Friday, August 7, 2020

National List of Reportable Animal Diseases (NLRAD) proposed rule CWD, Scrapie, BSE, TSE, Prion Disease Singeltary Submission Docket APHIS-2017-0002

National List of Reportable Animal Diseases (NLRAD) proposed rule CWD, Scrapie, BSE, TSE, Prion Disease Singeltary Submission Docket APHIS-2017-0002


August, 7, 2020

Docket No. APHIS-2017-0002

Greetings APHIS et al, i would kindly like to comment on National List of Reportable Animal Diseases (NLRAD) proposed rule Docket APHIS-2017-0002.

I kindly wish to remind you that Transmissible Spongiform Encephalopathy TSE Prion disease is mutating and spreading around the globe. The USA just documented another case of the atypical Nor-98 Scrapie, a case of atypical BSE in Florida just a year or so ago here, CWD TSE Prion spreading across the USA, the recent findings that CWD TSE Prion of cervids, and Sheep Scrapie can transmit to PIGS by the oral routes, and with the outbreak of a new TSE Prion disease in a new livestock species in Camels, a rather large outbreak, i believe it is paramount that the revised National List of Reportable Animal Diseases (NLRAD), address all these concerns. IF you will see, the Bovine Spongiform Encephalopathy BSE animal feeds for ruminants rule, 21 CFR 589.200, this feed ban was nothing but ink on paper, failed year after year after year, with these banned products fed out into commerce. WE must continue to be vigilant on protecting livestock and humans from this deadly TSE Prion pathogen, it has NOT gone away, or disappeared. Thank You...

Terry S. Singeltary Sr.

NEW TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY TSE PRION DISEASE (MAD CAMEL DISEASE) IN A NEW SPECIES

NEW OUTBREAK OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY TSE PRION DISEASE IN A NEW SPECIES

Subject: Prion Disease in Dromedary Camels, Algeria

Our identification of this prion disease in a geographically widespread livestock species requires urgent enforcement of surveillance and assessment of the potential risks to human and animal health.



***> IMPORTS AND EXPORTS <***

SEE MASSIVE AMOUNTS OF BANNED ANIMAL PROTEIN AKA MAD COW FEED IN COMMERCE USA DECADES AFTER POST BAN


cwd scrapie pigs oral routes

***> However, at 51 months of incubation or greater, 5 animals were positive by one or more diagnostic methods. Furthermore, positive bioassay results were obtained from all inoculated groups (oral and intracranial; market weight and end of study) suggesting that swine are potential hosts for the agent of scrapie. <*** 

 >*** Although the current U.S. feed ban is based on keeping tissues from TSE infected cattle from contaminating animal feed, swine rations in the U.S. could contain animal derived components including materials from scrapie infected sheep and goats. These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health. <*** 

***> Results: PrPSc was not detected by EIA and IHC in any RPLNs. All tonsils and MLNs were negative by IHC, though the MLN from one pig in the oral <6 month group was positive by EIA. PrPSc was detected by QuIC in at least one of the lymphoid tissues examined in 5/6 pigs in the intracranial <6 months group, 6/7 intracranial >6 months group, 5/6 pigs in the oral <6 months group, and 4/6 oral >6 months group. Overall, the MLN was positive in 14/19 (74%) of samples examined, the RPLN in 8/18 (44%), and the tonsil in 10/25 (40%). 

***> Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. 

This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. 

Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains. 




Friday, December 14, 2012

DEFRA U.K. What is the risk of Chronic Wasting Disease CWD being introduced into Great Britain? A Qualitative Risk Assessment October 2012

snip.....

In the USA, under the Food and Drug Administration's BSE Feed Regulation (21 CFR 589.2000) most material (exceptions include milk, tallow, and gelatin) from deer and elk is prohibited for use in feed for ruminant animals. With regards to feed for non-ruminant animals, under FDA law, CWD positive deer may not be used for any animal feed or feed ingredients. For elk and deer considered at high risk for CWD, the FDA recommends that these animals do not enter the animal feed system. However, this recommendation is guidance and not a requirement by law.

Animals considered at high risk for CWD include:

1) animals from areas declared to be endemic for CWD and/or to be CWD eradication zones and

2) deer and elk that at some time during the 60-month period prior to slaughter were in a captive herd that contained a CWD-positive animal.

Therefore, in the USA, materials from cervids other than CWD positive animals may be used in animal feed and feed ingredients for non-ruminants.

The amount of animal PAP that is of deer and/or elk origin imported from the USA to GB can not be determined, however, as it is not specified in TRACES. It may constitute a small percentage of the 8412 kilos of non-fish origin processed animal proteins that were imported from US into GB in 2011.

Overall, therefore, it is considered there is a __greater than negligible risk___ that (nonruminant) animal feed and pet food containing deer and/or elk protein is imported into GB.

There is uncertainty associated with this estimate given the lack of data on the amount of deer and/or elk protein possibly being imported in these products.

snip.....

36% in 2007 (Almberg et al., 2011). In such areas, population declines of deer of up to 30 to 50% have been observed (Almberg et al., 2011). In areas of Colorado, the prevalence can be as high as 30% (EFSA, 2011).

The clinical signs of CWD in affected adults are weight loss and behavioural changes that can span weeks or months (Williams, 2005). In addition, signs might include excessive salivation, behavioural alterations including a fixed stare and changes in interaction with other animals in the herd, and an altered stance (Williams, 2005). These signs are indistinguishable from cervids experimentally infected with bovine spongiform encephalopathy (BSE).

Given this, if CWD was to be introduced into countries with BSE such as GB, for example, infected deer populations would need to be tested to differentiate if they were infected with CWD or BSE to minimise the risk of BSE entering the human food-chain via affected venison.

snip.....

The rate of transmission of CWD has been reported to be as high as 30% and can approach 100% among captive animals in endemic areas (Safar et al., 2008).

snip.....

In summary, in endemic areas, there is a medium probability that the soil and surrounding environment is contaminated with CWD prions and in a bioavailable form. In rural areas where CWD has not been reported and deer are present, there is a greater than negligible risk the soil is contaminated with CWD prion.

snip.....

In summary, given the volume of tourists, hunters and servicemen moving between GB and North America, the probability of at least one person travelling to/from a CWD affected area and, in doing so, contaminating their clothing, footwear and/or equipment prior to arriving in GB is greater than negligible... For deer hunters, specifically, the risk is likely to be greater given the increased contact with deer and their environment. However, there is significant uncertainty associated with these estimates.

snip.....

Therefore, it is considered that farmed and park deer may have a higher probability of exposure to CWD transferred to the environment than wild deer given the restricted habitat range and higher frequency of contact with tourists and returning GB residents.

snip.....


the feds just released this statement and you should read this very carefully about the mad cow feed ban that never was, and still isn't, and why this is so important, since USDA APHIS ARS Scientist recent transmitted Chronic Wasting Disease CWD TSE Prion, BY ORAL ROUTES, to PIGS AND SHEEP. this is terrible news, and proves the mad cow feed ban never worked, especially since it really never existed;

ponder this; ***> Adriano Aguzzi...''We even showed that a prion AEROSOL will infect 100% of mice within 10 seconds of exposure''

SUNDAY, SEPTEMBER 1, 2019 

FDA Reports on VFD Compliance

Before and after the current Veterinary Feed Directive (VFD) rules took full effect in January, 2017, the FDA focused primarily on education and outreach to help feed mills, veterinarians and producers understand and comply with the requirements. Since then, FDA has gradually increased the number of VFD inspections and initiated enforcement actions when necessary.


***> Wednesday, January 23, 2019 

***> CFIA SFCR Guidance on Specified risk material (SRM) came into force on January 15, 2019 <***


THURSDAY, JULY 20, 2017 

USDA OIE Alabama Atypical L-type BASE Bovine Spongiform Encephalopathy BSE animal feeds for ruminants rule, 21 CFR 589.200


WEDNESDAY, APRIL 24, 2019 

***> USDA Announces Atypical Bovine Spongiform Encephalopathy Detection Aug 29, 2018 A Review of Science 2019 <***


MONDAY, JANUARY 09, 2017 

Oral Transmission of L-Type Bovine Spongiform Encephalopathy Agent among Cattle 

CDC Volume 23, Number 2—February 2017 

*** Consumption of L-BSE–contaminated feed may pose a risk for oral transmission of the disease agent to cattle.

*** Consumption of L-BSE–contaminated feed may pose a risk for oral transmission of the disease agent to cattle.


TUESDAY, AUGUST 28, 2018 

USDA finds BSE infection in Florida cow 08/28/18 6:43 PM


WEDNESDAY, AUGUST 29, 2018 

USDA Announces Atypical Bovine Spongiform Encephalopathy Detection USDA 08/29/2018 10:00 AM EDT


WEDNESDAY, AUGUST 29, 2018 

Transmissible Spongiform Encephalopathy TSE Prion Atypical BSE Confirmed Florida Update USA August 28, 2018


***> P.108: Successful oral challenge of adult cattle with classical BSE

Sandor Dudas1,*, Kristina Santiago-Mateo1, Tammy Pickles1, Catherine Graham2, and Stefanie Czub1 1Canadian Food Inspection Agency; NCAD Lethbridge; Lethbridge, Alberta, Canada; 2Nova Scotia Department of Agriculture; Pathology Laboratory; Truro, Nova Scotia, Canada

Classical Bovine spongiform encephalopathy (C-type BSE) is a feed- and food-borne fatal neurological disease which can be orally transmitted to cattle and humans. Due to the presence of contaminated milk replacer, it is generally assumed that cattle become infected early in life as calves and then succumb to disease as adults. Here we challenged three 14 months old cattle per-orally with 100 grams of C-type BSE brain to investigate age-related susceptibility or resistance. During incubation, the animals were sampled monthly for blood and feces and subjected to standardized testing to identify changes related to neurological disease. At 53 months post exposure, progressive signs of central nervous system disease were observed in these 3 animals, and they were euthanized. Two of the C-BSE animals tested strongly positive using standard BSE rapid tests, however in 1 C-type challenged animal, Prion 2015 Poster Abstracts S67 PrPsc was not detected using rapid tests for BSE. Subsequent testing resulted in the detection of pathologic lesion in unusual brain location and PrPsc detection by PMCA only. 

