Chronic Wasting Disease
Saturday, December 09, 2006 Chronic Wasting Disease
***UPDATE JANUARY 20, 2007
Increase of chronic-wasting in wild deer in Alberta & Saskatchewan
Jan, 15 2007 - 3:30 PM
EDMONTON - More cases of deadly chronic-wasting disease in wild deer are showing up in Saskatchewan and Alberta.
Saskatchewan officials say already 30 deer shot by hunters this fall have tested positive for the fatal disease.
In Alberta, there have been four cases since the fall in areas near the Saskatchewan boundary.
Officials in both provinces report some of the cases are showing up in new areas - a finding that suggests the disease may be spreading.
Scientists and wildlife officials are working to contain the disease before it ravages the deer population and jumps to other species, such as caribou.
Tests on about 450 white-tailed deer shot this fall in northwestern Ontario have so far come back negative. (BN, ccg)
http://www.630ched.com/news/news_local.cfm?cat=7428218912&rem=56168&red=80121823aPBIny&wids=410&gi=1&gm=news_local.cfm
SASKATCHEWAN
CWD Sample Totals Total Heads Submitted: 4280 As of January 9, 2007, hunters have turned in 1717 heads from white-tailed deer; 2447 heads from mule deer and 116 elk heads to test for CWD. Total Head Tested: So far 2821 heads have been tested with 26 confirmed positive results for CWD.
http://www.se.gov.sk.ca/fishwild/cwd_info.htm
ALBERTA
Monthly and Cumulative CWD Testing Totals in 2006
*Antelope, reindeer, moose **All results are negative, except for four wild mule deer detected in February 2006, one wild mule deer detected in March 2006, two wild mule deer detected in April 2006 and three wild mule deer confirmed by the CFIA detected in April 2006; TOTAL = 10 (9 cases from Alberta and 1 from Saskatchewan during the winter programs).
http://www1.agric.gov.ab.ca/$department/deptdocs.nsf/all/cpv9448
http://www1.agric.gov.ab.ca/$department/deptdocs.nsf/all/agdex3594
Subject: REPORT OF THE COMMITTEE ON CAPTIVE WILDLIFE AND ALTERNATIVE LIVESTOCK annual report (CWD) 2006 Date: January 15, 2007 at 7:37 pm PST
REPORT OF THE COMMITTEE ON CAPTIVE WILDLIFE AND ALTERNATIVE LIVESTOCK annual report (CWD) 2006
snip...
Drs. Dean Goeldner and Tom Gidlewski, VS, APHIS, USDA, presented the APHIS-VS chronic wasting disease (CWD) program update. CWD has been discovered in free-ranging cervids in 11 states and 41 captive cervid herds in nine states. There are currently four infected elk herds and one infected white-tailed deer herd that have chosen to remain under quarantine instead of depopulate. In 2006, the CWD program depopulated one elk herd in the endemic area which turned out to be infected as well as a chronically infected white-tailed deer herd and a mixed elk and white-tailed deer herd for a total of approximately 110 animals. For the last three years, the program has paid for testing about 15,000 captive cervids per year. Demand for testing is expected to increase with the implementation of the program. The first infected free-ranging white-tailed deer was found in northwest Kansas in 2006. On the positive side, New York found no additional positive free-ranging cervids in 2006 but West Virginia found four additional animals in Hampshire County. Wisconsin continues to aggressively battle CWD with over 100,000 animals submitted for testing since 2000 and over 650 positive deer identified. The infected area appears to be largely limited to the original counties. Interestingly, the number of deer in the Wisconsin endemic area does not appear to be decreasing despite the large number of animals that have been removed. Colorado has stopped culling deer in "hot spots" as they believe that it was not very successful. Alberta,
Canada continues to find more positive white-tailed deer adjacent to the infected Saskatchewan areas.
Appropriate tissue collection and submission for CWD diagnosis includes obex, medial retropharygeal lymph nodes and palatine tonsils. Submission of an ear with the official eartag attached or submission of fresh tissue accompanied by an appropriately executed chain of evidence document will allow DNA comparison in the event of a positive diagnosis. Archiving herd blood samples on special collection cards is also a way to compare DNA in the event of a positive diagnosis in the future. All positive cases are verified by two pathologists and the presumptive positive tissues are completely retested at the National Veterinary Services Laboratory (NVSL). Rectal biopsy continues to be examined as a tool for CWD ante-mortem diagnosis. Hundreds of animals have been examined and the results look promising. Larger numbers need to be examined in order to make final conclusions. Retrospective epidemiologic analysis and transgenic mouse research in 2006 still support the theory that CWD does not appear to affect people or non-cervids animals.
APHIS received approximately $18.5 million in appropriated CWD funding in FY 2006 including $2.44 million in congressional earmarks. The FY 2007 appropriations have not been passed by Congress; the president’s budget requests $15.4 million for CWD. On July 21, 2006, APHIS published its final CWD rule. The final rule added moose and all Cervus species to the previously announced deer and elk species covered in the herd certification program. It expanded the term "captive" to "farmed and captive", maintained a five-year surveillance standard for surveillance, clarified that two positive official tests are needed for a CWD diagnosis, reduced the minimum testing age to 12 months, adjusted commingling buffers, eliminated the 48-hour exemption for short-term commingling, changed the identification (ID) requirement to one official ID and one ID unique within the herd, and added the reporting of escapes and disappearances. Grandfathering of state programs will be accomplished through Memorandum of Understanding (MOUs) with the states followed by reviews of state programs for consistency with federal requirements. The interstate movement requirements maintained a "ramping up" process to reach the five year surveillance standard. An exemption was created for direct movement to slaughter. A permit will be required for interstate movement of research animals and two IDs will be required for wild cervids captured for translocation and release. Subsequent to publication of the rule, three petitions were received from organizations representing state agencies and officials challenging the interstate movement provisions in the rule and requesting a stay in the rule’s implementation. The petitions challenged the scientific basis for initially allowing the interstate movement of animals with only one or two years of surveillance. They also took issue with the federal preemption language in the rule. According to USDA legal counsel, federal preemption on interstate movement is implicit in all APHIS regulations; it was made explicit in this case in response to a comment on the proposed rule. Nevertheless, APHIS believes the petitions merit further consideration. On September 8, 2006, APHIS published a notice of delay of implementation for the rule. The petitions will be published soon for public comment. APHIS intends to resolve the issues quickly so that a final rule can be implemented as the state-federal-industry program it is intended to be.
Dr. Robert Kunkle, National Animal Disease Center (NADC), Agricultural Research Center (ARS), USDA, presented a time-specific Committee paper entitled "Experimental Transmission of Chronic Wasting Disease (CWD) of Elk (Cervus elapus nelsoni), White-tailed Deer
(Odocoileus virginianus), and Mule Deer (Odocoileus hemionus hemionus) to White-tailed Deer by Intracerebral Route. This paper is included in its entirety in these proceedings.
Dr. Michael Miller, Senior Wildlife Veterinarian, Colorado Division of Wildlife, provided an overview of recent progress in understanding various aspects of chronic wasting disease (CWD) epidemiology, diagnosis, and control. Dr. Miller used the occurrence of CWD in a moose to hypothesize that the potential natural host range of CWD may be predicted based on similarities between the native prion protein of known hosts (deer, wapiti, and moose) and other cervid species. He also reviewed findings related to CWD transmission and showed that simulation models of epidemic dynamics based on relatively simple transmission assumptions suggest that CWD is likely to persist in wild deer populations and depress population performance over time. Dr. Miller next described highlights of a new study on PrPCWD distribution in experimentally-infected mule deer that demonstrated marked genetic effects on CWD progression but not susceptibility in this species, and discussed the potential implications for CWD epidemiology. He then shared preliminary data on use of rectal mucosa biopsy to detect CWD infections in live white-tailed and mule deer, which suggest that rectal biopsy likely will be a useful herd screening test and surveillance tool provided PrP genotype data are available for sampled individuals. Dr. Miller concluded his presentation with a brief summary of unsuccessful attempts to control CWD in north central Colorado, emphasizing the challenges and obstacles that likely make eradication of CWD from the wild infeasible given present technology.
snip...
Dr. Keith Rohr presented a resolution on "The use of the ELISA test to diagnose Chronic Wasting Disease in Captive Wildlife". After discussion and modification to the original submission, the resolution was passed by the Committee and will be referred to the Committee on Nominations and Resolutions. Resolution passed by the Committee and referred to the Committee on Nominations and Resolution.
Experimental Transmission of Chronic Wasting Disease (CWD) of Elk (Cervus elaphus nelsoni), White-tailed Deer (Odocoileus virginianus), and Mule Deer (Odocoileus hemionus hemionus) to White-tailed Deer by Intracerebral Route
R.A. Kunkle, A.N. Hamir, J.M. Miller, J.A. Richt, J.J. Greenlee
National Animal Disease Center
Agriculture Research Center
United States Department of Agriculture
S.M. Hall
National Veterinary Services Laboratories, USDA-VS-APHIS
Animal and Plant Health Inspection Service
Veterinary Services
United States Department of Agriculture
E.S. Williams
Wyoming Veterinary Laboratory
University of Wyoming
Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy (TSE) affecting elk, white-tailed deer, and mule deer. Intra-species transmission of CWD is readily accomplished via oral administration of CWD-affected brain, and while the exact mode of natural transmission is unclear, horizontal transmission within species has been demonstrated.
The TSE’s are prion-associated diseases. Different prion strains are associated with variations in clinical course and pathology in susceptible animal hosts. To determine the potential existence of CWD pathotype strain differences, groups of five white-tailed deer were inoculated by intracerebral route (IC) with 1 ml of 10% (wt/vol) brain homogenates derived from CWD-affected elk, white-tailed deer, or mule deer. Two non-inoculated deer served as negative controls. All deer were homozygous at PrP gene polymorphic sites 95 (glutamine) and 138 (serine). Deer homozygous (glycine/glycine) or heterozygous (glycine/serine) at codon 96 were approximately equally divided between treatment groups. One deer from each treatment group was euthanized 10 months post-inoculation (PI); findings for these three deer were similar and included limited or mild spongiform encephalopathy (SE) and immunohistochemical (IHC) detection of prion in lymphoid tissue follicles and in the CNS, especially in subependymal areas. All remaining deer were euthanized at the terminal stage of disease. The clinical course of CWD appeared similar between groups. The survival period did not differ between groups, ranging from 14 to 26 months, with an average mean of 20 months. The severity of SE and magnitude of IHC staining appeared proportional to incubation period. Microscopic lesions in the CNS were typical of previously reported CWD SE, including the presence of cerebral florid plaques. IHC staining was multifocally extensive to diffuse, and was perineuronal, subependymal, and neuropil associated. Staining was pronounced in the midbrain, but relatively sparse in the hippocampus. Differences in histopathologic and IHC findings between groups were not noted. Negative control deer sacrificed at 26 months PI did not have SE and were IHC negative. The composite findings indicate the clinical course and pathology of CWD in IC challenged white-tailed deer was not influenced by species of the inoculum source or by PrP gene polymorphism at codon 96 in recipients.
http://www.usaha.org/committees/reports/2006/report-cwal-2006.pdf
UNITED STATES ANIMAL HEALTH ASSOCIATION – 2006
RESOLUTION NUMBER: 13 APPROVED
SOURCE: COMMITTEE ON CAPTIVE WILDLIFE
AND ALTERNATIVE LIVESTOCK
SUBJECT MATTER: THE USE OF THE ENZYME LINKED IMMUNOSORBENT ASSAY (ELISA) TEST TO DIAGNOSE CHRONIC WASTING DISEASE IN CAPTIVE WILDLIFE
DATES: MINNEAPOLIS, MINNESOTA – OCTOBER 12-18, 2006
BACKGROUND INFORMATION:
The enzyme-linked immunosorbent assay (ELlSA) for chronic wasting disease (CWD) is approved and licensed for free roaming mule deer, white tailed deer and elk. There is ample data indicating essentially equal sensitivity and specificity of ELISA tests compared to immunohistochemistry (IHC). The ELISA test can be done with faster turnaround times and is more efficient for the laboratory and requires fewer personnel than IHC. The ELISA test positives can be confirmed by IHC conducted by laboratory personnel who are experienced in identifying the obex and lymph node tissue to ensure proper tissue submission. More timely laboratory results are needed for producers to move animal product, to verify CWD status and for proper disposal of potentially CWD positive animals.
