarios incorporating reasonable high and low value estimates for such parameters. It was noted that some patients received a large number of transfusions of plasma and plasma products. It may be possible to rule out some scenarios on the basis of observations made on these groups of patients. Members reiterated the importance SEAC placed on obtaining better estimates for the prevalence of subclinical vCJD through a post mortem tissue archive.
35. A member suggested that experiments to examine the infectivity of unused batches of plasma products might provide useful data. It was considered that such experiments may be difficult to conduct as there may be a small number of contaminated batches and they would be difficult to identify.
36. A member asked why prion reduction technologies would only be applied to pooled plasma. Mr Dobra explained that the only proposal of which he was aware had been developed by a manufacturer of pooled plasma and involved filtering a large volume of plasma through a column. The Chair remarked on the lack of independent validation of the efficacy of prion reduction filters to date.
37. A member asked whether the risk assessment could take into account the measures taken in different countries to prevent dietary exposure to BSE and the movement of people from other countries to the UK during the BSE epidemic. Mr Dobra explained that there are no consistent data available to assess differences in dietary exposure to BSE in different European countries. Most countries excluded from donating blood anyone who had visited or been resident in the UK for a significant period of time.
38. A member noted that the risk of vCJD transmission alters depending on the age of the blood donor and asked whether restrictions on the age of donation could be introduced to manage the vCJD transmission risks. Mr Dobra explained that this was possible but would require clear advice from SEAC on the difference in risk and was complicated by the need to ensure sufficient supplies of blood.
39. The Chair summarised the discussion, noting that the committee felt that the best way of handling the uncertainties around key assumptions made in the risk assessment is to use reasonable high and low values for each parameter to derive a range of scenarios. Scenarios could be validated against the number of infections observed in populations that had received large numbers of transfusions of plasma products.
snip...
http://www.seac.gov.uk/papers/103-1.pdf
WHO WILL WATCH THE CHILDREN for CJD over the next 5 decades ?
FOR 4 years, the USDA fed dead stock downer cows, the most high risk cattle for mad cow disease and other dangerous pathogens to children all across the USA via the USDA certified dead stock downer cow school lunch program...
SCHOOL LUNCH PROGRAM FROM DOWNER CATTLE UPDATE
http://downercattle.blogspot.com/2009/0 ... ldren.html
http://downercattle.blogspot.com/
FNS All Regions Affected School Food Authorities By State United States Department of Agriculture Food and Nutrition Service National School Lunch Program March 24, 2008 School Food Authorities Affected by Hallmark/Westland Meat Packing Co. Beef Recall February 2006 - February 2008
http://www.fns.usda.gov/fns/safety/Hall ... yState.pdf
Approximately 50.3 million pounds of the beef recalled by HallmarkNVestland went to federal nutrition programs, including the National School Lunch Program, and of those 50.3 million pounds, about 19.6 million pounds had already been consumed at the time the recall was issued. Release No. 0054.08, USDA, Transcript of Technical Briefing - HallmarldWestland Meat Packing Company (Feb. 21, 2008).
9. HSUS members that consume meat products, including beef products, are concerned about eating adulterated meat products and the health risks associated with such adulterated meat. Specifically, they are concerned that downed cattle are at an increased risk for harboring and transmitting BSE prions and other pathogens. The consumption of meat products derived from BSE-infected cattle is believed to cause a human neurological disease known as variant Creutzfeldt-Jakob disease ("vCJD"). The disease is progressive, invariably fatal, and there is no known effective treatment or cure. Downed cattle may also be at higher risk for harboring other foodborne transmissible pathogens, including E. coli 0157:H7, Salmonella, and anthrax. By allowing downed cattle to enter the food supply, USDA's regulatory loophole injures members of The HSUS by placing them at an increased risk of contracting these food-borne illnesses each time they eat beef. 10. Members of The HSUS are also concerned about the meat products provided to their children through the National School Lunch Program. More than 31 million school children receive lunches through the program each school day. To assist states in providing healthful, low-cost or free meals, USDA provides states with various commodities including ground beef. As evidenced by the HallmarkNVestland investigation and recall, the potential for downed animals to make their way into the National School Lunch Program is neither speculative nor hypothetical.
http://biotech.law.lsu.edu/cases/FDA/hs ... plaint.pdf
United States of America Ex rel. The Humane Society of the United States v. Hallmark Meat Packing Company, Westland Meat Company Inc. (Downed animal abuse/government fraud)
Court or Agency: United States District Court for the Central District of California Plaintiff(s): United States of America Ex rel. The Humane Society of the United States Defendant: Hallmark Meat Packing Company, Westland Meat Company Inc. HSUS Counsel: Peter J. Petersan, Leana Stormont Outside Counsel: Milbank, Tweed, Hadley & McCloy LLP Status: In Briefing
Federal court action under the False Claims Act alleging that Westland/Hallmark defrauded the federal government by violating the terms of its school lunch program contracts requiring the humane handling of animals. The lawsuit was the result of an investigation by The HSUS which exposed the facility's mistreatment of animals too sick or injured to walk and led to the largest meat recall in the nation's history.