***Our study demonstrates susceptibility of adult cattle to oral transmission of classical BSE. 

We are further examining explanations for the unusual disease presentation in the third challenged animal.


***our findings suggest that possible transmission risk of H-type BSE to sheep and human. Bioassay will be required to determine whether the PMCA products are infectious to these animals.

P.86: Estimating the risk of transmission of BSE and scrapie to ruminants and humans by protein misfolding cyclic amplification

Morikazu Imamura, Naoko Tabeta, Yoshifumi Iwamaru, and Yuichi Murayama

National Institute of Animal Health; Tsukuba, Japan

To assess the risk of the transmission of ruminant prions to ruminants and humans at the molecular level, we investigated the ability of abnormal prion protein (PrPSc) of typical and atypical BSEs (L-type and H-type) and typical scrapie to convert normal prion protein (PrPC) from bovine, ovine, and human to proteinase K-resistant PrPSc-like form (PrPres) using serial protein misfolding cyclic amplification (PMCA).

Six rounds of serial PMCA was performed using 10% brain homogenates from transgenic mice expressing bovine, ovine or human PrPC in combination with PrPSc seed from typical and atypical BSE- or typical scrapie-infected brain homogenates from native host species. In the conventional PMCA, the conversion of PrPC to PrPres was observed only when the species of PrPC source and PrPSc seed matched. However, in the PMCA with supplements (digitonin, synthetic polyA and heparin), both bovine and ovine PrPC were converted by PrPSc from all tested prion strains. On the other hand, human PrPC was converted by PrPSc from typical and H-type BSE in this PMCA condition.

Although these results were not compatible with the previous reports describing the lack of transmissibility of H-type BSE to ovine and human transgenic mice, our findings suggest that possible transmission risk of H-type BSE to sheep and human. Bioassay will be required to determine whether the PMCA products are infectious to these animals.

 
***> why do we not want to do TSE transmission studies on chimpanzees $

5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. 

***> I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. 

***> Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.

snip...

R. BRADLEY


WEDNESDAY, JUNE 10, 2020 

Radical Change in Zoonotic Abilities of Atypical BSE Prion Strains as Evidenced by Crossing of Sheep Species Barrier in Transgenic Mice


MONDAY, JULY 27, 2020 

APHIS USDA Nor98-like scrapie was confirmed in a sheep sampled at slaughter in May 2020


TUESDAY, AUGUST 04, 2020 

Genetic and evolutionary considerations of the Chronic Wasting Disease – Human species barrier


-----Original Message-----
From: Terry Singeltary <flounder9@verizon.net>
To: aasv@aasv.org <aasv@aasv.org>; NLRAD.NAHRS@usda.gov <NLRAD.NAHRS@usda.gov>
Cc: snelson@aasv.org <snelson@aasv.org>; WagstromL@nppc.org <WagstromL@nppc.org>; psundberg@swinehealth.org <psundberg@swinehealth.org>
Sent: Mon, Aug 3, 2020 11:56 am
Subject: National List of Reportable Animal Diseases (NLRAD) proposed rule CWD, Scrapie, BSE, TSE, Prion Disease Singeltary Submission Docket APHIS-2017-0002

National List of Reportable Animal Diseases (NLRAD) proposed rule CWD, Scrapie, BSE, TSE, Prion Disease Singeltary Submission Docket APHIS-2017-0002

Regulatory Analysis and Development Policy and Program Development Division Animal and Plant Health Inspection Service Station 3A-03.8, 4700 River Road Unit 118 Riverdale, MD 20737-1238

Submitted electronically to docket APHIS-2017-0002 at http://www.regulations.gov

July 29, 2020

To Whom It May Concern:

We would like to thank the United States Department of Agriculture (USDA) Animal and Plant Health Inspection Service (APHIS) for the opportunity to comment on the National List of Reportable Animal Diseases (NLRAD) proposed rule. The National Pork Producers Council (NPPC) is the global voice for the US pork industry and consists of 42 affiliated state organizations representing America’s 60,000 pork producers. The American Association of Swine Veterinarians (AASV) is a professional association of veterinarians specializing in swine health, welfare, and production. Over 1,500 AASV members represent all facets of the veterinary profession including practice, research, allied industry, public health, government, academia, and education. The Swine Health Information Center (SHIC) has a mission to protect and enhance the health of the United States swine herd through coordinated global disease monitoring, targeted research investments that minimize the impact of future disease threats, and analysis of swine health data. SHIC works directly with the pork industry and public and private industry partners and organizations to facilitate effective and coordinated response to emerging swine diseases.

NPPC, AASV and SHIC support APHIS in its efforts to ensure that the United States is compliant with its reporting obligations as a member of the World Animal Health Organization (OIE) and has a rational, consistent procedure for collecting and responding to information concerning animal diseases of concern. The adoption of the NLRAD will satisfy this need. However, we do have some concerns with the NLRAD proposal that we encourage APHIS to address. The NLRAD has a discrete purpose: to collect and collate information concerning the diagnoses of specific named animal diseases and conditions. The pork industry also recognizes that APHIS has a legitimate need to solicit information about unusual animal health events from a wide range of stakeholders. However, we have significant concerns about conflating these two needs under one NLRAD reporting mechanism. We suggest that APHIS develop a second portal under the National Animal Health Monitoring System (NAHMS) umbrella to collect voluntary reports of suspicious animal health events. For clarity, we will address “suspicious animal health events,” “notifiable diseases and conditions,” and “monitored diseases” requirements separately.

Suspicious Animal Health Events

NPPC, AASV and SHIC concur with APHIS that under-reporting of notifiable animal diseases within the United States can have significant domestic and international ramifications. However, it must be acknowledged that false or premature reports can similarly have damaging consequences. For these reasons, animal health professionals, or indeed any individuals other than veterinarians, should not be obligated to report under the NLRAD when they are not credentialled to make a diagnosis of a specific animal disease. We also have significant concern with requiring veterinarians to report information concerning animal health events that do not meet the case definition of a disease or condition named on the NLRAD, especially since there is no defined response plan for such reports.

The pork industry appreciates APHIS’ rationale in wanting to collect information that falls under the “emerging disease” criteria in the proposed NLRAD rule. However, the proposed criteria are highly subjective—too subjective to mandate reporting. Requiring the reporting of an epidemiological change of a known (but not notifiable) animal disease, for example, creates a significant burden on veterinarians, other animal health professionals, APHIS, and state animal health agencies. For veterinarians and other animal health professionals, it removes discretion in determining the significance of clinical, diagnostic, and/or field observations. For APHIS and state animal health agencies, it will result in a significant increase of reports with no clear methodology or identified resources for analysis and response.

We strongly suggest that APHIS develop an additional portal to receive voluntary reports of suspicious animal health events. This portal could be utilized by veterinarians to report information that falls under the “emerging” classification in the proposed NLRAD rule when, in their professional judgement, they encounter an animal health event that is not classified under the NLRAD but that merits the attention of animal health officials.

Crucially, this system could also be used by other individuals—animal health professionals, producers, or other members of the public with specialized knowledge—to report suspicious animal health events.

Such a portal would have a logical home under the Center for Epidemiology and Animal Health’s Risk Identification Unit. It would provide APHIS with needed information without compromising the intent or utility of the proposed NLRAD system. APHIS must also ensure that information provided is secured, and that confidential information is fully protected. At the same time, validated reports of suspicious animal health events should be shared in summary form with animal industries and other animal health stakeholders.

Notifiable Diseases and Conditions

It is critical that APHIS implement a NLRAD system that has clear, carefully delineated reporting requirements, prevents duplicity of reporting, and that is fully resourced. As indicated above, the pork industry does not feel that this can be accomplished by folding in broader surveillance needs. These needs can be addressed by breaking down the NLRAD proposal into three components: what is to be reported, who is to report it, and how it is to be reported. What is to be reported: Reporting should only be mandated for specific diseases or conditions explicitly listed on the NLRAD. APHIS has indicated that it will publish case definitions for each listed disease and has proposed a process to amend the list itself when new diseases or conditions need to be added. These are appropriate and needed enhancements. It is critical that there be a clear case definition for every disease or condition listed on the NLRAD. What is missing is clarity around response to the report. A veterinary diagnostic laboratory or veterinarian making report of a notifiable animal disease or condition should be provided information concerning the likely response to said report.

A response plan must be published for each and every disease or condition. The case definition document should be extended to contain this information, or a companion document created specific to each response. For diseases or conditions with extensive response plans published elsewhere, these can be referenced and succinctly summarized. If the mandated response is a foreign animal disease investigation, this should be stated. If the report is only required to satisfy national reporting requirements to the OIE, this should be clearly indicated.

In other words, the likely response (realizing that not all mitigating circumstances can be addressed) to a report should be clearly stated for everything listed on the NLRAD. The response document should also clearly state the recommended and/or required actions for the reporter to take or convey as appropriate to the attending veterinarian or owner of the affected animals.

The pork industry does not support formal sub-classification of notifiable disease and conditions into “Emergency,” “Emerging,” and “Regulated” categories. While we appreciate the intent of this proposal, it unnecessarily blends response and surveillance activities with reporting requirements. As we have stated, the “emerging” category should be dealt with outside the NLRAD framework. An “emergency” categorization is irrelevant as all notifiable diseases and conditions must be immediately reported; the “regulated” category is also moot in the context of reporting requirements.