RESOLUTION:
The United States Animal Health Association (USAHA) requests that the United States Department of Agriculture (USDA), Animal and Plant Health Inspection Service (APHIS), Veterinary Services (VS) approve the USDA licensed enzyme-linked immunosorbent assay (ELISA) test for use on cervid species within the captive wildlife industry.
http://www.usaha.org/committees/resolutions/2006/resolution13-2006.pdf
Drs. Dean Goeldner and Tom Gidlewski, VS-APHIS-USDA provided updated information to the committee regarding APHIS-VS efforts directed at chronic wasting disease (CWD). CWD has been discovered in free-ranging cervids in 11 states and in 41 captive cervid herds in nine states. There are currently four infected elk herds and one infected white-tailed deer herd that have chosen to remain under quarantine instead of depopulate.
In 2006, the CWD program depopulated one elk herd in the endemic area, which turned out to be infected, as well as a chronically infected white-tailed deer herd and a mixed elk and white-tailed deer herd for a total of approximately 110 animals. For the last three years, the program has paid for testing about 15,000 captive cervids per year. Demand for testing is expected to increase with the implementation of the program.
Rectal biopsy continues to be examined as a tool for CWD ante-mortem diagnosis. Hundreds of animals have been examined and the results look promising. Larger numbers need to be examined in order to make final conclusions.
On July 21, 2006, APHIS published its final CWD rule. Subsequently three petitions were received from organizations representing state agencies and officials challenging the interstate movement provisions in the rule and requesting a stay in the rule’s implementation. Believing the petitions merit further consideration, APHIS published a notice of delay of implementation for the rule on September 8, 2006. The petitions will be published soon for public comment.
APHIS received approximately $18.5 million in appropriated CWD funding in FY 2006 including $2.44 million in congressional earmarks. The FY 2007 appropriations have not been passed by Congress; the president’s budget requests $15.4 million for CWD. APHIS again made $5 million in CWD cooperative agreement funding available to state wildlife agencies in FY 2006. The formula for distributing the funds was revised after consultation with the Association of Fish and Wildlife Agencies. Forty-nine states applied for and received funding. APHIS also provided $750,000 for tribal CWD activities, the funding going to the Native American Fish and Wildlife Society and 20 individual tribes. After internal discussions, it was decided to leave the state and tribal wildlife cooperative agreements for CWD on a fiscal year basis, rather than moving them to a calendar year basis in FY 2007 with other VS agreements.
As some states reduce the amount of hunter-killed surveillance for CWD, APHIS is urging those states to utilize targeted and road-killed surveillance to increase the likelihood of detecting the disease.
Dr. Michael Miller, Senior Wildlife Veterinarian with the Colorado Division of Wildlife, provided a brief and lucid overview of recent progress and remaining needs in understanding various aspects of chronic wasting disease (CWD) epidemiology, diagnosis, and control. Dr. Miller alerted committee members to several upcoming research publications on CWD epidemic dynamics, host range, prion distribution patterns, management efforts, and new antemortem diagnostic approaches, and posed five questions that he considers most important to answer in future research: "Can we reliably predict the host range of CWD using non-experimental approaches?" "Why is CWD transmission so efficient?" "Can CWD foci arise from natural exposure to the scrapie agent?" "Does CWD affect populations and ecosystems?" "What can we reasonably do to control CWD?"
The remainder of Dr. Miller’s presentation focused on opportunities for improving the efficiency of surveillance to detect new CWD foci in free-ranging wildlife by using structured, non-random sampling approaches. This strategy has been adopted by the World Organziation for Animal Health (OIE) and accepted internationally as the standard for bovine spongiform encephalopathy (BSE) surveillance. As outlined in the OIE International Animal Health Code ("Surveillance for bovine spongiform encephalopathy", Appendix 3.8.4, OIE 2006, http://www.oie.int/eng/normes/mcode/en_chapitre_3.8.4.htm), the structured, non-random
sampling strategy developed for BSE uses a point-based quota system that weights sample sources based on the likelihood of detecting disease in that subpopulation. This contrasts to the standard random sampling paradigm presently used as the basis for most CWD surveillance programs, wherein all sample sources are assumed equal with respect to disease detection probability and epidemiological "value." According to Dr. Miller, the main advantage of OIE’s structured, non-random sampling approach is that it would allow agencies to combine several survey approaches (e.g., targeted surveillance, vehicle-kill surveys, and harvest surveys) in a straightforward and epidemiologically meaningful way, adding value for "high risk" samples (e.g., cervids showing clinical sighs of CWD). Dr. Miller suggested that we already have sufficient knowledge about CWD epidemiology to adopt this approach to ongoing surveillance. Critical elements would be defining target populations based on biological criteria, choosing desired prevalence thresholds for detection of foci, establishing a timeframe for sample collection based on natural disease course, calculating "point" quotas per target population based on prevalence thresholds, and assigning values to subpopulation (source) samples based on likelihood of infection as reflected published field data (e.g., males > females, middle-aged individuals > young, clinical suspects > vehicle kills > harvested animals). Dr. Miller concluded by pointing out that although the most immediate applications of this structured, non-random sampling surveillance approach might be in CWD surveillance, similar strategies could be devised to improve the efficiency of surveillance for detecting foci of other diseases like bovine tuberculosis or avian influenza in free-ranging wildlife populations.
Dr. Kevin Keel, Southeastern Cooperative Wildlife Disease Study reported that on September 9, 2005 the West Virginia Division of Natural Resources announced that a two-and-a-half year-old buck was determined to be positive for CWD. This deer was found in Hampshire County in close proximity to Virginia, Maryland and Pennsylvania. Special collection teams were dispatched to Hampshire County to collect deer within five miles of the index case. Of the 195 deer sampled from September 14 to October 14, four additional deer were found to be positive for CWD. Subsequently, during the first three days of the firearms seasons, 1,016 hunter-killed deer were sampled from throughout Hampshire County; however, none of the hunter-killed deer tested positive. A second special collection of 80 deer within one mile of the known positives led to the detection of four additional deer that were positive for CWD.
Initial observations suggested that the distribution of hunter-killed samples was relatively uniform throughout the County. However an evaluation of the number of hunter-killed samples at close proximity to the known positives revealed the statistical limitations of the sample size. The 256 samples collected within five miles of known positives are sufficient to detect CWD at a prevalence of 1.2% with a 95% confidence interval. However, the 17 hunter-killed samples collected within one mile of known positives were only sufficient to detect CWD at a prevalence of 16% or greater with a 95% confidence interval. The special collections resulted in the sampling of 146 deer in the one-mile margin. The total of 166 deer sampled within this region would be 95% certain to detect CWD at a prevalence of 1.5% or greater. The data available suggest that CWD is currently confined to a very small region in Hampshire County.
http://www.usaha.org/committees/reports/2006/report-wd-2006.pdf
Chronic Wasting Disease
Chronic Wasting Disease (CWD), a TSE, is a fatal neurological disease of
farmed and wild deer and elk in North America. CWD is generally similar to BSE,
and is thought to be caused by a similar type of infectious prion protein. CWD
differs from BSE in a number of significant ways, however, including the types of
tissues involved and the fact that it is contagious among animals in a herd. A study
under experimental conditions suggested that CWD may be transmissible through
contaminated environments long after infectious animals were no longer present.110
Since 1997, CWD has been detected in captive cervid herds in nine states:
Colorado, Kansas, Minnesota, Montana, Nebraska, New York, Oklahoma, South
Dakota, and Wisconsin. As of July 2006, there were six known positive captive
herds in the United States: four elk herds in Colorado, and one deer herd each in
Wisconsin and Minnesota. Federal and state policy is to depopulate (destroy) these
herds (see below). CWD has been detected in wild cervids in 11 states: Colorado,
Illinois, Kansas, Nebraska, New Mexico, New York, South Dakota, Utah, West
Virginia, Wisconsin, and Wyoming.111 It has also been found in Canada and the
Republic of Korea.
The Centers for Disease Control and Prevention (CDC) says regarding the
potential for CWD transmission to humans:
It is generally prudent to avoid consuming food derived from any animal with
evidence of a TSE (a “transmissible spongiform encephalopathy,” or prion
disease such as BSE and CWD). To date, there is no evidence that CWD has
been transmitted or can be transmitted to humans under natural conditions.
However, there is not yet strong evidence that such transmissions could not
occur. To further assess the possibility that the CWD agent might occasionally
cause disease in humans, additional epidemiologic and laboratory studies could
be helpful. Such studies include molecular characterization and strain typing of
the agents causing CWD in deer and elk and CJD (the human form of prion
disease) in potentially exposed patients. Ongoing national surveillance for CJD
and other neurological cases will remain important for continuing to assess the
risk, if any, of CWD transmission to humans.112
CRS-50
113 Ibid.
114 FDA, Guidance for Industry #158: Use of Material from Deer and Elk in Animal Feed,
Sept. 15, 2003, at [http://www.fda.gov/cvm/Documents/guide158.pdf].
115 See ARS research website to search for CWD studies at [http://www.ars.usda.gov/
Research/Research.htm].
116 USDA/ DOI, Plan for Assisting States, Federal Agencies, and Tribes in Managing
Chronic Wasting Disease in Wild and Captive Cervids, June 26, 2002.
117 71 Federal Register 41682.
With regard to the potential for CWD transmission to cattle, possibly causing
BSE or a related disease that could pose a food safety hazard, USDA says:
During the approximately two decades of monitoring, researchers have not found
any evidence that CWD can be transmitted to domestic cattle under natural
conditions. Ongoing experiments involving oral exposure and contact exposure
on heavily CWD contaminated sites have not resulted in infection of cattle.
These experiments, however, require additional time before they are completed.
CWD has been experimentally transmitted by artificial means to mice, ferrets,
mink, goats, squirrel monkeys, and calves.113
FDA prohibits the feeding of rendered deer and elk to ruminants. In addition,
FDA prohibits the use of known-CWD positive animals in any animal feeds, and
recommends against the use of rendered deer and elk material considered high-risk
in any animal feeds.114
Activities related to CWD control are also conducted by USDA’s Agricultural
Research Service (ARS) and Cooperative State Research, Education and Extension
Service (CSREES)115 and several agencies in the Department of the Interior (DOI).
In recognition that CWD is being found in more areas, and that resource limitations
and program inconsistencies exist among the states, a national CWD Task Force was
formed in 2002 “to ensure that federal and state agencies cooperate in the
development and implementation of an effective national CWD program.”116 This
task force, initiated between USDA, DOI, and state wildlife and agriculture agencies,
produced the strategic plan (see footnote) which, among other things, states that the
primary federal role will be to provide coordination and assistance with research,
surveillance, disease management, diagnostic testing, technology, communications,
information, education, and funding for state CWD programs. The task force has
working groups with action plans organized around most of these topics, though
there have been concerns about delays in its implementation.