http://www.hsus.org/in_the_courts/docke ... lmark.html
http://www.law.gmu.edu/assets/files/aca ... 090818.pdf
http://www.hsus.org/farm/news/ournews/d ... 50409.html
FOIA
http://www.fsis.usda.gov/PDF/FOIA_Requests_0308.pdf
"California Firm Recalls Beef Products". USDA. February 17, 2008.
http://www.fsis.usda.gov/PDF/Recall_005 ... elease.pdf
AP (February 17, 2008). "USDA recalls 143 million pounds of beef". MSNBC.
http://www.msnbc.msn.com/id/23212514/
"Statement by Secretary of Agriculture Ed Schafer Regarding Animal Cruelty Charges Filed Against Employees at Hallmark/Westland Meat Packing Company". USDA. February 15, 2008.
http://www.usda.gov/wps/portal/!ut/p/_s ... 2/0044.xml
"USDA Q&A". USDA. February 19, 2008.
http://www.usda.gov/wps/portal/usdahome ... mation.xml
The Case is captioned as United States of America ex rel. The Humane Society of the United States v. Hallmark Meat Packing Company; Westland Meat Company, Inc.
http://www.hsus.org/farm/news/ournews/d ... 50409.html
http://www.econ.iastate.edu/classes/eco ... erCows.pdf
Thursday, September 24, 2009
(09-24) 15:00 PDT LOS ANGELES (AP) --
A Southern California meatpacking plant that supplied beef to the nation's school lunch program slaughtered stumbling, potentially contaminated cows for four years before undercover video of animal abuse prompted a massive beef recall, federal court filings say...
http://downercattle.blogspot.com/2009/0 ... for-4.html
also ;
http://www.usda.gov/oig/webdocs/24601-07-KC.pdf
USDA officials cited three other interlocking safeguards that protect the public even if other safeguards, such as ante-mortem inspection, should fail; these safeguards are the removal of Specified Risk Materials (SRM),5 BSE surveillance testing, and the feed ban.6 Under the Federal Meat Inspection Act (FMIA),7<<<
Thursday, November 12, 2009
BSE FEED RECALL Misbranding of product by partial label removal to hide original source of materials 2009
http://madcowfeed.blogspot.com/2009/11/ ... oduct.html
Friday, September 4, 2009
FOIA REQUEST ON FEED RECALL PRODUCT 429,128 lbs. feed for ruminant animals may have been contaminated with prohibited material Recall # V-258-2009
http://madcowfeed.blogspot.com/2009/09/ ... oduct.html
Saturday, August 29, 2009
FOIA REQUEST FEED RECALL 2009 Product may have contained prohibited materials Bulk Whole Barley, Recall # V-256-2009
http://madcowfeed.blogspot.com/2009/08/ ... oduct.html
2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006
http://bse-atypical.blogspot.com/2006/0 ... pdate.html
"Everything they did on the Texas cow makes everything USDA did before 2005 suspect," Brown said. ...snip...end
http://www.upi.com/ConsumerHealthDaily/ ... 5557-1284r
PAUL BROWN COMMENT TO ME ON THIS ISSUE
Tuesday, September 12, 2006 11:10 AM
"Actually, Terry, I have been critical of the USDA handling of the mad cow issue for some years, and with Linda Detwiler and others sent lengthy detailed critiques and recommendations to both the USDA and the Canadian Food Agency."
http://madcowtesting.blogspot.com/
see full text sporadic CJD the big lie;
http://lists.ifas.ufl.edu/cgi-bin/wa.ex ... =&P=135027
PLUS
http://cjdmadcowbaseoct2007.blogspot.co ... prion.html
Sunday, August 10, 2008
A New Prionopathy OR more of the same old BSe and sporadic CJD
http://creutzfeldt-jakob-disease.blogsp ... d-bse.html
HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory
http://www.regulations.gov/search/Regs/ ... ntType=pdf
AS I SAID BEFORE, WHO WATCH THE CHILDREN FOR CJD FOR THE NEXT 5 DECADES ???
Thursday, October 15, 2009
Cell Study Explains Why Younger People More At Risk Of Variant Creutzfeldt-Jakob Disease (vCJD)
http://downercattle.blogspot.com/2009/1 ... eople.html
Tuesday, November 17, 2009
SEAC NEW RESULTS ON IDIOPATHIC BRAINSTEM NEURONAL CHROMATOLYSIS (IBNC) FROM THE VETERINARY LABORATORIES AGENCY (VLA) SEAC 103/1
http://bse-atypical.blogspot.com/2009/1 ... athic.html
TSS
Tuesday, November 17, 2009
SEAC EFFECT OF AGE ON THE PATHOGENESIS OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES SEAC 103/2
http://downercattle.blogspot.com/2009/1 ... is-of.html