Should it be necessary, APHIS has already established under this proposal a mechanism to mandate reporting to the NLRAD for new or emerging syndromes. It can name the syndrome as a condition, establish a clear case definition, and publish it to the NLRAD. This can be accomplished rapidly and with appropriate consultation with industry and other animal health stakeholders. It will also allow for concurrent development of a response plan, and allocation of resources if needed. Again, the disease or condition should not be added to the NLRAD if a response plan has not been developed. Satisfaction of international reporting obligations is itself a response plan for endemic disease, in some cases, and this should be clearly stated if no other response is intended.

We also strongly suggest that APHIS not exclude “wildlife” from the NLRAD reporting requirements. Any disease or condition listed on the NLRAD should be reportable for any subject species, any population within that species be it wild, feral, captive or farmed.

In summary, reporting should only be mandated for specific, named diseases and conditions. Each named disease or condition should have a clear case definition. All relevant state and federal animal health authorities, as well as industry, should have common understanding of the response, if any, to the report of the disease or condition.

Who is to report it: Given the potential ramifications of a report of a notifiable disease, official reporting should be limited to both public and private veterinary diagnostic laboratories, which are credentialled to confirm the diagnosis of notifiable diseases. Veterinarians who diagnose a notifiable disease or condition without utilizing a veterinary diagnostic laboratory should also have a duty to report.

We have already suggested a mechanism by which other animal health professionals, and indeed any other individuals, can notify state and/or federal animal health officials of unusual animal health observations or events if they are not in a position to bring their concerns to the attention of a veterinarian with whom they have a relationship. This notification pathway again would be independent of that used by veterinarians to report confirmed or suspected diagnoses of discrete notifiable animal disease or conditions. Ultimately any observation of a suspicious animal health event will require the intervention of a veterinarian and/or veterinary diagnostic laboratory if substantiated. It is the laboratory or veterinarian who should officially notify if the ultimate diagnosis is reportable.

In summary, mandated reporting under the NLRAD should be limited to veterinary diagnostic laboratories that have confirmed the diagnosis of a reportable disease or condition. Veterinarians who have arrived at a confirmed or presumed diagnosis that falls under an established case definition without utilizing a veterinary diagnostic laboratory should also have a duty to report. Alternative mechanisms, distinct from NLRAD, should be developed and/or promoted for laypersons to notify animal health officials of suspicious events.

How it is to be reported: NPPC appreciates that APHIS explored a single portal for reporting, and that not all states have equally robust animal health agencies. We support that APHIS require all veterinary diagnostic laboratories, or veterinarians operating independent of such, report notifiable animal diseases and conditions to the NLRAD electronic portal as proposed upon diagnosis.

It is not clear, however, why APHIS is mandating that report also be made to the state in which the animal is located. APHIS should be in position to ensure that the state is immediately and automatically notified of a report to the NLRAD portal.

We understand, and support that many states do in fact have a reporting requirement for certain animal diseases or conditions. These should stand. Many veterinarians will prefer to notify their state animal health officials of their diagnosis as well as report to the NLRAD. This should also remain a viable option. The federal regulation should dictate federal reporting requirements; state regulations and accepted practice should dictate state reporting requirements.

As with the proposed portal for reporting suspicious animal health events, APHIS should ensure that the reporting system is secure and that confidential and identifying information is protected from public release. Reports should be summarized and provided to the animal industries and other animal health stakeholders promptly— especially if they concern non-endemic diseases or conditions.

In summary, the NLRAD should only mandate reporting to the proposed federal NLRAD portal. APHIS should ensure that the appropriate state agency is immediately notified upon receipt of a report. APHIS should let states determine their own direct reporting requirements for diagnostic laboratories, veterinarians and other animal health professionals under their jurisdiction, as is current practice.

NPPC, AASV and SHIC support the establishment of a NLRAD for notifiable animal diseases and conditions. The NLRAD should consist of a discrete list of named animal diseases and conditions, each with a published case definition and response plan. Reporting requirements should be limited to veterinarians who have a confirmed or presumed diagnosis of a disease or condition named on the NLRAD. The report should be made to the federal NLRAD portal as proposed, with states reserving the right to establish their own reporting requirements. The NLRAD should inform but not replace robust animal health monitoring and surveillance programs.

Monitored Animal Diseases

NPPC, AASV and SHIC can only support mandating state reporting of monitored animal diseases if both APHIS and states have the necessary resources to compile and transmit this information electronically, and if APHIS has the resources to analyze and contextualize this information when making it publicly available or reporting it to the OIE. In proposing the NLRAD, APHIS indicates that it expects up to a tenfold increase in reported data. While this does support the assertation that it will provide a clearer picture of the status of monitored diseases, it will be more resource intensive to manage this amount of data. APHIS should ensure that it can accomplish this without compromising other animal health programs. It also suggests that many, if not most, states are not currently voluntarily providing this information. It is even more critical that states not have to divert resources from other animal health programs to fulfill this reporting requirement. If APHIS is not in position to provide states with funding and other needed resources required to fulfill this mandate, it should not be enacted at this time. In summary, NPPC, AASV and SHIC support the establishment of a NLRAD that consists of named animal diseases and conditions with established case definitions. Reporting requirements for notifiable animal diseases and conditions should be limited to veterinary diagnostic laboratories and veterinarians, and such reports under the federal rule should be made exclusively to the proposed NLRAD portal. Mandated reporting of monitored animal diseases by states should only be enacted if states can be provided the resources needed to facilitate such reporting. APHIS should develop a voluntary portal separate and distinct from the NLRAD for receiving reports of suspicious animal health events.

Thank you for the opportunity to comment on this important topic.

Sincerely,

Harry Snelson, DVM Executive Director American Association of Swine Veterinarians

Liz Wagstrom, DVM, MS, DACVPM Chief Veterinarian National Pork Producers Council

Paul Sundberg, DVM, PhD, DACVPM Executive Director, Swine Health Information Center


U.S. National List of Reportable Animal Diseases (NLRAD) System Standards - Proposed



In summary, NPPC, AASV and SHIC support the establishment of a NLRAD that consists of named animal diseases and conditions with established case definitions. Reporting requirements for notifiable animal diseases and conditions should be limited to veterinary diagnostic laboratories and veterinarians, and such reports under the federal rule should be made exclusively to the proposed NLRAD portal. Mandated reporting of monitored animal diseases by states should only be enacted if states can be provided the resources needed to facilitate such reporting. APHIS should develop a voluntary portal separate and distinct from the NLRAD for receiving reports of suspicious animal health events...end

MY question is, who is to monitor the real threat of chronic wasting disease and or scrapie TSE Prion in Pigs, if it has not happened already and in the food supply chain for human and animals, and who is to debate what that might look like? 

I believe front line inspectors, veterinarians, the ones that see different disease in the field every day SHOULD IMMEDIATELY start to investigate, report, any new livestock animal disease, any delay in reporting would only help spread the disease. there is enough bureaucracy and red tape now, a fine example is separating TSE Prion disease into different disease entities i.e. cwd, scrapie, bse, cpd (CAMEL PRION DISEASE). To bring another disease reporting entity into play would only delay action imo.   

Also, state by state regulations on surveillance, tracing efforts, testing, of livestock disease, there must a a scientific standard for all to follow. you cannot have one state doing one thing, and another state doing something else, just does not work...terry

***> cattle, pigs, sheep, cwd, tse, prion, oh my!

***> In contrast, cattle are highly susceptible to white-tailed deer CWD and mule deer CWD in experimental conditions but no natural CWD infections in cattle have been reported (Sigurdson, 2008; Hamir et al., 2006). 

Sheep and cattle may be exposed to CWD via common grazing areas with affected deer but so far, appear to be poorly susceptible to mule deer CWD (Sigurdson, 2008). In contrast, cattle are highly susceptible to white-tailed deer CWD and mule deer CWD in experimental conditions but no natural CWD infections in cattle have been reported (Sigurdson, 2008; Hamir et al., 2006). It is not known how susceptible humans are to CWD but given that the prion can be present in muscle, it is likely that humans have been exposed to the agent via consumption of venison (Sigurdson, 2008). Initial experimental research suggests that human susceptibility to CWD is low and there may be a robust species barrier for CWD transmission to humans (Sigurdson, 2008), however the risk appetite for a public health threat may still find this level unacceptable.



cwd scrapie pigs oral routes

***> However, at 51 months of incubation or greater, 5 animals were positive by one or more diagnostic methods. Furthermore, positive bioassay results were obtained from all inoculated groups (oral and intracranial; market weight and end of study) suggesting that swine are potential hosts for the agent of scrapie. <*** 

 >*** Although the current U.S. feed ban is based on keeping tissues from TSE infected cattle from contaminating animal feed, swine rations in the U.S. could contain animal derived components including materials from scrapie infected sheep and goats. These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health. <*** 

***> Results: PrPSc was not detected by EIA and IHC in any RPLNs. All tonsils and MLNs were negative by IHC, though the MLN from one pig in the oral <6 month group was positive by EIA. PrPSc was detected by QuIC in at least one of the lymphoid tissues examined in 5/6 pigs in the intracranial <6 months group, 6/7 intracranial >6 months group, 5/6 pigs in the oral <6 months group, and 4/6 oral >6 months group. Overall, the MLN was positive in 14/19 (74%) of samples examined, the RPLN in 8/18 (44%), and the tonsil in 10/25 (40%). 

***> Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. 

This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. 

Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains. 