APHIS, whose regulations govern cooperative programs to control animal
diseases, had published a final rule in the July 21, 2006, Federal Register to establish
a captive herd certification program for CWD, and rules on interstate movement of
cervids.117 The rule was to take effect on October 19, 2006, but APHIS published (in
the September 8, 2006 Federal Register) an indefinite delay in this effective date.
The agency had received petitions from the Association of Fish and Wildlife
Agencies, the U. S. Animal Health Association, and the National Assembly of State
Animal Health Officials raising concerns about the rule, such as whether federal
CRS-51
118 Chronic Wasting Disease Alliance, “CWD Update,” September 13, 2006, at
http://www.cwd-info.org/index.php/fuseaction/news.detail/ID/31747682f9f71e0d5d12e12a8f322288
119 Progress Report on the Plan for Assisting States, Federal Agencies and Tribes in
Managing Chronic Wasting Disease in Wild and Captive Herds (October 2002-September
2003), May 2004.
120 Cornell Feline Health Center, “Mad Cow Disease and Cats,” accessed Sept. 20, 2006, at
[http://www.vet.cornell.edu/fhc/resources/madcow.htm].
interstate movement regulations should preempt state requirements for importation,
and the scientific basis underlying federal interstate movement requirements.118
According to the 2004 CWD task force’s progress report, total federal spending
for CWD approximates $25 million annually, of which nearly $20 million are USDA
funds. The report estimates state spending to be roughly $15-20 million per year.119
Several bills addressing research, surveillance, or control of CWD were introduced
in the 108th Congress, including H.R. 2057; H.R. 2430; H.R. 2431; H.R. 2636; H.R.
3714, and S. 1036; S. 1366 and S. 2007. A hearing on CWD was held by the Senate
Environment and Public Works Committee, Fisheries, Wildlife and Water
Subcommittee on April 5, 2004. Much of the CWD interest in the 109th Congress
has been centered around the funding levels in USDA’s annual appropriation
measures.
SNIP...
http://ncseonline.org/NLE/CRSreports/06Oct/RL32199.pdf
IMPORT AND EXPORT
http://www.usaha.org/committees/reports/2006/report-ie-2006.pdf
Brain disease stalks Alberta deer Bigger populations make wasting more likely to spread in threat to Alberta's $100M-a-year hunting industry Hanneke Brooymans, The Edmonton Journal Published: Monday, January 15, 2007 WAINWRIGHT -- As the helicopter turns a corkscrew in the sky to get a better look, panicked deer bound away, bashing through brush and ice-glazed snow drifts.
Wildlife technician Traci Morgan peers out her window behind the pilot as the chopper swirls downward on this survey flight. She presses the button of her mike: "Whitetails -- two does, four fawns," she says, marking them down on her clipboard.
"I can't believe the number of critters out there," says Lyle Fullerton, another staffer with Alberta Sustainable Resource Development.
University of Alberta student Chris Garrett, left, and Gary Hornbeck, a consultant with Wildlife and Company, help in the battle against chronic wasting disease by attaching collars to wild Alberta deer. By day's end, Morgan tallies 939 deer and moose in a 55-square-kilometre area. That's surprisingly high, given that fish and wildlife officers had conducted a cull here in March 2005 to battle the spread of chronic wasting disease.
"We've probably never had as many deer in the province as we do now," Fullerton says later.
The denser the population, the more likely that chronic wasting disease will spread.
The brain-wasting disease has been found in wild deer in 11 American states and two provinces, first in Saskatchewan and then in Alberta in 2005. No jurisdiction has been able to halt its spread.
It's a prion disease like bovine spongiform encephalopathy -- mad cow disease -- and it is always fatal. It isn't known to infect humans, but the World Health Organization nevertheless has cautioned people not to eat the meat of affected animals.
If the disease spreads further, it could scare off recreational hunters from Alberta and out of province -- and each group spends about $50 million a year, said Vic Adamowicz, a University of Alberta professor of rural economy.
Scientists don't know yet what would happen to wild deer populations if the disease went unchecked. In an area of Colorado where up to 30 per cent of the deer are infected, scientists say it's plausible the population will die out within 35 years.
Alberta's already-threatened woodland caribou are another cause for concern. Could they catch the disease? No one knows for sure.
With test results pouring in from the current hunting season in Alberta, the number of wild deer testing positive for chronic wasting disease has crept up to 17 since the first case was found in late 2005. The most recent positive was confirmed Jan. 2.
Even more worrisome is where the cases are turning up.
Until now, the infected mule deer and white-tailed deer have been found along the Alberta/Saskatchewan border. One outbreak appeared around Empress and the South Saskatchewan River valley. The second "spark," as some biologists call it, is further north, around Chauvin.
Since the first Alberta case staggered into a farmyard in September 2005, a small rash of cases has spread further into the province.
One of the most recent was found near Edgerton, about 30 kilometres from the Saskatchewan border.
"That's the furthest into Alberta that a positive deer has been found," said Fullerton, an information officer with Sustainable Resource Development and a past executive director of the Alberta Fish and Game Association.
http://www.canada.com/edmontonjournal/news/story.html?id=665c8db6-093c-45ef-a4c7-a692d25098de
Hanneke Brooymans, The Edmonton Journal
Published: Monday, January 15, 2007 "That deer had the potential to infect other deer when it was living -- how long did it have the disease?"
The Edgerton case was perilously close to CFB Wainwright, which has an ecological reserve attached to its southern edge. The deer density there is extremely high, and the logistics of a cull would be nightmarish.
Further south, at the other outbreak area, the rash of positive cases follows upstream along the South Saskatchewan River valley. If the last case had travelled another 20 kilometres down the valley, it would have entered CFB Suffield. Another nightmare.
Fullerton loves to hunt and stocks his freezer with venison for the winter, but the growing deer population is no boon because of the risk of further spreading the wasting disease. "So what we're trying to do is get on it now, before it spreads out."
For now, that means deer surveys by helicopter at a cost of up to $35,000 per wildlife management unit, putting collars on deer and conducting genetic studies.
By analyzing the genetic differences among deer samples, David Coltman and his associates at the University of Alberta will try to predict where the disease will spread.
If two deer populations have similar genetic profiles, it means they're mixing and the disease is likely to spread between them, although no one knows exactly how it passes from one deer to the next.
The researchers hope to create a map of Alberta shaded in categories of high, medium and low risk.
The genetic studies will also show if pockets of deer populations are resistant to the disease. Although these animals do eventually get infected, they usually take longer to succumb. This information would be worked into the predictive model.
Chris Garrett adds to the projection work by attaching tracking collars to deer. The U of A master's student and his colleagues run traps to catch the deer.
Each 70-kilogram animal is wrestled to the ground and blindfolded for calming before the team hobbles its legs and takes a tissue sample by punching a hole in the ear for a tag. After looking at its teeth to determine the animal's age, they release it.
All of these studies will help Alberta Sustainable Resource Development develop a plan to try to stop the disease from spreading.
But the ministry isn't idle while waiting for the results. It conducts culls in a 10-kilometre radius around an area after a deer tests positive, and it thins herds in select locations.
From September 2005 to March 2006, wildlife officers shot 1,850 deer.
The department has also eased hunting restrictions along the Saskatchewan border to encourage recreational hunters to kill more deer. Hunters are required to turn in deer heads for testing in five wildlife management units in this area.
So far, 2,831 heads have been turned in this season. Four have tested positive.
Ninety-five heads are left to test, and more may arrive -- hunting season closes today for landowners in the areas where chronic wasting disease has been found.
Fullerton said the deer survey results, the positive tests and any data the U of A scientists can provide will be gathered to formulate the next plan of attack in the battle to contain chronic wasting disease.
http://www.canada.com/edmontonjournal/news/story.html?id=665c8db6-093c-45ef-a4c7-a692d25098de&p=2
Michael Samuel applauds the province's aggressive approach.
"If they can prevent the disease from getting into Alberta, that's the best thing," says Samuel, assistant unit leader with the U.S. Geological Survey co-operative wildlife research unit in Wisconsin. "Once it gets there, it's very difficult and costly to control."
But he doesn't know if the aggressive tactics will succeed. "I think nobody can promise that's going to work because no one's been successful yet."
hbrooymans@thejournal.canwest.com
http://www.canada.com/edmontonjournal/news/story.html?id=665c8db6-093c-45ef-a4c7-a692d25098de&p=3
WEB EXTRA: Game ranch near Swan River quarantined
Thu Jan 11 2007
A game ranch about 30 kilometres from Swan River is under quarantine by the Canadian Food Inspection Agency, after elk in a Saskatchewan ranch tested positive for chronic wasting disease.
"There's been no confirmed case (in Manitoba)," said Wayne Lees, chief veterinary officer for the province, on Wednesday.
Lees could not comment on the exact location of the Manitoban game ranch under quarantine.
The neurological disease is transmissable among deer and elk, and can cause small lesions in brains of infected animals. For infected creatures, the disease can lead to loss of bodily functions, abnormal behaviour and death.
"As part of any of these normal investigations, (inspectors) follow animals that travel in and out of the (elk) herd, and that's where the Manitoba connection comes in," said Lees.
"The investigation might take a number of weeks."
Lees said if any animals are found to have moved from the Saskatchewan ranch where animals tested positive for CWD, officials must conduct a 'trace-out' to ascertain the health of animals which may have come in contact with them.
Chronic wasting disease can be slow to develop, said Lees, and can take years for animals to show signs of illness.
"Animals that have been bought or sold from (the Saskatchewan) farm...(inspectors) determine the identity of those animals, and what's happened since," said Lees. "These investigations have to go back a number of years...and that's the reason these tracebacks can take some time. Several years of animal movements have to be checked out."
http://www.winnipegfreepress.com/subscriber/local/story/3838936p-4441931c.html
Revised: January 2007
Purpose:
To support the development of the game farm industry by delivering licensing and regulatory programs for domestic game farms.
Background:
All domestic farm operators are licensed and regulated according to The Domestic Game Farm Animal Regulations under The Animal Products Act. The act and regulations provide a framework for the Saskatchewan domestic game farm industry.
Impact:
Saskatchewan currently has 572 licensed domestic game farms with approximately 30,500 elk, 1,350 fallow deer, 7,400 white-tailed deer and 300 animals of other species being farmed. All domestic game farms are enrolled in Saskatchewan's Cervid Chronic Wasting Disease (CWD) Surveillance Program, with the additional option of enrolling in the Canadian CWD Voluntary Herd Certification Program.
Licensed Domestic Game Farm Operators
Contact:
To discuss Game Farm licensing: Rusty Hawryluk Room 202 - 3085 Albert Street Regina, SK S4S 0B1 Phone: 787-4682 Fax: 787-1315 Email: RHawryluk@agr.gov.sk.ca
To discuss tagging options or order unique identification tags for game farm species other than elk: Game Farm Statistics Clerk or Rusty Hawryluk Room 202 - 3085 Albert Street Regina, SK S4S 0B1 Phone: 787-7053 (Game Farm Statistics Clerk) Phone: 787-4606 (Game Farm Statistics Clerk) Phone: 787-4682 (Rusty Hawryluk) Fax: 787-1315
To obtain import or export permits: Crystal Lozinski Room 202 - 3085 Albert Street Regina, SK S4S 0B1 Phone: 787-6469 Fax: 787-1315 Email: CLozinski@agr.gov.sk.ca
To obtain export certificates: Rusty Hawryluk Room 202 - 3085 Albert Street Regina, SK S4S 0B1 Phone: 787-4682 Fax: 787-1315 Email: RHawryluk@agr.gov.sk.ca
To discuss tagging options or order unique identification tags for elk only: Saskatchewan Elk Breeders Association (SEBA) 381 Parkview Road Yorkton, SK S3N 2L4 Phone: 782-6500 (Maria Bartok) Fax: 782-6501 Email: seba@sasktel.net or maria@elkbreeders.sk.ca Regulatory related inquiries or concerns: Murray Phipps Provincial Game Farm Investigator 3830 Thatcher Avenue Saskatoon, SK S7K 2H6 Phone: 933-6138 Fax: 933-5715 Email: MPhipps@agr.gov.sk.ca For other information on game farming and related industries: Sherri Dobbs (development) Provincial Livestock Development Specialist Room 226 - 3085 Albert Street Regina, SK S4S 0B1 Phone: 787-4657 Fax: 787-9297 Email: SDobbs@agr.gov.sk.ca Kevin Augustine Provincial Game Farm Investigator 800 Central Avenue, Box 3003 Prince Albert, SK S6V 6G1 Phone: 953-2729 Fax: 953-2440 Email: KAugustine@agr.gov.sk.ca Renee Chartier Provincial Game Farm Inspector 3085 Albert Street Regina, SK S4S 0B1 Phone: 787-0514 Fax: 787-1315 Email: RChartier@agr.gov.sk.ca Murray Feist (nutrition & technology transfer) Feed Industry Development Specialist Agriculture Knowledge Centre 45 Thatcher Avenue Moose Jaw, SK S6J 1L8 Phone: 694-3492 Fax: 694-3938 Email: MFeist@agr.gov.sk.ca Dick Johnson Provincial Manager, Inspections & Investigations 3830 Thatcher Avenue Saskatoon, SK S7K 2H6 Phone: 933-6191 Fax: 933-5715 Email: DJohnson@agr.gov.sk.ca Saskatchewan's Cervid Chronic Wasting Disease Surveillance Program Applications, Certificates & Inventory Records: Phone: 787-4264 (Mary Jane Laville) Physical Inventory & Herd Appointments by District: (see map)
1 Renee Chartier 787-0514 2 Renee Chartier 787-0514 3 Dave Augustine 778-8312 4 Bob Solomon 786-5712 5 Murray Phipps 933-6138 6 Kevin Augustine 953-2729 7 Mike Lessard 446-7404 8 Danny L'Heureux 236-5456
Collection & Submission of Samples: Phone: 787-6069 (Dr. LeeAnn Forsythe)
Notification of Discovery of Deaths: Phone: 787-6469 (Animal Health Unit)
Game Farm Inspection Districts:
http://www.agr.gov.sk.ca/docs/programs_services/GF_Inspect_Program.asp
From: TSS (216-119-163-189.ipset45.wt.net) Subject: CWD aka MAD DEER/ELK TO HUMANS ??? Date: September 30, 2002 at 7:06 am PST
From: "Belay, Ermias" To: Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias" Sent: Monday, September 30, 2002 9:22 AM Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Dear Sir/Madam, In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.
That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: 404-639-3091). Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated.
Ermias Belay, M.D. Centers for Disease Control and Prevention
-----Original Message----- From: Sent: Sunday, September 29, 2002 10:15 AM To: rr26k@nih.gov; rrace@niaid.nih.gov; ebb8@CDC.GOV Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Sunday, November 10, 2002 6:26 PM ......snip........end..............TSS
also,
A. Aguzzi - Chronic Wasting Disease (CWD) also needs to be addressed. Most serious because of rapid horizontal spread and higher prevalence than BSE in UK, up to 15% in some populations. Also may be a risk to humans - evidence that it is not dangerous to humans is thin.
http://www.tseandfoodsafety.org/activities/bse_conference_basel_april_02/2summary_of_conference.htm
Chronic Wasting Disease and Potential Transmission to Humans Ermias D. Belay,* Ryan A. Maddox,* Elizabeth S. Williams,† Michael W. Miller,‡ Pierluigi Gambetti,§ and Lawrence B. Schonberger* *Centers for Disease Control and Prevention, Atlanta, Georgia, USA; †University of Wyoming, Laramie, Wyoming, USA; ‡Colorado Division of Wildlife, Fort Collins, Colorado, USA; and §Case Western Reserve University, Cleveland, Ohio, USA
Suggested citation for this article: Belay ED, Maddox RA, Williams ES, Miller MW, Gambetti P, Schonberger LB. Chronic wasting disease and potential transmission to humans. Emerg Infect Dis [serial on the Internet]. 2004 Jun [date cited]. Available from: http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm
--------------------------------------------------------------------------------
Chronic wasting disease (CWD) of deer and elk is endemic in a tri-corner area of Colorado, Wyoming, and Nebraska, and new foci of CWD have been detected in other parts of the United States. Although detection in some areas may be related to increased surveillance, introduction of CWD due to translocation or natural migration of animals may account for some new foci of infection. Increasing spread of CWD has raised concerns about the potential for increasing human exposure to the CWD agent. The foodborne transmission of bovine spongiform encephalopathy to humans indicates that the species barrier may not completely protect humans from animal prion diseases. Conversion of human prion protein by CWD-associated prions has been demonstrated in an in vitro cell-free experiment, but limited investigations have not identified strong evidence for CWD transmission to humans. More epidemiologic and laboratory studies are needed to monitor the possibility of such transmissions.
snip...full text ;
http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm
Volume 12, Number 10–October 2006 Research Human Prion Disease and Relative Risk Associated with Chronic Wasting Disease Samantha MaWhinney,* W. John Pape,† Jeri E. Forster,* C. Alan Anderson,‡§ Patrick Bosque,‡¶ and Michael W. Miller# *University of Colorado at Denver and Health Sciences Center, Denver, Colorado, USA; †Colorado Department of Public Health and Environment, Denver, Colorado, USA; ‡University of Colorado School of Medicine, Denver, Colorado, USA; §Denver Veteran's Affairs Medical Center, Denver, Colorado, USA; ¶Denver Health Medical Center, Denver, Colorado, USA; and #Colorado Division of Wildlife, Fort Collins, Colorado, USA
Suggested citation for this article
The transmission of the prion disease bovine spongiform encephalopathy (BSE) to humans raises concern about chronic wasting disease (CWD), a prion disease of deer and elk. In 7 Colorado counties with high CWD prevalence, 75% of state hunting licenses are issued locally, which suggests that residents consume most regionally harvested game. We used Colorado death certificate data from 1979 through 2001 to evaluate rates of death from the human prion disease Creutzfeldt-Jakob disease (CJD). The relative risk (RR) of CJD for CWD-endemic county residents was not significantly increased (RR 0.81, 95% confidence interval [CI] 0.40–1.63), and the rate of CJD did not increase over time (5-year RR 0.92, 95% CI 0.73–1.16). In Colorado, human prion disease resulting from CWD exposure is rare or nonexistent. However, given uncertainties about the incubation period, exposure, and clinical presentation, the possibility that the CWD agent might cause human disease cannot be eliminated.
snip... full text ;
http://0-www.cdc.gov.mill1.sjlibrary.org/ncidod/EID/vol12no10/06-0019.htm
3:00 Afternoon Refreshment Break, Poster and Exhibit Viewing in the Exhibit Hall
3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse
Models Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western Reserve University
Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain discovered recently in Italy, and similar or different atypical BSE cases were also reported in other countries. The infectivity and phenotypes of these atypical BSE strains in humans are unknown. In collaboration with Pierluigi Gambetti, as well as Maria Caramelli and her co-workers, we have inoculated transgenic mice expressing human prion protein with brain homogenates from BASE or BSE infected cattle. Our data shows that about half of the BASE-inoculated mice became infected with an average incubation time of about 19 months; in contrast, none of the BSE-inoculated mice appear to be infected after more than 2 years. ***These results indicate that BASE is transmissible to humans and suggest that BASE is more virulent than classical BSE in humans.
6:30 Close of Day One
http://www.healthtech.com/2007/tse/day1.asp
SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype of 'UNKNOWN' strain growing. ...
http://www.cjdsurveillance.com/resources-casereport.html
There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.
He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf
JOURNAL OF NEUROLOGY
MARCH 26, 2003
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States
Email Terry S. Singeltary:
flounder@wt.net
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to
comment on the CDC's attempts to monitor the occurrence of emerging
forms of CJD. Asante, Collinge et al [1] have reported that BSE
transmission to the 129-methionine genotype can lead to an alternate
phenotype that is indistinguishable from type 2 PrPSc, the commonest
sporadic CJD. However, CJD and all human TSEs are not reportable
nationally. CJD and all human TSEs must be made reportable in every
state and internationally. I hope that the CDC does not continue to
expect us to still believe that the 85%+ of all CJD cases which are
sporadic are all spontaneous, without route/source. We have many TSEs in
the USA in both animal and man. CWD in deer/elk is spreading rapidly and
CWD does transmit to mink, ferret, cattle, and squirrel monkey by
intracerebral inoculation. With the known incubation periods in other
TSEs, oral transmission studies of CWD may take much longer. Every
victim/family of CJD/TSEs should be asked about route and source of this
agent. To prolong this will only spread the agent and needlessly expose
others. In light of the findings of Asante and Collinge et al, there
should be drastic measures to safeguard the medical and surgical arena
from sporadic CJDs and all human TSEs. I only ponder how many sporadic
CJDs in the USA are type 2 PrPSc?
http://www.neurology.org/cgi/eletters/60/2/176#535
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Singeltary, Sr et al. JAMA.2001; 285: 733-734.
http://jama.ama-assn.org/
TSS
=====================================================
MY greatest fear is of iCJD via CWD tainted humans in the surgical and medical arena x 1, 2, 3, 4, 5, passages etc. from someone that has been exposed to the cwd agent, but not gone clinical, and may never go clinical. WE must realize that the USA has the most documented TSE in different species than any other country in the world. all of which has been rendered and fed back to animals for human and animal consumption. the incubation period is what is fooling everyone. ...tss
Prion protein in cardiac
elaphus nelsoni)
Odocoileus virginianus)
wasting disease
snip...
infectivity in skeletal muscle of CWD-infected mule deer (Angers et al.,
2006) has raised the level of concern on the issue of potential
human health risks that might be encountered by consuming
prion-containing meat.
Prions in Skeletal Muscles of Deer with Chronic Wasting Disease
Rachel C. Angers,1* Shawn R. Browning,1*† Tanya S. Seward,2 Christina J. Sigurdson,4‡ Michael W. Miller,5 Edward A. Hoover,4 Glenn C. Telling1,2,3§
1Department of Microbiology, Immunology and Molecular Genetics, 2Sanders Brown Center on Aging, 3Department of Neurology, University of Kentucky, Lexington, KY 40536, USA. 4Department of Microbiology, Immunology and Pathology, Colorado State University, Fort Collins, CO 80523, USA. 5Colorado Division of Wildlife, Wildlife Research Center, Fort Collins, CO 80526, USA.
*These authors contributed equally to this work.
†Present address: Department of Infectology, Scripps Research Institute, 5353 Parkside Drive, RF-2, Jupiter, Florida, 33458, USA.
‡Present address: Institute of Neuropathology, University of Zurich, Schmelzbergstrasse 12, 8091 Zurich, Switzerland.
§To whom correspondence should be addressed: E-mail: gtell2@uky.edu
Prions are transmissible proteinaceous agents of mammals that cause fatal neurodegenerative diseases of the central nervous system (CNS). The presence of infectivity in skeletal muscle of experimentally infected mice raised the possibility that dietary exposure to prions might occur through meat consumption (1). Chronic wasting disease (CWD), an enigmatic and contagious prion disease of North American cervids, is of particular concern. The emergence of CWD in an increasingly wide geographic area and the interspecies transmission of bovine spongiform encephalopathy (BSE) to humans as variant Creutzfeldt Jakob disease (vCJD) have raised concerns about zoonotic transmission of CWD.