Friday, December 14, 2012

DEFRA U.K. What is the risk of Chronic Wasting Disease CWD being introduced into Great Britain? A Qualitative Risk Assessment October 2012

snip.....

In the USA, under the Food and Drug Administration's BSE Feed Regulation (21 CFR 589.2000) most material (exceptions include milk, tallow, and gelatin) from deer and elk is prohibited for use in feed for ruminant animals. With regards to feed for non-ruminant animals, under FDA law, CWD positive deer may not be used for any animal feed or feed ingredients. For elk and deer considered at high risk for CWD, the FDA recommends that these animals do not enter the animal feed system. However, this recommendation is guidance and not a requirement by law.

Animals considered at high risk for CWD include:

1) animals from areas declared to be endemic for CWD and/or to be CWD eradication zones and

2) deer and elk that at some time during the 60-month period prior to slaughter were in a captive herd that contained a CWD-positive animal.

Therefore, in the USA, materials from cervids other than CWD positive animals may be used in animal feed and feed ingredients for non-ruminants.

The amount of animal PAP that is of deer and/or elk origin imported from the USA to GB can not be determined, however, as it is not specified in TRACES. It may constitute a small percentage of the 8412 kilos of non-fish origin processed animal proteins that were imported from US into GB in 2011.

Overall, therefore, it is considered there is a __greater than negligible risk___ that (nonruminant) animal feed and pet food containing deer and/or elk protein is imported into GB.

There is uncertainty associated with this estimate given the lack of data on the amount of deer and/or elk protein possibly being imported in these products.

snip.....

36% in 2007 (Almberg et al., 2011). In such areas, population declines of deer of up to 30 to 50% have been observed (Almberg et al., 2011). In areas of Colorado, the prevalence can be as high as 30% (EFSA, 2011).

The clinical signs of CWD in affected adults are weight loss and behavioural changes that can span weeks or months (Williams, 2005). In addition, signs might include excessive salivation, behavioural alterations including a fixed stare and changes in interaction with other animals in the herd, and an altered stance (Williams, 2005). These signs are indistinguishable from cervids experimentally infected with bovine spongiform encephalopathy (BSE).

Given this, if CWD was to be introduced into countries with BSE such as GB, for example, infected deer populations would need to be tested to differentiate if they were infected with CWD or BSE to minimise the risk of BSE entering the human food-chain via affected venison.

snip.....

The rate of transmission of CWD has been reported to be as high as 30% and can approach 100% among captive animals in endemic areas (Safar et al., 2008).

snip.....

In summary, in endemic areas, there is a medium probability that the soil and surrounding environment is contaminated with CWD prions and in a bioavailable form. In rural areas where CWD has not been reported and deer are present, there is a greater than negligible risk the soil is contaminated with CWD prion.

snip.....

In summary, given the volume of tourists, hunters and servicemen moving between GB and North America, the probability of at least one person travelling to/from a CWD affected area and, in doing so, contaminating their clothing, footwear and/or equipment prior to arriving in GB is greater than negligible... For deer hunters, specifically, the risk is likely to be greater given the increased contact with deer and their environment. However, there is significant uncertainty associated with these estimates.

snip.....

Therefore, it is considered that farmed and park deer may have a higher probability of exposure to CWD transferred to the environment than wild deer given the restricted habitat range and higher frequency of contact with tourists and returning GB residents.

snip.....


the feds just released this statement and you should read this very carefully about the mad cow feed ban that never was, and still isn't, and why this is so important, since USDA APHIS ARS Scientist recent transmitted Chronic Wasting Disease CWD TSE Prion, BY ORAL ROUTES, to PIGS AND SHEEP. this is terrible news, and proves the mad cow feed ban never worked, especially since it really never existed;

ponder this; ***> Adriano Aguzzi...''We even showed that a prion AEROSOL will infect 100% of mice within 10 seconds of exposure''

SUNDAY, SEPTEMBER 1, 2019 

FDA Reports on VFD Compliance

Before and after the current Veterinary Feed Directive (VFD) rules took full effect in January, 2017, the FDA focused primarily on education and outreach to help feed mills, veterinarians and producers understand and comply with the requirements. Since then, FDA has gradually increased the number of VFD inspections and initiated enforcement actions when necessary.


***> Wednesday, January 23, 2019 

***> CFIA SFCR Guidance on Specified risk material (SRM) came into force on January 15, 2019 <***


WEDNESDAY, JUNE 10, 2020 

Radical Change in Zoonotic Abilities of Atypical BSE Prion Strains as Evidenced by Crossing of Sheep Species Barrier in Transgenic Mice

Radical Change in Zoonotic Abilities of Atypical BSE Prion Strains as Evidenced by Crossing of Sheep Species Barrier in Transgenic Mice

Atypical BSE prions showed a modification in their zoonotic ability after adaptation to sheep-PrP producing agents able to infect TgMet129 and TgVal129, bearing features that make them indistinguishable of sporadic Creutzfeldt-Jakob disease prions.

our results clearly indicate that atypical BSE adaptation to an ovine-PrP sequence could modify the prion agent to potentially infect humans, showing strain features indistinguishable from those of classic sCJD prions, even though they might or might not be different agents.

However, the expanding range of TSE agents displaying the capacity to transmit in human-PrP–expressing hosts warrants the continuation of the ban on meat and bone meal recycling and underscores the ongoing need for active surveillance


A REVIEW of facts and science on scrapie zoonosis potential/likelihood and the USA incredible failure of the BSE 589.2001 FEED REGULATIONS (another colossal failure, and proven to be a sham) 

1st up BSE 589.2001 FEED REGULATIONS 


MONDAY, JULY 27, 2020 

Experimental study using multiple strains of prion disease in cattle reveals an inverse relationship between incubation time and misfolded prion accumulation, neuroinflammation and autophagy


THURSDAY, JUNE 25, 2020 

First Report of the Potential Bovine Spongiform Encephalopathy (BSE)-Related Somatic Mutation E211K of the Prion Protein Gene (PRNP) in Cattle


MONDAY, JULY 27, 2020 

APHIS USDA Nor98-like scrapie was confirmed in a sheep sampled at slaughter in May 2020


TUESDAY, JULY 28, 2020 

Geographic variation in the PRNP gene and its promoter, and their relationship to chronic wasting disease in North American deer


Camel prion disease

Camelus Spongiform Encephalopathy


MONDAY, JULY 6, 2020 

Guidance for reporting 2020 surveillance data on Transmissible Spongiform Encephalopathies (TSE)


MONDAY, JULY 27, 2020 

BSE Inquiry DFA's a review


friendly fire from all of the above

Volume 26, Number 8—August 2020 

Sporadic Creutzfeldt-Jakob Disease among Physicians, Germany, 1993–2018 high proportion of physicians with sCJD were surgeons


THURSDAY, JULY 02, 2020 

Variant Creutzfeldt–Jakob Disease Diagnosed 7.5 Years after Occupational Exposure


SUNDAY, MAY 26, 2019 

Arguments for Alzheimer’s and Parkinson’s diseases caused by prions Stanley B. Prusiner 

''From a large array of bioassays, we conclude that AD, PD, MSA, and the frontotemporal dementias, including PSP and CBD, are all prion diseases''

snip...

P132 Aged cattle brain displays Alzheimer’s-like pathology that can be propagated in a prionlike manner

Ines Moreno-Gonzalez (1), George Edwards III (1), Rodrigo Morales (1), Claudia Duran-Aniotz (1), Mercedes Marquez (2), Marti Pumarola (2), Claudio Soto (1) 

snip...

These results may contribute to uncover a previously unsuspected etiology surrounding some cases of sporadic AD. However, the early and controversial stage of the field of prion-like transmission in non-prion diseases added to the artificial nature of the animal models utilized for these studies, indicate that extrapolation of the results to humans should not be done without further experiments. 

P75 Determining transmissibility and proteome changes associated with abnormal bovine prionopathy 

Dudas S (1,2), Seuberlich T (3), Czub S (1,2) 

In prion diseases, it is believed that altered protein conformation encodes for different pathogenic strains. Currently 3 different strains of bovine spongiform encephalopathy (BSE) are confirmed. Diagnostic tests for BSE are able to identify animals infected with all 3 strains, however, several diagnostic laboratories have reported samples with inconclusive results which are challenging to classify. It was suggested that these may be novel strains of BSE; to determine transmissibility, brain material from index cases were inoculated into cattle. 

In the first passage, cattle were intra-cranially challenged with brain homogenate from 2 Swiss animals with abnormal prionopathy. The challenged cattle incubated for 3 years and were euthanized with no clinical signs of neurologic disease. Animals were negative when tested on validated diagnostic tests but several research methods demonstrated changes in the prion conformation in these cattle, including density gradient centrifugation and immunohistochemistry. Currently, samples from the P1 animals are being tested for changes in protein levels using 2-D Fluorescence Difference Gel Electrophoresis (2D DIGE) and mass spectrometry. It is anticipated that, if a prionopathy is present, this approach should identify pathways and targets to decipher the source of altered protein conformation. In addition, a second set of cattle have been challenged with brain material from the first passage. Ideally, these cattle will be given a sufficient incubation period to provide a definitive answer to the question of transmissibility. 

=====prion 2018===





***however in 1 C-type challenged animal, Prion 2015 Poster Abstracts 

S67 PrPsc was not detected using rapid tests for BSE.

***Subsequent testing resulted in the detection of pathologic lesion in unusual brain location and PrPsc detection by PMCA only.

*** IBNC Tauopathy or TSE Prion disease, it appears, no one is sure ***

Posted by Terry S. Singeltary Sr. on 03 Jul 2015 at 16:53 GMT


P.9.21

Molecular characterization of BSE in Canada

Jianmin Yang 1 , Sandor Dudas 2 , Catherine Graham 2 , Markus Czub 3 , Tim McAllister 1 , Stefanie Czub 1 1 Agriculture and Agri-Food Canada Research Centre, Canada; 2 National and OIE BSE Reference Laboratory, Canada; 3 University of Calgary, Canada

Background: Three BSE types (classical and two atypical) have been identified on the basis of molecular characteristics of the misfolded protein associated with the disease. To date, each of these three types have been detected in Canadian cattle. Objectives: This study was conducted to further characterize the 16 Canadian BSE cases based on the biochemical properties of there associated PrPres.

Methods: Immuno-reactivity, molecular weight, glycoform profiles and relative proteinase K sensitivity of the PrPres from each of the 16 confirmed Canadian BSE cases was determined using modified Western blot analysis.

Results: Fourteen of the 16 Canadian BSE cases were C type, 1 was H type and 1 was L type. The Canadian H and L-type BSE cases exhibited size shifts and changes in glycosylation similar to other atypical BSE cases. PK digestion under mild and stringent conditions revealed a reduced protease resistance of the atypical cases compared to the C-type cases. N terminal-specific antibodies bound to PrPres from H type but not from C or L type. The C-terminal-specific antibodies resulted in a shift in the glycoform profile and detected a fourth band in the Canadian H-type BSE.

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

Discussion: The C, L and H type BSE cases in Canada exhibit molecular characteristics similar to those described for classical and atypical BSE cases from Europe and Japan.

*** This supports the theory that the importation of BSE contaminated feedstuff is the source of C-type BSE in Canada.

*** It also suggests a similar cause or source for atypical BSE in these countries. ***

see page 176 of 201 pages...tss



*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply;




snip...

Re-Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy 

>>> The only tenable public line will be that "more research is required’’ <<< 

>>> possibility on a transmissible prion remains open<<< 

O.K., so it’s about 23 years later, so somebody please tell me, when is "more research is required’’ enough time for evaluation ? 

Re-Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy 

Nature 525, 247?250 (10 September 2015) doi:10.1038/nature15369 Received 26 April 2015 Accepted 14 August 2015 Published online 09 September 2015 Updated online 11 September 2015 Erratum (October, 2015) 

snip...see full Singeltary Nature comment here; 

Alzheimer's disease

let's not forget the elephant in the room. curing Alzheimer's would be a great and wonderful thing, but for starters, why not start with the obvious, lets prove the cause or causes, and then start to stop that. think iatrogenic, friendly fire, or the pass it forward mode of transmission. think medical, surgical, dental, tissue, blood, related transmission. think transmissible spongiform encephalopathy aka tse prion disease aka mad cow type disease... 

Commentary: Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy





Self-Propagative Replication of Ab Oligomers Suggests Potential Transmissibility in Alzheimer Disease 

*** Singeltary comment PLoS *** 

Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ? 

Posted by flounder on 05 Nov 2014 at 21:27 GMT 


IN CONFIDENCE

5 NOVEMBER 1992

TRANSMISSION OF ALZHEIMER TYPE PLAQUES TO PRIMATES

[9. Whilst this matter is not at the moment directly concerned with the iatrogenic CJD cases from hgH, there remains a possibility of litigation here, and this presents an added complication. 

There are also results to be made available shortly 

(1) concerning a farmer with CJD who had BSE animals, 

(2) on the possible transmissibility of Alzheimer’s and 

(3) a CMO letter on prevention of iatrogenic CJD transmission in neurosurgery, all of which will serve to increase media interest.]




snip...see full text


Terry S. Singeltary Sr.



Posted by at No comments:

Wednesday, August 5, 2020

1996-12-04: BBC - Horizon BSE1 - BSE2 The Invisible Enemy, The British Disease, CWD, sporadic CJD

1996-12-04: BBC - Horizon BSE1 - BSE2 The Invisible Enemy, The British Disease, CWD, sporadic CJD

1996-12-04: BBC - Horizon BSE1 - The Invisible Enemy

https://histodb11.usz.ch/Images/videos/video-007/video-007.html

1996-12-04: BBC - Horizon BSE2 - The Invisible Enemy


1997-11-10: Panorama - The british disease


sporadic CJD, it too linked to mad cow disease? CBC NEWS Jeff Schwan sCJD and CWD




Terry S. Singeltary

Posted by at No comments:

Sunday, May 24, 2020

Cosmetics, Intestines, FDA, BSE, TSE, Prion, and Improving Biosecurity Procedures to Minimize the Risk of Spreading Pathogenic Infections Agents After Carcass Recycling

REVIEW ARTICLE


Front. Microbiol., 22 April 2020 | https://doi.org/10.3389/fmicb.2020.00623

Improving Biosecurity Procedures to Minimize the Risk of Spreading Pathogenic Infections Agents After Carcass Recycling

Pramod Pandey1*, Sriram K. Vidyarthi2,3, Venkata Vaddella1, Chandrasekar Venkitasamy1, Maurice Pitesky1, Bart Weimer1 and Alda F. A. Pires1
  • 1Department of Population Health and Reproduction, University of California, Davis, Davis, CA, United States
  • 2Department of Biological and Agricultural Engineering, University of California, Davis, Davis, CA, United States
  • 3The Morning Star Company, Woodland, CA, United States
Animal proteins are essential elements of human and animal feed chain and improving the safety of human and animal feed requires understanding and controlling of the transmission of infectious agents in food chain. Many pathogenic infectious agents, such as prion protein is known to damage the central nervous system in the cattle. Bovine spongiform encephalopathy (BSE) results from infection agent (prion), and affects number of species such as cattle, human, and cats. In addition, Salmonella, pathogenic E. coli O157:H7, and Listeria monocytogenes were found in animal by-products used in the human and animal feed production. Increased interest in controlling microbial risks in human and animal feed is evidenced by a large number of publications, which highlights the need for examining the animal disposal method such as rendering process and provides a broader perspective of rendering process. While existing practices help greatly in controlling microbial contamination, this overview study showed that additional biosafety measures are necessary to ensure microbial safety in animal feed.
snip...
Further, in 2013, the FDA announced a comment reopening on use of materials derived from cattle in human food and cosmetics. The FDA amended the rule indicating the small intestine of cattle, formerly prohibited cattle material, could be used in human food and cosmetics if the distal ileum was removed by a specified procedure or one that the establishment could demonstrate is equally effective in ensuring complete removal of the distal ileum (US FDA, 2013).

https://europepmc.org/backend/ptpmcrender.fcgi?accid=PMC7188909&blobtype=pdf



Detection of PrPBSE and prion infectivity in the ileal Peyer’s patch of young calves as early as 2 months after oral challenge with classical bovine spongiform encephalopathy 

Ivett Ackermann1 , Anne Balkema‑Buschmann1 , Reiner Ulrich2 , Kerstin Tauscher2 , James C. Shawulu1 , Markus Keller1 , Olanrewaju I. Fatola1 , Paul Brown3 and Martin H. Groschup1* 

Abstract 

In classical bovine spongiform encephalopathy (C-BSE), an orally acquired prion disease of cattle, the ileal Peyer’s patch (IPP) represents the main entry port for the BSE agent. In earlier C-BSE pathogenesis studies, cattle at 4–6 months of age were orally challenged, while there are strong indications that the risk of infection is highest in young animals. In the present study, unweaned calves aged 4–6 weeks were orally challenged to determine the earli‑ est time point at which newly formed PrPBSE and BSE infectivity are detectable in the IPP. For this purpose, calves were culled 1 week as well as 2, 4, 6 and 8 months post-infection (mpi) and IPPs were examined for BSE infectivity using a bovine PrP transgenic mouse bioassay, and for PrPBSE by immunohistochemistry (IHC) and protein misfolding cyclic amplifcation (PMCA) assays. For the frst time, BSE prions were detected in the IPP as early as 2 mpi by transgenic mouse bioassay and PMCA and 4 mpi by IHC in the follicular dendritic cells (FDCs) of the IPP follicles. These data indi‑ cate that BSE prions propagate in the IPP of unweaned calves within 2 months of oral uptake of the agent.

In summary, our study demonstrates for the frst time PrPBSE (by PMCA) and prion infectivity (by mouse bioassay) in the ileal Peyer’s patch (IPP) of young calves as early as 2 months after infection. From 4 mpi nearly all calves showed PrPBSE positive IPP follicles (by IHC), even with PrPBSE accumulation detectable in FDCs in some animals. Finally, our results confrm the IPP as the early port of entry for the BSE agent and a site of initial propagation of PrPBSE and infectivity during the early pathogenesis of the disease. Therefore, our study supports the recommendation to remove the last four metres of the small intestine (distal ileum) at slaughter, as designated by current legal requirements for countries with a controlled BSE risk status, as an essential measure for consumer and public health protection.



Therefore, our study supports the recommendation to remove the last four metres of the small intestine (distal ileum) at slaughter, as designated by current legal requirements for countries with a controlled BSE risk status, as an essential measure for consumer and public health protection.



BSE INQUIRY DFA 18 COSMETICS

Subject: DFA 18 Cosmetics...[There have been reports of BSE outbreaks in Germany, France, and even in the U.S.A., a prime market for Jersey cattle]

From: "Terry S. Singeltary Sr."

Reply-To: Bovine Spongiform Encephalopathy

Date: Mon, 1 Nov 1999 09:28:04 -0600


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

Subject: BSE--U.S. 50 STATE CONFERENCE CALL Jan. 9, 2001

Date: Tue, 9 Jan 2001 16:49:00 -0800

From: "Terry S. Singeltary Sr."

Reply-To: Bovine Spongiform Encephalopathy

To: BSE-L@uni-karlsruhe.de

snip...

[host Richard Barns] and now a question from Terry S. Singeltary of CJD Watch.