To test whether skeletal muscle of diseased cervids contained prion infectivity, Tg(CerPrP)1536 mice (2) expressing cervid prion protein (CerPrP), were inoculated intracerebrally with extracts prepared from the semitendinosus/semimembranosus muscle group of CWD-affected mule deer or from CWD-negative deer. The availability of CNS materials also afforded direct comparisons of prion infectivity in skeletal muscle and brain. All skeletal muscle extracts from CWD-affected deer induced progressive neurological dysfunction in Tg(CerPrP)1536 mice with mean incubation times ranging between 360 and ~490 d, whereas the incubation times of prions from the CNS ranged from ~230 to 280 d (Table 1). For each inoculation group, the diagnosis of prion disease was confirmed by the presence of PrPSc in the brains of multiple infected Tg(CerPrP)1536 mice (see supporting online material for examples). In contrast, skeletal muscle and brain material from CWD-negative deer failed to induce disease in Tg(CerPrP)1536 mice (Table 1) and PrPSc was not detected in the brains of sacrificed asymptomatic mice as late as 523 d after inoculation (supporting online material).
Our results show that skeletal muscle as well as CNS tissue of deer with CWD contains infectious prions. Similar analyses of skeletal muscle BSE-affected cattle did not reveal high levels of prion infectivity (3). It will be important to assess the cellular location of PrPSc in muscle. Notably, while PrPSc has been detected in muscles of scrapie-affected sheep (4), previous studies failed to detect PrPSc by immunohistochemical analysis of skeletal muscle from deer with natural or experimental CWD (5, 6). Since the time of disease onset is inversely proportional to prion dose (7), the longer incubation times of prions from skeletal muscle extracts compared to matched brain samples indicated that prion titers were lower in muscle than in CNS where infectivity titers are known to reach high levels. Although possible effects of CWD strains or strain mixtures on these incubation times cannot be excluded, the variable 360 to ~490 d incubation times suggested a range of prion titers in skeletal muscles of CWD-affected deer. Muscle prion titers at the high end of the range produced the fastest incubation times that were ~30% longer than the incubation times of prions from the CNS of the same animal. Since all mice in each inoculation group developed disease, prion titers in muscle samples producing the longest incubation times were higher than the end point of the bioassay, defined as the infectious dose at which half the inoculated mice develop disease. Studies are in progress to accurately assess prion titers.
While the risk of exposure to CWD infectivity following consumption of prions in muscle is mitigated by relatively inefficient prion transmission via the oral route (8), these
/ www.sciencexpress.org / 26 January 2006 / Page 1 / 10.1126/science.1122864
results show that semitendinosus/semimembranosus muscle, which is likely to be consumed by humans, is a significant source of prion infectivity. Humans consuming or handling meat from CWD-infected deer are therefore at risk to prion exposure.
CWD
Human health implications
14. Epidemiological data on possible CWD infection of humans are
very limited. The possibility that clinical symptoms of CWD in
humans differ from those of Creutzfeldt-Jakob Disease (CJD)
cannot be excluded. There is no significant difference between the
prevalence of CJD in CWD endemic areas and other areas of the
world. However, because CJD surveillance in the USA is relatively
recent, not all CJD cases may have been identified. Additionally,
detection of a small increase in prevalence of such a rare disease
is very difficult. Investigation of six cases of prion disease in young
people ((> 54 years of age) diagnosed
with sporadic CJD, no link with consumption of venison from CWD
endemic areas was found. No causal link was found in an
investigation of three men with neurological illnesses who were
known to partake in “wild game feasts”. Only one of these subjects
was found to have a prion disease and this was also
indistinguishable from sporadic CJD.
snip...
http://www.seac.gov.uk/statements/statement180105.pdf
10) HUMAN HEALTH CONCERNS
217. To date there are no known cases of human prion disease attributable to CWD
transmitted to humans (Belay et al., 2004). While limited epidemiological investigations to
date have not shown any links between CWD and humans with spongiform encephalopathies
CWD Review / Dr Debra Bourne / October 2004 / For SEAC / Page 45 of 66
this data must be considered along with a caveat: “because CWD is a relatively new TSE, it is
unlikely that enough people have consumed enough CWD-affected cervids to result in a
clinically or pathologically recognizable disease attributable to CWD, especially considering
the very long incubation periods characteristic of TSE diseases.” (Race et al., 2002)
Epidemiological investigations
218. Epidemiological investigations have failed to show any links between cases of prion
disease in unusually young people or in hunters in the USA and CWD (CDC, 2003). Two
major epidemiological investigations have been carried out, one on cases of CJD in unusually
young individuals in the USA, the second on a group of men from Wisconsin who developed
neurological diseases.
219. The first study (Belay et al., 2001) focused on three individuals, two 28 years of age
and the third 30 years old, diagnosed with CJD in the USA between 1st January 1997 and 31st
May 2000, and without any established risk factors for CJD (family history, receipt of human
growth hormone, receipt of grafts of dura mater or cornea, or previous neurological surgery)
and concluded that there was no strong evidence for a causal link with CWD. None of the
individuals had travelled to Europe (therefore a link with BSE was unlikely). Two of the
individuals were hunters who regularly consumed game meat while the third (case 1) had, as a
young child, regularly consumed venison from animals hunted by family members and on two
occasions from a family friend. Two of the individuals (cases 1 and 2) had undergone tonsillar
surgery as children; the third had never received any surgical treatment. One individual (case
1) had eaten venison mainly hunted in Maine, occasionally hunted in New Jersey, and, on two
occasions at about six years old, elk meat which had probably been harvested in Wyoming.
The second person (case 2) had hunted cervids mainly in Utah, but had harvested an elk in
southwestern Wyoming on one occasion (less than three years before onset of clinical signs)
and had hunted in British Columbia on one occasion nine years before onset of illness. The
third person (case 3) had hunted close to home and never in Colorado or Wyoming although
the plant where he took his carcasses for processing did also process some elk from Colorado
each year. The clinical signs, duration of illness and histopathological findings for the three
individuals showed no obvious similarities to one another. One individual was
methionine/methionine homozygous at codon 129 of the PRNP (case 1), one was
homozygous for valine at this gene (case 2) and the third (case 3) was heterozygous
methionine/valine. Immunohistochemistry revealed strong staining with a “synaptic” pattern
in the first individual and weak staining with a “synaptic” pattern in the second case; in case
3, based on a brain biopsy sample obtained at an early point in the illness, staining was
questionable and possibly showed a synaptic pattern. Cases 2 and 3 showed a “Type 1”
immunoblot pattern, this test had not been carried out for case 1. It was noted that none of
these three individuals had a definite history of consumption of venison from the geographical
areas in which CWD was known to be endemic in Colorado and Wyoming, and no CWD had
been identified in 299 deer and sampled from the area in which most of the venison consumed
by patient 1 had originated, nor in 404 deer and 196 elk sampled from the area in which most
of the venison consumed by patient 2 had originated, nor in 138 deer samples from the area in
which most of the venison consumed by patient 1 had originated. Additionally, there was no
homogeneity in phenotypic expression of the disease and all three possible options for coding
at codon 129 of the PRNP gene were represented. Since a survey had indicated that
approximately 40% of blood donors in the USA consumed venison from wild cervids, it was
considered most likely that coincidence explained why three of the four young (30 years old
or younger) individuals with sporadic CJD reported in the USA after March 1996 had
consumed such meat (Belay et al., 2001).
CWD Review / Dr Debra Bourne / October 2004 / For SEAC / Page 46 of 66
220. The second major epidemiological investigation centred around three men from
Wisconsin and Minnesota who had died from degenerative neurological illnesses and who
had participated in “wild game feasts” in northern Wisconsin. Full investigation including
examination of fixed brain tissue confirmed CJD in only one of the three individuals. Wild
game eaten during the feasts was harvested mainly in Wisconsin but also in areas of
Colorado, Wyoming and Montana; CWD was not known to be endemic in the areas where the
game was hunted at the time that the game was harvested. Further investigations of other
possible attendees of the feasts revealed 34 participants, all male, of whom a total of seven
were deceased, including the three individuals in the initial investigation. Causes of death in
the other four deceased individuals were not attributed to nor associated with any
degenerative neurological disorder and no signs or symptoms associated with a degenerative
neurological disorder were noted for any of the remaining living participants of the feasts. It
was noted that only one case of CJD had occurred among known participants at the feasts,
that this case was consistent with the commonest form of sporadic CJD, that this individual
had only participated in one feast and that it was unlikely that he had consumed CWDinfected
venison at the feasts “because venison and other game from outside Wisconsin that
was served at these feasts did not originate from known CWD-endemic areas.” Limitations of
the investigations were noted to include reliance on recall of events from up to 25 years
previously and the fact that not all participants in the feasts could be contacted and
interviewed. However, those who were interviewed agreed in their recall of events (CDC,
2003).
221. It is important to recognise that the limited epidemiological investigations that have
been carried out are not able to rule out the possibility that CWD might play a role in causing
illness in humans (CDC, 2003).
222. Three further cases of prion disease in young humans in the USA have been
investigated for possible links to CWD (Belay et al., 2004). The first case was a 25-year-old
man who died in 2001 after about 22 months of illness. Gerstmann-Sträussler-Scheinker
syndrome (GSS) was diagnosed by analysis of the prion gene, with a P102L mutation
together with valine at codon 129 in the mutant allele. It was noted that the disease had
occurred at an unusually young age, even for GSS, and the possibility that exposure to CWDinfected
venison contributed to early onset of the disease could not be ruled out; the patient’s
grandfather had regularly hunted in southeastern Wyoming, around the known CWD-endemic
area, and had given venison to the patient’s family. Two other cases of prion disease occurred
in individuals of 26 and 28 years of age, from adjacent counties, and with onset of illness only
months apart, therefore an environmental source of infection was investigated. However,
these two individuals were finally diagnosed with different prion diseases: sporadic CJD in
one case and GSS in the other, indicating that a common cause was unlikely. In the first case
CJD was confirmed from autopsy samples (by histopathology, immunohistochemistry and
immunoblotting); the individual had no history of hunting nor of regular consumption of
venison, and although he may have eaten venison originating from the Upper Peninsula of
Michigan while at college CWD has never been detected in deer from Michigan.
Phenotypically this individual fit the “MM2 sporadic CJD” phenotype described by Parchi et
al. (1999). In the other case post mortem immunohistochemistry revealed prion deposition
which was consistent with GSS and a GSS P102L mutation was detected in a blood sample
from one parent (appropriate samples were not available from the affected patient); this
individual may possibly have eaten venison from Michigan on one occasion at about two
years of age (Belay et al., 2004).
223. A further three cases of CJD in individuals of 54 to 66 years old who were deer and
elk hunters (two individuals) or ate wild-harvested venison (one individual) have been
CWD Review / Dr Debra Bourne / October 2004 / For SEAC / Page 47 of 66
investigated. There was no evidence that any of these individuals had hunted in known CWD
endemic areas; information available indicated hunting or eating venison from Washington
State and Pennsylvania. Two individuals were V/V at codon 129 the third was M/M; they
were considered to fit known subtypes of sporadic CJD (MM1, VV1 and VV2 subtypes as
described by Parchi et al. (1999)). Further investigations were also made on the only two
nonfamilial cases of CJD in individuals with a history of eating venison from the known
CWD-endemic areas. One was reported to have eaten venison from two deer harvested in an
area with endemic CWD, but both deer had been tested and not found to be CWD-positive;
the patient’s illness was consistent with the CJD subtype MM1. The other individual grew up
in a CWD-endemic area and ate locally-harvested venison; her disease fit the MM1 CJD
phenotype and no atypical neurological features were noted (Belay et al., 2004).