[TSS] yes, thank you, U.S. cattle, what kind of guarantee can you give for serum or tissue donor herds?

[no answer, you could hear in the back ground, mumbling and 'we can't. have him ask the question again.]

[host Richard] could you repeat the question?

[TSS] U.S. cattle, what kind of guarantee can you give for serum or tissue donor herds?

[not sure whom ask this] what group are you with?

[TSS] CJD Watch, my Mom died from hvCJD and we are tracking CJD world-wide.

[not sure who is speaking] could you please disconnect Mr. Singeltary

[TSS] you are not going to answer my question?

[not sure whom speaking] NO

snip...see full archive and more of this;



Scientific Advisors and Consultants Staff 2001 Advisory Committee TSE PRION Singeltary Submission Freas Monday, January 08,2001 3:03 PM FDA Singeltary submission 2001 

Greetings again Dr. Freas and Committee Members, 

I wish to submit the following information to the Scientific Advisors and Consultants Staff 2001 Advisory Committee (short version). I understand the reason of having to shorten my submission, but only hope that you add it to a copy of the long version, for members to take and read at their pleasure, (if cost is problem, bill me, address below). So when they realize some time in the near future of the 'real' risks i speak of from human/animal TSEs and blood/surgical products. I cannot explain the 'real' risk of this in 5 or 10 minutes at some meeting, or on 2 or 3 pages, but will attempt here: 

snip...see full text ; 

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

Subject: Prion Scientific Advisors and Consultants Staff Meeting Singeltary Submission Freas Monday, January 08,2001 3:03 PM

PLEASE be aware, my submission here has now been removed from the www, or changed to a different url that no one knows now, and does not come up in search engines anymore, after 17 years...wonder why that could be, i guess the truth just hurt to much$$$ 

Freas, William

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

Sent: Monday, January 08,2001 3:03 PM

TO: freas@CBS5055530.CBER.FDA.GOV

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

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

Greetings again Dr. Freas and Committee Members,

I wish to submit the following information to the Scientific Advisors and Consultants Staff 2001 Advisory Committee (short version).

I understand the reason of having to shorten my submission, but only hope that you add it to a copy of the long version, for members to take and read at their pleasure, (if cost is problem, bill me, address below). So when they realize some time in the near future of the 'real' risks i speak of from human/animal TSEs and blood/surgical products. I cannot explain the 'real' risk of this in 5 or 10 minutes at some meeting, or on 2 or 3 pages, but will attempt here:

remember AIDS/HIV, 'no problem to heterosexuals in the U.S.? no need to go into that, you know of this blunder:

DO NOT make these same stupid mistakes again with human/animal TSE's aka MADCOW DISEASE. I lost my Mom to hvCJD, and my neighbor lost his Mother to sCJD as well (both cases confirmed). I have seen many deaths, from many diseases. I have never seen anything as CJD, I still see my Mom laying helpless, jerking tremendously, and screaming "God, what's wrong with me, why can't I stop this". I still see this, and will never forget. Approximately 10 weeks from 1st of symptoms to death. This is what drives me. I have learned more in 3 years about not only human/animal TSE's but the cattle/rendering/feeding industry/government than i ever wished to.

I think you are all aware of CJD vs vCJD, but i don't think you all know the facts of human/animal TSE's as a whole, they are all very very similar, and are all tied to the same thing, GREED and MAN.

I am beginning to think that the endless attempt to track down and ban, potential victims from known BSE Countries from giving blood will be futile. You would have to ban everyone on the Globe eventually? AS well, I think we MUST ACT SWIFTLY to find blood test for TSE's, whether it be blood test, urine test, .eyelid test, anything at whatever cost, we need a test FAST.

DO NOT let the incubation time period of these TSEs fool you.

To think of Scrapie as the prime agent to compare CJD, but yet overlook the Louping-ill vaccine event in 1930's of which 1000's of sheep where infected by scrapie from a vaccine made of scrapie infected sheep brains, would be foolish. I acquired this full text version of the event which was recorded in the Annual Congress of 1946 National Vet. Med. Ass. of Great Britain and Ireland. from the BVA and the URL is posted in my (long version).

U.S.A. should make all human/animal TSE's notifiable at all ages, with requirements for a thorough surveillance and post-mortem examinations free of charge, if you are serious about eradicating this horrible disease in man and animal.

There is histopathology reports describing o florid plaques" in CJD victims in the USA and some of these victims are getting younger. I have copies of such autopsies, there has to be more. PLUS, sub-clinical human TSE's will most definitely be a problem.

THEN think of vaccineCJD in children and the bovine tissues used in the manufacturing process, think of the FACT that this agent surviving 6OO*C. PNAS -- Brown et al. 97 (7): 3418 scrapie agent live at 600*C

Then think of the CONFIDENTIAL documents of what was known of human/animal TSE and vaccines in the mid to late 80s, it was all about depletion of stock, to hell with the kids, BUT yet they knew. To think of the recall and worry of TSE's from the polio vaccine, (one taken orally i think?), but yet neglect to act on the other potential TSE vaccines (inoculations, the most effective mode to transmit TSEs) of which thousands of doses were kept and used, to deplete stockpile, again would be foolish.

--Oral polio; up to 1988, foetal calf serum was used from UK and New Zealand (pooled); since 1988 foetal calf serum only from New Zealand. Large stocks are held.

--Rubella; bulk was made before 1979 from foetal calf serum from UK and New Zealand. None has been made as there are some 15 years stock.

--Diphtheria; UK bovine beef muscle and ox heart is used but since the end of 1988 this has been sourced from Eire. There are 1,250 litres of stock.

--Tetanus; this involves bovine material from the UK mainly Scottish. There are 21,000 litres of stock.

--Pertussis; uses bovine material from the UK. There are 63,000 litres of stock. --They consider that to switch to a non-UK source will take a minimum of 6-18 months and to switch to a non-bovine source will take a minimum of five years.

3. XXXXXXXXXXX have measles, mumps, MMR, rubella vaccines. These are sourced from the USA and the company believes that US material only is used.

89/2.14/2.1

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

BSE3/1 0251

4. XXXXXXXXXXX have a measles vaccine using bovine serum from the UK. there are 440,000 units of stock. They have also got MMR using bovine serum from the UK.

5. XXXXXXXXXXX have influenza, rubella, measles,' MMR vaccines likely to be used in children. Of those they think that only MMR contains bovine material which is probably a French origin.

6. XXXXXXXXXXX have diphtheria/tetanus and potasses on clinical trial. hese use veal material, some of which has come from the UK and has been ade by XXXXXXXXXXX (see above).

I have documents of imports from known BSE Countries, of ferments, whole blood, antiallergenic preparations,

2 

human blood plasma, normal human blood sera, human immune blood sera, fetal bovine serum, and other blood fractions not elsewhere specified or included, imported glands, catgut, vaccines for both human/animal, as late as 1998. Let us not forget about PITUITARY EXTRACT. This was used to help COWS super ovulate. This tissue was considered to be of greatest risk of containing BSE and consequently transmitting the disease.

ANNEX 6

MEETING HELD ON 8 JUNE 1988 TO DISCUSS THE IMPLICATIONS OF BSE TO BIOLOGICAL PRODUCTS CONTAINING BOVINE - EXTRACTED MATERIAL

How much of this was used in the U.S.?

Please do not keep making the same mistakes; 'Absence of evidence is not evidence of absence'.

What are the U.S. rules for importing and manufacturing vaccines, medicines and medical devices?

Does the U.S.A. allow sourcing of raw material of ruminants from the U.S.A.?

U.S. cattle, what kind of guarantee can you give for serum or tissue donor herds? . The U.S. rendering system would easily amplify T.S.E.'s:

Have we increased the stability of the system (improved heat treatments) since the EU SSC report on the U.S.A. was published in july 2000?

What is done to avoid cross-contaminations in the U.S.A.?

How can the U.S. control absence of cross-contaminations of animal TSE's when pig and horse MBM and even deer and elk are allowed in ruminant feed, as well as bovine blood? I sadly think of the rendering and feeding policy before the Aug. 4, 1997 'partial' feed ban, where anything went, from the city police horse, to the circus elephant, i will not mention all the scrapie infected sheep. I am surprised that we have not included man 'aka soyent green'. It is a disgusting industry and nothing more than greed fuels it.

When will the U.S.. start real surveillance of the U.S. bovine population (not passive, this will not work)?

When will U.S. start removing SRMs?

Have they stopped the use of pneumatic stunners in the U.S.?

If so, will we stop it in all U.S. abattoirs or only in those abattoirs exporting to Europe?

If not, WHY NOT?

same questions for removal of SRM in the U.S.A., or just for export?

If not, WHY NOT?

How do we now sterilize surgical/dental instruments in the U.S.A.?

Where have we been sourcing surgical catgut?

(i have copies of imports to U.S., and it would floor you) hen will re-usable surgical instruments be banned?

'Unregulated "foods" such as 'nutritional supplements' containing various extracts from ruminants, whether imported or derived from

3 

US cattle/sheep/cervids ("antler velvet" extracts!) should be forbidden or at least very seriously regulated. (neighbors Mom, whom also died from CJD, had been taking bovine based supplement, which contained brain, eye, and many other bovine/ovine tissues for years, 'IPLEX').

What is the use of banning blood or tissue donors from Germany, France, etc... when the U.S.A. continues exposing cattle, sheep and people to SRM, refuses to have a serious feed ban, refuses to do systematic BSE-surveillance?

The FDA should feel responsible for the safety of what people eat, prohibit the most dangerous foods, not only prohibit a few more donors - the FDA should be responsible for the safe sourcing of medical devices, not only rely on banning donors "from Europe", The 'real' risks are here in the U.S. as well, and nave been for some time.

We must not forget the studies that have proven infectivity in blood from TSE's.

The Lancet, November 9, 1985

Sir, --Professor Manuelidis and his colleagues (Oct 19, p896) report transmission to animals of Creutzfeldt-Jakob disease (CJD) from the buffy coat from two patients. We also transmitted the disease from, whole blood samples of a patient (and of mice) infected with CJD.l Brain, Cornea, and urine from this patient were also infectious, and the clinicopathological findings2 are summarised as follows.

snip...

Samples,were taken aseptically at necropsy. 10% crude homogenates of brain and cornea in saline, whole blood (after crushing a clot), and untreated CSF and urine were innoculated intracerebrally into CFl strain mice (20 ul per animal). Some mice showed emaciation, bradykinesia, rigidity of the body and tail, and sometimes tremor after long incubation periods. Tissues obtained after the animal died (or was killed) were studied histologically (table). Animals infected by various inocula showed common pathological changes, consisting of severe spongiform changes, glial proliferation, and a moderate loss of nerve cells. A few mice inoculated with brain tissue or urine had the same amyloid plaques found in patients and animals with CJD.3

snip...

Department of Neuropathology,. Neurological Institute, Faculty of Medicine, Kyushu University, Fukuoka812, Japan JUN TATEISHI

(full text-long version)

and

CWD and transmission to man will be no different than other TSE's.

"Clearly, it is premature to draw firm conclusions about CWD passing naturally into humans, cattle and sheep, but the present results suggest that CWD transmissions to humans would be as limited by PrP incompatibility as transmissions of BSE or sheep scrapie to humans. Although there is no evidence that sheep scrapie has affected humans, it is likely that BSE has

4

caused variant CJD in 74 people (definite and probable variant CJD cases to date according to the UK CJD Surveillance Unit). Given the presumably large number of people exposed to BSE infectivity, the susceptibility of humans may still be very low compared with cattle, which would be consistent with the relatively inefficient conversion of human PrP-sen by PrPBSE. Nonetheless, since humans have apparently been infected by BSE, it would seem prudent to take reasonable measures to limit exposure of humans (as well as sheep and cattle) to CWD infectivity as has been recommended for other animal TSEs,"

G.J. Raymond1, A. Bossers2, L.D. Raymond1, K.I. O'Rourke3, L.E. McHolland4, P.K. Bryant III4, M.W. Miller5, E.S. Williams6, M. Smits2 and B. Caughey1,7

or more recently transmission of BSE to sheep via whole blood Research letters Volume 356, Number 9234 16 September 2000

Transmission of BSE by blood transfusion in sheep

Lancet 2000; 356: 999 – 1000

F Houston, J D Foster, Angela Chong, N Hunter, C J Bostock

See Commentary

"We have shown that it is possible to transmit bovine spongiform encephalopathy (BSE) to a sheep by transfusion with whole blood taken from another sheep during the symptom-free phase of an experimental BSE infection. BSE and variant Creutzfeldt-Jakob disease (vCJD) in human beings are caused by the same infectious agent, and the sheep-BSE experimental model has a similar pathogenesis to that of human vCJD. Although UK blood transfusions are leucodepleted--a possible protective measure against any risk from blood transmission-- this report suggests that blood donated by symptom-free vCJD-infected human beings may represent a risk of spread of vCJD infection among the human population of the UK."

"The demonstration that the new variant of Creutzfeldt-Jakob disease (vCJD) is caused by the same agent that causes bovine spongiform encephalopathy (BSE) in cattle1 has raised concerns that blood from human beings in the symptom-free stages of vCJD could transmit infection to recipients of blood transfusions (full text long version)"

and...

"The large number of cases (1040), temporal clustering of the outbreaks (15 in the first 6 months of 1997), the high in-flock incidence, and the exceptional involvement of goats (390 cases), suggested an accidental infection. The source of the epidemic might have been TSE-contaminated meat and bonemeal, but eight flocks had never been fed any commercial feedstuff. Infection might have risen from the use of a formol-inactivated vaccine against contagious agalactia prepared by a single laboratory with brain and mammary gland homogenates of sheep infected with Mycoplasma agalactiae. Although clinical signs of TSE in the donor sheep have not been found, it is possible that one or more of them were harbouring the

5

infectious agent. Between 1995 and 1996, this vaccine was given subcutaneously to 15 of the affected flocks (to one flock in 1994) ; in these animals the disease appeared between 23 and 35 months after vaccination. No information is available for herd 13 because it was made up of stolen animals. Sheep from the remaining three flocks (1-3, figure) did not receive the vaccine, thus suggesting a naturally occurring disease.’’ (again, full text long version).

IN SHORT, please do under estimate this data and or human/animal TSE's including CWD in the U.S.A.

A few last words, please.

The cattle industry would love to have us turn our focus to CWD and forget about our own home grown TSE in Bovines. This would be easy to do. Marsh's work was from downer cattle feed, NOT downer deer/elk feed. This has been proven.

DO NOT MAKE THAT MISTAKE.

There should be NO LESS THAN 1,000,000 tests for BSE/TSE ' in 2001 for U.S.A. French are testing 20,000 a week. The tests are available. Why wait until we stumble across a case from passive surveillance, by then it is to late. IF we want the truth, this is a must???

United States Total ,Bovine Brain Submissions by State,

May 10 ,1990 thru October 31, 2000

Total 11,700

FROM 1.5 BILLION HEAD OF CATTLE since 1990 ???

with same feeding and rendering practices as that of U.K. for years and years, same scrapie infected sheep used in feed, for years and years, 950 scrapie infect FLOCKS in the U.S. and over 20 different strains of scrapie known to date. (hmmm, i am thinking why there is not a variant scrapie, that is totally different than all the rest)? just being sarcastic.

with only PARTIAL FEED BAN implemented on Aug. 4, 1997??? (you really need to reconsider that blood meal etc. 'TOTAL BAN')

http://www.aphis.usda.gov/oa/bse/bsesurv,ey.html#charts

AND PLEASE FOR GODS SAKE, STOP saying vCJD victims are the only ones tied to this environmental death sentence. "PROVE IT". It's just not true. The 'CHOSEN ONES' are not the only ones dying because of this man-made death sentence. When making regulations for human health from human/animal TSEs, you had better include ALL human TSE's, not just vCJD. Do NOT underestimate sporadic CJD with the 'prehistoric' testing available to date. This could be a deadly mistake. Remember, sCJD kills much faster from 1st onset of symptoms to death, and hvCJD is the fastest. Could it just be a higher titre of infectivity, or route or source, or all three?

Last, but not least. The illegal/legal harvesting of body parts and tissues will come back to haunt you. Maybe not morally, but due to NO background checks and human TSEs, again it will continue to spread.

Stupidity, Ignorance and Greed is what fuels this disease. You must stop all of this, and ACT AT ONCE...

Sent: Monday, January 08,2001 3:03 PM

TO: freas@CBS5055530.CBER.FDA.GOV

FDA CJD BSE TSE Prion Scientific Advisors and Consultants Staff January 2001 Meeting Singeltary Submission

2001 FDA CJD TSE Prion Singeltary Submission

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


From: TSS (216-119-138-155.ipset18.wt.net)

Subject: FAT LIPS/SHINY HAIR/Creams (Cosmetics) PRETTY WOMEN $ MOVIE STARS $ MAD COW DISEASE ...

Date: June 10, 2001 at 8:24 am PST

Greetings ALL,

was reading a 'smut' magazine about the 'babes' and came across this article about the different movie stars 'fat lips' (collagen injections). something in the article caught my eye. ONE was Collagen and the other was HASK PLACENTA No-Rinse Treatment. (if containing animal tissues, and then running down into the eye's, seems like a potential transmission route, if you consider kuru and the fact transmission of that TSE agent via topical applications {rubbing of organs etc on skin, cuts etc...TSS}).

""Attention, Goldie Hawn: You might want to forget about more collagen infections for that full-lipped look. Collagen for the procedure usually comes from cows -- as in "mad cow disease". So what's a girl to do? Some docs are using an acid found in roosters' combs instead of collagen. Others use collagen from 'ELITE' herds that don't mix with common bovines. And one scientist is awaiting approval for a human collagen from the foreskin of infant boys -- further proof that beauty is only skin deep""-- 'The National Enquirer' 5/6/01 

are these babes in far a 'rude' awakening. firstly, these so called 'ELITE' herds they speak of, are what they call, 'tissue donor herds', that are suppose to be fed 'only' certain products that _do not_ include ruminant feed of any sort. AND from the exact question i asked at the infamous '50 STATE EMERGENCY CONFERENCE CALL' of Jan, 9, 2001, sadly we find, there is absolutley, NO SUCH THING. It was all a joke. The 'partial' ruminant to ruminant feed ban of August 4, 1997, never was enforced, and most knew nothing about it, and/or chose to ignore it. 



THURSDAY, FEBRUARY 25, 2016 

U.S. Food & Drug Administration (FDA) FDA/CFSAN Cosmetics Update: Cosmetics Program; Import and Domestic and Transmissible Spongiform Encephalopathy TSE Prion Disease Risk Factors 

***WARNING TO ALL CONSUMERS AND COUNTRIES AROUND THE WORLD*** 

***Note: FDA labs do not conduct BSE analysis and thus no sampling guidance is issued for BSE. *** 

*** The basis for any regulatory action on a cosmetic product with respect to prohibited cattle material relies on review of records as described above, labeling and other documentation.*** 

U.S. Food & Drug Administration (FDA) FDA/CFSAN Cosmetics News Update: Cosmetics Program; Import and Domestic and Transmissible Spongiform Encephalopathy TSE Prion Disease Risk Factors


-------- Original Message -------- 

Subject: Docket No. 2004N-0081 and or RIN number RIN-0910-AF47 Use of Materials Derived From Cattle in Human Food and Cosmetics [comment submission] 

Date: Tue, 13 Jul 2004 16:08:38 -0500 

From: "Terry S. Singeltary Sr." To: fdadockets@oc.fda.gov 

CC: burt.pritchett@fda.gov, Agriculture@mail.house.gov 

COMMENT SUBMISSION [Docket No. 2004N-O081] RIN-0910--AF47 Use of Materials Derived From Cattle in Human Food and Cosmetics http://www.fda.gov/OHRMS/DOCKETS/98fr/04n-0081-nir0001.pdf

Greetings FDA,

I would kindly like to comment on the potential for TSE transmission from cosmetics to humans and why I think that ALL animal by-products should be excluded from cosmetics. IF we look at the TSE 'KURU'.