224. Additional epidemiological notes are that the incidence and age distribution of CJD in
Colorado and Wyoming, where CWD is thought to have been endemic for decades, are
similar to those found in other areas of the USA. In Wyoming, seven cases of CJD have been
reported between 1979 and 2000 with an average annual age-adjusted CJD death rate of 0.8
per million and no cases reported in humans less than 55 years old. In Colorado in the same
period 67 cases of CJD have been reported, with an average annual age-adjusted CJD death
rate of 1.2 per million (Belay et al., 2004).
225. In summary, there is no evidence of an increase in incidence of CJD in Colorado and
Wyoming, nor have epidemiological investigations carried out so far found any evidence of a
link between CWD and cases of CJD in persons in the USA (Belay et al., 2001; CDC, 2003;
Belay et al., 2004).
Laboratory studies
226. There is evidence from an in vitro cell-free system that there may be a considerable
“species barrier” reducing the probability that CWD will affect humans. It was shown that
PrPres associated with chronic wasting disease (PrPCWD) from elk, mule deer or white-tailed
deer was able to readily induce substantial conversion of recombinant cervid PrPsen
molecules form any of these three species to the protease-resistant state. In the same system,
CWD-associated PrPres was shown to convert human PrPsen but at a much lower efficiency:
more than 14-fold lower efficiency than inter-cervid conversion reactions and more than
fivefold
lower than conversion of human PrPsen by PrPres from the brains of humans with CJD
(Raymond et al., 2000). While encouraging, interpretation of this study is complicated by the
fact that conversion of human PrPC by PrPBSE and PrPSc from sheep were of similar efficacy,
both being more than 10-fold less efficient compared with corresponding homologous
conversions) and one of these appears to be orally transmissible to humans (BSE) while the
other (scrapie) appears not to be (Raymond et al., 2000). In previous experiments PrPBSE had
showed 10-fold greater conversion efficacy for bovine PrPsen than for human codon 129-M
(methionine) PrPsen and 30-fold greater conversion efficacy than for human codon 129-V
(valine) PrPsen, while ovine PrPSc showed five-fold greater conversion efficacy for ovine
PrPsen than for human 129-M PrPsen and eight-fold greater conversion than for human 129-
V PrPsen (Raymond et al., 1997).
227. Results of recent work in transgenic mice expressing human PrP (see paragraph 71), in
which transmission of CWD from elk by intracerebral inoculation failed, was considered to
“strongly suggest” a species barrier to transmission of elk CWD to humans (Kong et al.,
2004).
CWD Review / Dr Debra Bourne / October 2004 / For SEAC / Page 48 of 66
Potential risk from consuming cervid products
Velvet antler
228. Limited studies to date indicate risk from this product may be very low. No CWDspecific
PrP accumulation was detected in a sample of velvet from an elk stag which
developed clinical CWD about three months later; there were severe brain lesions and
extensive CWD-specific PrP staining in both the brain and peripheral lymphoid tissue of the
stag (Kahn et al., 2004).
Consumption of venison and other parts of the animal
229. PrPCWD has not been detected in muscle tissue from infected cervids (Spraker et al.,
2002c). However, it has been recommended by the World Health Organisation that no parts or
products of any animal know to be CWD-positive should be consumed (WHO, 2000). Public
health authorities in the USA and Canada have indicated agreement with this (Canadian Food
Inspection Agency, 2003; Chronic Wasting Disease Alliance, 2004). It has been suggested
that if a harvested cervid is being tested for CWD, the test results should be awaited before
the meat is eaten (Wisconsin Department of Agriculture, Trade and Consumer Protection,
2002). Authorities in North America have widely advised that (a) tissues likely to contain the
greatest amount of CWD agent in infected cervids, including the brain, spinal cord, lymph
nodes, spleen, tonsils and eyes, should not be consumed from any harvested deer; (b) meat
should be boned out and fat and connective tissue removed (which would also remove lymph
nodes); and (c) hunters should avoid eating meat from deer or elk which look sick or which
test positive for CWD (Buege, 2002; Chronic Wasting Disease Alliance, 2004; Williams et
al., 2002; Wisconsin Department of Agriculture, Trade and Consumer Protection, 2002;
Belay et al., 2004).
Potential risk from handling and processing cervids
230. In order to minimise any potential risk from exposure to the agent of CWD, hunters,
meat processors and taxidermists handling cervid carcasses are advised to wear latex or
rubber gloves when handling or dressing cervids from CWD-endemic areas, to minimise
handling of brain and spinal cord, and to thoroughly wash knives and other implements after
use on deer or elk carcasses (Belay, 2004; Williams et al., 2002). It has been suggested that
the risk of “build-up” of infectious CWD agent in a venison processing plant would be
unlikely (Buege, 2002).
Potential risk from disposal of carcasses and subsequent contamination of
ground/water/air
231. In 2002, a risk analysis was produced on disposal of deer from Wisconsin in
municipal solid landfills. It was noted that it is not known how much infected material a
human (or animal) must consume or be exposed to in order to be infected with CWD. The
report took into account the probable species barrier for transmission to humans (Raymond et
al., 2000). It was noted that the CWD agent is hydrophobic and likely to adhere to organic
materials within a landfill, taking several months to move through the landfill, and that any
infectivity exiting the landfill would be captured in the landfill effluent. If effluent was
transferred to a wastewater plant (rather than recirculated in the landfill) the agent would be
expected to partition with the sludge fraction, which would be diluted greatly with other
solids and mixed with nine inches (22.5 cm) of topsoil, providing “an extremely large dilution
factor.” It was concluded that there was no significant risk to human health from disposing of
deer infected with CWD in properly constructed landfill sites (Olander, 2002).
snip...full text 66 pages ;
http://www.seac.gov.uk/papers/CWD-bournereview.pdf
SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype of 'UNKNOWN' strain growing. ...
http://www.cjdsurveillance.com/resources-casereport.html
HUMAN TSE USA 2005
Animal Prion Diseases Relevant to Humans (unknown types?) Thu Oct 27, 2005 12:05 71.248.128.109
About Human Prion Diseases / Animal Prion Diseases Relevant to Humans
Bovine Spongiform Encephalopathy (BSE) is a prion disease of cattle. Since 1986, when BSE was recognized, over 180,000 cattle in the UK have developed the disease, and approximately one to three million are likely to have been infected with the BSE agent, most of which were slaughtered for human consumption before developing signs of the disease. The origin of the first case of BSE is unknown, but the epidemic was caused by the recycling of processed waste parts of cattle, some of which were infected with the BSE agent and given to other cattle in feed. Control measures have resulted in the consistent decline of the epidemic in the UK since 1992. Infected cattle and feed exported from the UK have resulted in smaller epidemics in other European countries, where control measures were applied later.
Compelling evidence indicates that BSE can be transmitted to humans through the consumption of prion contaminated meat. BSE-infected individuals eventually develop vCJD with an incubation time believed to be on average 10 years. As of November 2004, three cases of BSE have been reported in North America. One had been imported to Canada from the UK, one was grown in Canada, and one discovered in the USA but of Canadian origin. There has been only one case of vCJD reported in the USA, but the patient most likely acquired the disease in the United Kingdom. If current control measures intended to protect public and animal health are well enforced, the cattle epidemic should be largely under control and any remaining risk to humans through beef consumption should be very small. (For more details see Smith et al. British Medical Bulletin, 66: 185. 2003.)
Chronic Wasting Disease (CWD) is a prion disease of elk and deer, both free range and in captivity. CWD is endemic in areas of Colorado, Wyoming, and Nebraska, but new foci of this disease have been detected in Nebraska, South Dakota, New Mexico, Wisconsin, Mississippi Kansas, Oklahoma, Minnesota, Montana, and Canada. Since there are an estimated 22 million elk and deer in the USA and a large number of hunters who consume elk and deer meat, there is the possibility that CWD can be transmitted from elk and deer to humans. As of November 2004, the NPDPSC has examined 26 hunters with a suspected prion disease. However, all of them appeared to have either typical sporadic or familial forms of the disease. The NPDPSC coordinates with the Centers for Disease Control and state health departments to monitor cases from CWD-endemic areas. Furthermore, it is doing experimental research on CWD transmissibility using animal models. (For details see Sigurdson et al. British Medical Bulletin. 66: 199. 2003 and Belay et al. Emerging Infectious Diseases. 10(6): 977. 2004.)
http://www.cjdsurveillance.com/abouthpd-animal.html
Interspecies Transmission of Chronic Wasting Disease Prions to
Squirrel Monkeys (Saimiri sciureus)
Richard F. Marsh,1? Anthony E. Kincaid,2 Richard A. Bessen,3 and Jason C. Bartz4*
Department of Animal Health and Biomedical Sciences, University of Wisconsin, Madison 537061; Department of
Physical Therapy2 and Department of Medical Microbiology and Immunology,4 Creighton University, Omaha,
Nebraska 68178; and Department of Veterinary Molecular Biology, Montana
State University, Bozeman, Montana 597183
Received 3 May 2005/Accepted 10 August 2005
Chronic wasting disease (CWD) is an emerging prion disease of deer and elk. The risk of CWD transmission to humans following exposure to CWD-infected tissues is unknown. To assess the susceptibility of nonhuman primates to CWD, two squirrel monkeys were inoculated with brain tissue from a CWD-infected mule deer. The CWD-inoculated squirrel monkeys developed a progressive neurodegenerative disease and were euthanized at 31 and 34 months postinfection. Brain tissue from the CWD-infected squirrel monkeys contained the abnormal
isoform of the prion protein, PrP-res, and displayed spongiform degeneration. This is the first reported transmission of CWD to primates.
snip...
JOURNAL OF VIROLOGY, Nov. 2005, p. 13794-13796 Vol. 79, No. 21
0022-538X/05/$08.00!0 doi:10.1128/JVI.79.21.13794-13796.2005
Copyright © 2005, American Society for Microbiology. All Rights Reserved.
=============================================
The EMBO Journal, Vol. 19, No. 17 pp. 4425-4430, 2000 © European Molecular Biology Organization
Evidence of a molecular barrier limiting susceptibility of humans, cattle and sheep to chronic wasting disease
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
1NIAID/NIH Rocky Mountain Laboratories, Hamilton, MT 59840, 3USDA/ARS/ADRU, Pullman, WA 99164-7030, 4USDA/ARS/ABADRL, Laramie, WY 82071, 5Colorado Division of Wildlife, Wildlife Research Center, Fort Collins, CO 80526-2097, 6Department of Veterinary Sciences, University of Wyoming, Laramie, WY 82070, USA and 2ID-Lelystad, Institute for Animal Science and Health, Lelystad, The Netherlands 7Corresponding author e-mail: bcaughey@nih.gov Received June 7, 2000; revised July 3, 2000; accepted July 5, 2000.
Abstract
Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy (TSE) of deer and elk, and little is known about its transmissibility to other species. An important factor controlling interspecies TSE susceptibility is prion protein (PrP) homology between the source and recipient species/genotypes. Furthermore, the efficiency with which the protease-resistant PrP (PrP-res) of one species induces the in vitro conversion of the normal PrP (PrP-sen) of another species to the protease-resistant state correlates with the cross-species transmissibility of TSE agents. Here we show that the CWD-associated PrP-res (PrPCWD) of cervids readily induces the conversion of recombinant cervid PrP-sen molecules to the protease-resistant state in accordance with the known transmissibility of CWD between cervids. In contrast, PrPCWD-induced conversions of human and bovine PrP-sen were much less efficient, and conversion of ovine PrP-sen was intermediate. These results demonstrate a barrier at the molecular level that should limit the susceptibility of these non-cervid species to CWD.
snip...
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 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.
snip...
http://www.emboj.org/current.shtm
Subject: Infectious Prions in the Saliva and Blood of Deer with Chronic Wasting Disease
Date: October 5, 2006 at 1:45 pm PST
Infectious Prions in the Saliva
and Blood of Deer with Chronic
Wasting Disease
Candace K. Mathiason,1 Jenny G. Powers,3 Sallie J. Dahmes,4 David A. Osborn,5 Karl V. Miller,5
Robert J. Warren,5 Gary L. Mason,1 Sheila A. Hays,1 Jeanette Hayes-Klug,1 Davis M. Seelig,1
Margaret A. Wild,3 Lisa L. Wolfe,6 Terry R. Spraker,1,2 Michael W. Miller,6 Christina J. Sigurdson,1
Glenn C. Telling,7 Edward A. Hoover1*
A critical concern in the transmission of prion diseases, including chronic wasting disease (CWD) of cervids, is the potential presence of prions in body fluids. To address this issue directly, we exposed cohorts of CWD-nai¨ve deer to saliva, blood, or urine and feces from CWD-positive deer. We found infectious prions capable of transmitting CWD in saliva (by the oral route) and in blood (by transfusion). The results help to explain the facile transmission of CWD among cervids and prompt caution concerning contact with body fluids in prion infections.
SNIP...
Deer cohorts 1 (blood), 2 (saliva), and 3
(urine and feces) were electively euthanized at
18 months pi to permit whole-body examination
for PrPCWD. The greatest scrutiny was directed
toward those tissues previously established
to have highest frequency of PrPCWD deposition
in infected deer and generally regarded
as the most sensitive indicators of infection—
medulla oblongata and other brainstem regions,
tonsil, and retropharyngeal lymph node. We
found unequivocal evidence of PrPCWD in brain
and lymphoid tissue of all six tonsil biopsy–
positive deer in cohorts 1 (blood) and 2 (saliva),
whereas all deer in cohorts 3 and 5 were negative
for PrPCWD in all tissues (Table 2 and
Figs. 1 and 2).
The transmission of CWD by a single blood
transfusion from two symptomatic and one
asymptomatic CWDþ donor is important in at
least three contexts: (i) It reinforces that no tissue
from CWD-infected cervids can be considered
free of prion infectivity; (ii) it poses the
possibility of hematogenous spread of CWD,
such as through insects; and (iii) it provides a
basis for seeking in vitro assays sufficiently
sensitive to demonstrate PrPCWD or alternate
prion protein conformers in blood—one of the
grails of prion biology and epidemiology.
The identification of blood-borne prion
transmission has been sought before with mixed
results (9–11). Bovine spongiform encephalopathy
and scrapie have been transmitted to naBve
sheep through the transfer of 500 ml of blood
or buffy coat white blood cells from infected
sheep (12, 13). In addition, limited but compelling
evidence argues for the transmission of variant
Creutzfeldt-Jakob disease (vCJD) through blood
from asymptomatic donors (14–16). Even in
sporadic CJD, PrPres has been found in periph-
eral organs of some patients (17). The present
work helps establish that prion diseases can be
transmitted through blood.
The presence of infectious CWD prions in
saliva may explain the facile transmission of
CWD. Cervid-to-cervid interactions (SOM text),
especially in high density and captive situations,
would be expected to facilitate salivary crosscontact
(11, 18, 19). Salivary dissemination of
prions may not be limited to CWD. Proteaseresistant
prion protein has been demonstrated in
the oral mucosa, taste buds, lingual epithelium,
vomeronasal organ, and olfactory mucosa of
hamsters infected with transmissible mink
encephalopathy (19) and ferrets infected with
CWD (20). Although no instance of CWD
transmission to humans has been detected, the
present results emphasize the prudence of using
impervious gloves during contact with saliva or
blood of cervids that may be CWD-infected.
Environmental contamination by excreta
from infected cervids has traditionally seemed
the most plausible explanation for the dissemination
of CWD (21). However, we could not
detect PrPCWD in cohort 3 deer inoculated repeatedly
with urine and feces from CWDþ deer and examined up to 18 months pi (Table 2).
There are several reasons to view this negative
finding cautiously, including small sample size,
elective preclinical termination, and potential
variation in individual susceptibility that may
be associated with the 96 G/S polymorphism in
the PRNP gene (7, 22). Although no genotype
of white-tailed deer is resistant to CWD infection,
PRNP genotypes S/S or G/S at codon 96
appear to have reduced susceptibility manifest
by longer survival (7). Both deer in cohort 3
(urine and feces) were subsequently shown to
be of the PRNP 96 G/S genotype. Thus, it is
possible, although we think unlikely, that these
deer had a prolonged incubation period (918
months pi) before the amplification of PrPCWD
became detectable in tissues. Recent studies
have shown that PrPres is poorly preserved
after incubation with intestinal or fecal content
(23, 24). Further research using cervid and surrogate
cervid PrP transgenic mice (25) are indicated
to continue to address the presence of
infectious CWD prions in excreta of CWDþ deer and to
provide a more substantial basis for
reconsideration of the assumption that excreta
are the chief vehicle for CWDdissemination and
transmission.
The results reported here provide a plausible
basis for the efficient transmission of CWD in
nature. We demonstrate that blood and saliva in
particular are able to transmit CWD to naBve deer
and produce incubation periods consistent with
those observed in naturally acquired infections
(3, 26). The time from exposure to first detection
of PrPCWD by tonsil biopsy was variable—as
short as 3 months but as long as 18 months (likely
underestimates due to sampling frequency).
The results also reinforce a cautious view of the
exposure risk presented by body fluids, excreta,
and all tissues from CWDþ cervids. ...
SNIP...END
http://www.sciencemag.org/cgi/content/abstract/314/5796/133
http://www.sciencemag.org/
While it is early in the investigation of these events, New York officials have documented several facts: (1) the owner of the index captive herd was also a wildlife rehabilitator, including white-tailed deer, and that wild deer were commingled with his captive herd and subsequently released back to the wild; (2) he was also a taxidermist, possibly having handled animals that originated in CWD-endemic regions in other states; and (3) he had recently sold captive stock to additional cervid farms within New York (the state has quarantined these herds).
http://www.nwhc.usgs.gov/publications/wildlife_health_bulletins/WHB_05_01.jsp
What Every Taxidermist Should Know
About Chronic Wasting Disease (CWD)
snip...
What actions have been taken to prevent the spread of CWD?
The movement of high-risk carcass parts (brain, spinal cord, lymph tissues) is a potential avenue through which CWD could be spread from infected areas. Investigations in New York indicate that the infection could have been spread by a taxidermist who accepted specimens from CWD-positive states, allowed rehabilitated fawns access to the taxidermy workshop and spread potentially infectious curing salt waste as a fence line weed killer on his deer
Updated 8/24/06 1 of 1
farm. Several states, including Pennsylvania, have developed regulations to prohibit the importation of high-risk carcass parts from CWD endemic states. As of April 1, 2006 Pennsylvania’s importation ban prohibits the importation of high-risk carcass parts from Colorado, Illinois, Kansas, Nebraska, New Mexico, New York (CWD containment area only), South Dakota, Utah, West Virginia (Hampshire County only), Wisconsin, Wyoming and the Canadian provinces of Alberta and Saskatchewan. Pennsylvanians hunting in CWD-positive areas should get their animals tested and should leave high-risk carcass parts in the area where the animal was hunted.
snip...
http://www.pgc.state.pa.us/pgc/lib/pgc/taxidermists_cwd.pdf
CWD AND ENVIRONMENTAL FACTORS i.e. saliva, fecal shedding and fecal-oral transmission is likely
http://p079.ezboard.com/fwolftracksproductionsfrm2.showMessage?topicID=592.topic
From: TSS (216-119-163-189.ipset45.wt.net) Subject: CWD aka MAD DEER/ELK TO HUMANS ??? Date: September 30, 2002 at 7:06 am PST
From: "Belay, Ermias" To: Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias" Sent: Monday, September 30, 2002 9:22 AM Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Dear Sir/Madam, In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.
That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: 404-639-3091). Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated.
Ermias Belay, M.D. Centers for Disease Control and Prevention
-----Original Message----- From: Sent: Sunday, September 29, 2002 10:15 AM To: rr26k@nih.gov; rrace@niaid.nih.gov; ebb8@CDC.GOV Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Sunday, November 10, 2002 6:26 PM ......snip........end..............TSS
also,
A. Aguzzi - Chronic Wasting Disease (CWD) also needs to be addressed. Most serious because of rapid horizontal spread and higher prevalence than BSE in UK, up to 15% in some populations. Also may be a risk to humans - evidence that it is not dangerous to humans is thin.
http://www.tseandfoodsafety.org/activities/bse_conference_basel_april_02/2summary_of_conference.htm
PLUS, i like to never forget this, but just what the hell would human cwd look like??? which one, or all of the 6 documented phenotypes of the sporadic CJD might cwd be envolved with, first passage, 2nd, 3rd etc, direct route and or friendly fire??? will they all look the same pathologically???
HOUND STUDY (CSE???)
http://www.priondata.org/data/A_deerdog.html#Hound
HOUND STUDY
AS implied in the Inset 25 we must not _ASSUME_ that transmission of BSE to other species will invariably present pathology typical of a scrapie-like disease.
snip...
http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf
Image] Research letters Volume 352, Number 9134 [Image] 3 October 1998 [Previous] [Next]
[Image][Image]Simultaneous occurrence of spongiform encephalopathy in a man and his cat in Italy [Image]
Gianluigi Zanusso, Ettore Nardelli, Anna Rosati, GianMaria Fabrizi, Sergio Ferrari, Antonella Carteri, Franco De Simone, Nicola Rizzuto, Salvatore Monaco
Transmissible spongiform encephalopathies (TSE) encompass inherited, acquired, and sporadic mammalian neurological disorders, and are characterised by the conversion of the cellular prion protein (PrP) in an insoluble and protease-resistant isoform (PrPres). In human TSE, four types of PrPres have been identified according to size and glycoform ratios, which may represent different prion strains. Type-1 and type-2 PrPres are associated with sporadic Creutzfeldt-Jakob disease (CJD), type 3 with iatrogenic CJD, and type 4 with variant CJD.1,2 There is evidence that variant CJD is caused by the bovine spongiform encephalopathy (BSE)-prion strain.2-4 The BSE strain has been identified in three cats with feline spongiform encephalopathy (FSE), a prion disease which appeared in 1990 in the UK.5 We report the simultaneous occurrence of sporadic CJD in a man and a new variety of FSE in his cat.
A 60-year-old man, with no unusual dietary habits, was admitted in November, 1993, because of dysarthria, cerebellar ataxic gait, visual agnosia, and myoclonus. An electroencephalogram (EEG) showed diffuse theta-delta activity. A brain magnetic resonance imaging scan was unremarkable. 10 days later, he was speechless and able to follow only simple commands. Repeat EEGs showed periodic triphasic complexes. 2 weeks after admission, he was mute, akinetic, and unable to swallow. He died in early January, 1994.
His 7-year-old, neutered, female shorthaired cat presented in November, 1993, with episodes of frenzy, twitching of its body, and hyperaesthesia. The cat was usually fed on canned food and slept on its owner's bed. No bites from the cat were recalled. In the next few days, the cat became ataxic, with hindquarter locomotor dysfunction; the ataxia got worse and there was diffuse myoclonus. The cat was killed in mid-January, 1994.