----- Original Message ----- 

From: Terry S. Singeltary Sr. 

To: fdadockets@oc.fda.gov 

Sent: Wednesday, September 07, 2005 9:44 PM 

Subject: Use of Materials Derived From Cattle in Human Food and Cosmetics [Docket No. 2004N-0081] RIN 0910-AF47 

Greetings FDA, 

I would kindly like to comment on ; 

Use of Materials Derived From Cattle in Human Food and Cosmetics [Docket No. 2004N-0081] RIN 0910-AF47


THURSDAY, APRIL 17, 2008 

Use of Materials Derived From Cattle in Human Food and Cosmetics [Docket No. 2004N-0081] RIN 0910-AF47 [Federal Register: April 17, 2008 (Volume 73, Number 75)] [Rules and Regulations] [Page 20785-20794] From the Federal Register Online via GPO Access [wais.access.gpo.gov] [DOCID:fr17ap08-7]



Monday, February 01, 2010

Import Alert 17-04 BSE CJD HIGH RISK TISSUES, Nutritional Supplements and Cosmetics


WEDNESDAY, JUNE 21, 2017 

Docket No. FDA– 2009–N–0505 Agency Information Collection Activities; Proposed Collection; Recordkeeping and Reporting Requirements for Human Food and Cosmetics Manufactured From, Processed With, or Otherwise Containing Material From Cattle Docket No. FDA– 2009–N–0505 


FRIDAY, OCTOBER 25, 2019 

27th ANNUAL REPORT 2018 CREUTZFELDT-JAKOB DISEASE SURVEILLANCE


SUNDAY, SEPTEMBER 1, 2019 

FDA Reports on VFD Compliance

Before and after the current Veterinary Feed Directive (VFD) rules took full effect in January, 2017, the FDA focused primarily on education and outreach to help feed mills, veterinarians and producers understand and comply with the requirements. Since then, FDA has gradually increased the number of VFD inspections and initiated enforcement actions when necessary.


Chronic Wasting Disease CWD TSE Prion USA update

SATURDAY, MAY 16, 2020 

Ohio Chronic Wasting Disease Detected on Wayne County Farm 


ZOONOSIS OF SCRAPIE TSE PRION

O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations 

Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni, Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys Atomic Energy Commission; Fontenay-aux-Roses, France 

Prion diseases (PD) are the unique neurodegenerative proteinopathies reputed to be transmissible under field conditions since decades. The transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that an animal PD might be zoonotic under appropriate conditions. Contrarily, in the absence of obvious (epidemiological or experimental) elements supporting a transmission or genetic predispositions, PD, like the other proteinopathies, are reputed to occur spontaneously (atpical animal prion strains, sporadic CJD summing 80% of human prion cases). 

Non-human primate models provided the first evidences supporting the transmissibiity of human prion strains and the zoonotic potential of BSE. Among them, cynomolgus macaques brought major information for BSE risk assessment for human health (Chen, 2014), according to their phylogenetic proximity to humans and extended lifetime. We used this model to assess the zoonotic potential of other animal PD from bovine, ovine and cervid origins even after very long silent incubation periods. 

*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period, 

***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014), 

***is the third potentially zoonotic PD (with BSE and L-type BSE), 

***thus questioning the origin of human sporadic cases. 

We will present an updated panorama of our different transmission studies and discuss the implications of such extended incubation periods on risk assessment of animal PD for human health. 

=============== 

***thus questioning the origin of human sporadic cases*** 

=============== 

***our findings suggest that possible transmission risk of H-type BSE to sheep and human. Bioassay will be required to determine whether the PMCA products are infectious to these animals. 

============== 


***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice. 

***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. 

***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. 


***> Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility. <*** 


SATURDAY, JUNE 23, 2018

CDC 

***> Diagnosis of Methionine/Valine Variant Creutzfeldt-Jakob Disease by Protein Misfolding Cyclic Amplification 

Volume 24, Number 7—July 2018 Dispatch


Monday, April 20, 2020 

PRION2020 POSTPONED TO 2021 – DUE TO CORONAVIRUS (COVID-19)


MONDAY, AUGUST 26, 2019

Creutzfeldt Jakob Disease CJD, TSE, Prion, Surveillance Update August 2019


SUNDAY, MARCH 10, 2019 

National Prion Disease Pathology Surveillance Center Cases Examined¹ Updated Feb 1, 2019 Variably protease-sensitive prionopathy VPSPr


***> In conclusion, sensory symptoms and loss of reflexes in Gerstmann-Sträussler-Scheinker syndrome can be explained by neuropathological changes in the spinal cord. We conclude that the sensory symptoms and loss of lower limb reflexes in Gerstmann-Sträussler-Scheinker syndrome is due to pathology in the caudal spinal cord. <***

***> The clinical and pathological presentation in macaques was mostly atypical, with a strong emphasis on spinal cord pathology.<*** 

***> The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD. <***

***> All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals.<*** 

***> In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids.'' Scientific opinion on chronic wasting disease (II) <***


THURSDAY, JANUARY 30, 2020 

Docket Number: FDA-2012-D-0307 Recommendations to Reduce the Possible Risk of Transmission of Creutzfeldt-Jakob Disease and Variant Creutzfeldt-Jakob Disease by Blood and Blood Components; Draft Guidance for Industry Draft Guidance for Industry Singeltary Submission


FRIDAY, JANUARY 31, 2020

CJD TSE Prion Blood Products, iatrogenic transmission, Confucius is confused again, WHAT IF? Docket Number: FDA-2012-D-0307


Updated April 3, 2020

Year Total Referrals² Prion Disease Sporadic Familial Iatrogenic vCJD

1999 & earlier 381 230 200 27 3 0

2000 145 102 90 12 0 0

2001 209 118 110 8 0 0

2002 241 144 124 18 2 0

2003 259 160 137 21 2 0

2004 316 181 164 16 0 1³

2005 327 178 156 21 1 0

2006 365 179 159 17 1 2⁴

2007 374 210 191 19 0 0

2008 384 221 205 16 0 0

2009 397 231 210 20 1 0

2010 401 246 218 28 0 0

2011 392 238 214 24 0 0

2012 413 244 221 23 0 0

2013 416 258 223 34 1 0

2014 355 208 185 21 1 1⁵

2015 401 263 243 20 0 0

2016 396 277 248 29 0 0

2017 375 266 247 19 0 0

2018 309 223 204 18 1 0

2019 416 270 240 21 0 0

2020 84 56 21 2 0 0

TOTAL 73566 45037 40108 4349 13 4

1Listed based on the year of death or, if not available, on the year of referral; 

2Cases with suspected prion disease for which brain tissue was submitted; 

3Disease acquired in the United Kingdom; 

4Disease acquired in the United Kingdom in one case and in Saudi Arabia in the other; 

5Disease possibly acquired in a Middle Eastern or Eastern European country; 

6Includes 14 cases in which the diagnosis is pending, and 19 inconclusive cases; 

7Includes 42 (9 from 2019, 33 from 2020) cases with type determination pending in which the diagnosis of vCJD has been excluded. 

8The sporadic cases include 3906 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 69 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 35 cases of sporadic Fatal Insomnia (sFI). 

9Total does not include 272 Familial cases diagnosed by blood test only.


Monday, February 3, 2020 

Informing Patient Contacts About Iatrogenic Creutzfeldt Jakob Disease


Creutzfeldt Jakob Disease CJD 


 SUNDAY, DECEMBER 29, 2019 

Variant CJD 18 years of research and surveillance Variant CJD

18 years of research and surveillance


FRIDAY, MAY 22, 2020 

No Adaptation of the Prion Strain in a Heterozygous Case of Variant Creutzfeldt-Jakob Disease Volume 26, Number 6—June 2020 


BSE INQUIRY

Volume 2: Science 

4. The link between BSE and vCJD 

Summary 

4.29 The evidence discussed above that vCJD is caused by BSE seems overwhelming. Uncertainties exist about the cause of CJD in farmers, their wives and in several abattoir workers. It seems that farmers at least might be at higher risk than others in the general population. 1 Increased ascertainment (ie, increased identification of cases as a result of greater awareness of the condition) seems unlikely, as other groups exposed to risk, such as butchers and veterinarians, do not appear to have been affected. The CJD in farmers seems to be similar to other sporadic CJD in age of onset, in respect to glycosylation patterns, and in strain-typing in experimental mice. Some farmers are heterozygous for the methionine/valine variant at codon 129, and their lymphoreticular system (LRS) does not contain the high levels of PrPSc found in vCJD. It remains a remote possibility that when older people contract CJD from BSE the resulting phenotype is like sporadic CJD and is distinct from the vCJD phenotype in younger people...END..TSS

Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. 

JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA 

Diagnosis and Reporting of Creutzfeldt-Jakob Disease To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally. 

Terry S. Singeltary, Sr Bacliff, Tex 1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. 


Terry S. Singeltary Sr.

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