No pathogenic mutations in the patient's PrP gene were found. The patient and the cat were methionine homozygous at codon 129. Histology of the patient's brain showed neocortical and cerebellar neuronal loss, astrocytosis, and spongiosis (figure A). PrP immunoreactivity showed a punctate pattern and paralleled spongiform changes (figure B). The cat's brain showed mild and focal spongiosis in deeper cortical layers of all four lobes (figure C), vacuolated cortical neurons (figure D), and mild astrogliosis. The cerebellar cortex and the dentate nucleus were gliosed. Immunoreactive PrP showed a punctate pattern in neocortex, allocortex, and caudate nucleus (figure E). Western blot analysis of control and affected human and cat brain homogenates showed 3 PrP bands of 27-35 kDa. After digestion with proteinase K and deglycosylation, only samples from the affected patient and cat showed type-1 PrPres, with PrP glycoform ratios comparable to those observed in sporadic CJD1 (details available from author).
[Image]
Microscopic sections of patient and cat brains
A: Occipital cortex of the patient showing moderate spongiform degeneration and neuronal loss (haematoxylin and eosin) and B: punctate perineuronal pattern of PrP immunoreactivity; peroxidase immunohistochemistry with monoclonal antibody 3F4. C: cat parietal cortex showing mild spongiform degeneration (haematoxylin and eosin).D: vacuolated neurons (arrow, haematoxylin and eosin), E: peroxidase immunohistochemistry with antibody 3F4 shows punctate perineuronal deposition of PrP in temporal cortex.
This study shows a spatio-temporal association between human and feline prion diseases. The clinical features of the cat were different from previously reported cases of FSE which were characterised by gradual onset of behavioural changes preceding locomotor dysfunction and ataxia.5 Neuropathological changes were also at variance with the diffuse spongiosis and vacuolation of brainstem neurons, seen in FSE.5 The synaptic pattern of PrP deposition, similar in the cat and in the patient, was atypical for a BSE-related condition. Evidence of a new type of FSE was further provided by the detection of a type-1 PrPres, other than the BSE-associated type 4.2 Taken together, our data suggest that the same agent strain of sporadic CJD was involved in the patient and in his cat.
It is unknown whether these TSE occurred as the result of horizontal transmission in either direction, infection from an unknown common source, or the chance occurrence of two sporadic forms.
1 Parchi P, Castellani R, Capellari S, et al. Molecular basis of phenotypic variablity in sporadic Creutzfeldt-Jakob disease. Ann Neurol 1996; 39: 767-78 [PubMed].
2 Collinge J, Sidle KCL, Meads J, Ironside J, Hill AF. Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD. Nature 1996; 383: 685-90 [PubMed].
3 Bruce ME, Will RG, Ironside JW, et al. Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent. Nature 1997; 389: 498-501 [PubMed].
4 Hill AF, Desbruslais M, Joiner S, et al. The same prion strain causes vCJD and BSE. Nature 1997; 389: 448-50 [PubMed].
5 Pearson GR, Wyatt JM, Henderson JP, Gruffydd-Jones TJ. Feline spongiform encephalopathy: a review. Vet Annual 1993; 33: 1-10.
------------------------------------------------------------------------ Sezione di Neurologie Clinica, Dipartimento di Scienze Neurologiche e della Visione, Università di Verona, Policlinico Borgo Roma, 37134 Verona, Italy (S Monaco; e mail rizzuto@Gorgorna.univr.it); and Istituto Zooprofilattico Sperimentale della Lombardia e dell' Emilia, Brescia
=========================================
PET FOODS MAD CATS AND MAD DOGS BSE/TSEs
worse still, there is serious risk the media could get to hear of such a meeting...
snip...
Crushed heads (which inevitably involve brain and spinal cord material) are used to a limited extent but will also form one of the constituent raw materials of meat and bone meal, which is used extensively in pet food manufacturer...
http://www.bseinquiry.gov.uk/files/yb/1989/03/17004001.pdf
2. The Parliamentary Secretary said that he was concerned about the possibility that countries in which BSE had not yet been detected could be exporting raw meat materials (in particular crushed heads) contaminated with the disease to the UK for use in petfood manufacture...
snip...
YOU explained that imported crushed heads were extensively used in the petfood industry...
http://www.bseinquiry.gov.uk/files/yb/1989/04/14001001.pdf
In particular I do not believe one can say that the levels of the scrapie agent in pet food are so low that domestic animals are not exposed...
http://www.bseinquiry.gov.uk/files/yb/1989/04/24003001.pdf
40. When Sir Richard Southwood gave oral evidence to the Agriculture Select Committee, he said:
'The case of the cat is the first case of spongiform encephalopathy ever in a cat. Well, it appears to be - but if poor moggie got a bit of the staggers, you took it along to the vet and said it was not very happy, it would be put to sleep in the past and no one would have paid £300 to have a post mortem undertaken. So it is just possible that these were occurring before and were in fact caught from moggie being fed scrapie offal from sheep. I cannot say.'[46]
NEXT, ask yourself how many cats and dogs have ever been tested for TSE in the USA ???
Research Project: Transmission, Differentiation, and Pathobiology of Transmissible Spongiform Encephalopathies Location: Virus and Prion Diseases of Livestock
Title: Identification and Characterization of U.S. Bse Cases
Authors
Hall, S - USDA, APHIS, VS, NVSL Richt, Juergen Davis, A - USDA, APHIS, VS, NVSL Kluge, J - USDA, APHIS, VS, NVSL Simmons, M - VETERINARY LABS AGENCY,UK Stack, M - VETERINARY LABS AGENCY,UK Spencer, Y - VETERINARY LABS AGENCY,UK
Submitted to: Meeting Abstract Publication Type: Abstract Publication Acceptance Date: April 25, 2006 Publication Date: May 28, 2006 Citation: Hall, S.M., Richt, J., Davis, A., Kluge, J., Simmons, M., Stack, M., Spencer, Y. 2006. Identification and characterization of U.S. BSE cases [abstract]. Prion Diseases of Domestic Livestock. p. 25.
Technical Abstract: Bovine Spongiform Encephalopathy (BSE) surveillance has been ongoing in the USA since the early 1990¿s and initial testing was done at the USDA, National Veterinary Services Laboratories (NVSL) utilizing routine histopathology exclusively. In 1995, the immunohistochemistry (IHC) test was incorporated into surveillance testing in addition to routine histopathology. By 1999 virtually all BSE screening was performed by IHC and by 2001 the NVSL had switched to an automated IHC procedure. In 2002 and 2003 the NVSL tested about 20,000 high risk animals each year by IHC. In December, 2003 an animal was identified by IHC as positive for BSE (Case 1); this animal was determined to be imported from Canada. After this animal was identified, in June 2004 the USDA began its enhanced surveillance program as a shared effort between selected state veterinary diagnostic laboratories and NVSL, as part of the National Animal Health Laboratory Network. The plan called for testing as many targeted high risk animals as possible in a 12-18 month period. From June 1, 2004 through March 21, 2006, over 650,000 animals have been tested (Bio-Rad ELISA). Of those tested, two animals (Cases 2 and 3) have been identified as positive for BSE. While all three cases were strongly positive by Bio-Rad ELISA, Cases 2 and 3 have common features which are distinct from Case 1. Definitive spongiform changes in the obex, strong immunohistochemical reactions, and Western blot patterns similar to European BSE cases were observed in Case 1. In contrast, Cases 2 and 3 did not contain definitive histological lesions of BSE and the IHC staining was less intense than Case 1. In addition, Cases 2 (approximately 12 years) and Case 3 (approximately ten years) were older animals while Case 1 was 6.5 years old. Western blot analysis, PrP**Sc from Case 1 showed molecular features similar to typical BSE isolates, whereas PrP**Sc from Cases 2 and 3 revealed an unusual molecular PrP**Sc pattern: molecular mass of the unglycosylated and monoglycosylated isoform was higher than that of typical BSE isolates. Case 1 contained more PrP**Sc per brain tissue mg equivalent compared with Cases 2 and 3 using antibody 6H4. In Western Blot analysis, Case 2 and Case 3 were strongly positive with antibody P4, while Case 1 was negative or weakly positive with P4.
http://www.ars.usda.gov/research/publications/publications.htm?seq_no_115=194279
Differences in tissue distribution could require new regulations regarding specific risk material (SRM) removal.
snip...end
full text 33 PAGES ;
http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf
http://www.bseinquiry.gov.uk/files/yb/1988/10/00001001.pdf
It was, however, performed in the USA in 1979, when it was shown that cattle inoculated with the scrapie agent endemic in the flock of Suffolk sheep at the United States Department of Agriculture in Mission, Texas, developed a TSE quite unlike BSE. 32 The findings of the initial transmission, though not of the clinical or neurohistological examination, were communicated in October 1988 to Dr Watson, Director of the CVL, following a visit by Dr Wrathall, one of the project leaders in the Pathology Department of the CVL, to the United States Department of Agriculture. 33 The results were not published at this point, since the attempted transmission to mice from the experimental cow brain had been inconclusive. The results of the clinical and histological differences between scrapie-affected sheep and cattle were published in 1995. Similar studies in which cattle were inoculated intracerebrally with scrapie inocula derived from a number of scrapie-affected sheep of different breeds and from different States, were carried out at the US National Animal Disease Centre. 34 The results, published in 1994, showed that this source of scrapie agent, though pathogenic for cattle, did not produce the same clinical signs of brain lesions characteristic of BSE.
http://www.bseinquiry.gov.uk/
FULL TEXT ;
http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf
THE SEVEN SCIENTIST REPORT ***
http://www.fda.gov/ohrms/dockets/dockets/02n0273/02n-0273-EC244-Attach-1.pdf
Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)
http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf
[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and Requirement for the Disposition of Non-Ambulatory Disabled Cattle
03-025IFA 03-025IFA-2 Terry S. Singeltary
9/13/2005
http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518
CHRONIC WASTING DISEASE, CERVIDS - USA (NEW MEXICO) (02) ******************************************************** A ProMED-mail post
ProMED-mail is a program of the International Society for Infectious Diseases
Sponsored in part by Elsevier, publisher of Infectious Disease Gateway Alert
Date: Tue, 13 Dec 2005 11:53:52 -0600 From: Terry S Singeltary Sr Source: NM Press Release, 9 Dec 2005 [edited]
2 elk killed in the southern Sacramento Mountains of south east New Mexico have tested positive for chronic wasting disease (CWD), the Department of Game and Fish announced. The animals were the first elk in New Mexico to test positive for CWD since the disease was first discovered in mule deer in 2002.
Both CWD-afflicted elk were killed in an area 10 to 15 miles southeast of Cloudcroft in Game Management Unit 34, the same general area where the state's most recent case of CWD was detected in a mule deer. One of the elk -- a mature male -- was taken on 3 Oct 2005 by a hunter and showed no symptoms of the disease. The other elk -- a yearling female -- was in very poor condition and unable to stand when a Department of Game and Fish conservation officer found it 1 Oct 2005. Testing and verification of the samples required about 2 months. Future testing is expected to occur more quickly as the Department of Game and Fish and the Veterinary Diagnostic Services in the New Mexico Department of Agriculture further implement recently achieved in-state CWD testing capabilities.
"The range in which the disease is found appears to be expanding, so finding it in more animals in that area is not surprising," said Kerry Mower, the department's lead wildlife disease biologist. "But it is disappointing to find our first cases of CWD in free-ranging elk."
Brain stem samples from the 2 elk were among more than 100 taken from deer and elk in Unit 34 this year and sent to the Veterinary Diagnostic Services Laboratory in Albuquerque. The Albuquerque laboratory's "presumptive positive" samples from the 2 elk were confirmed as CWD-positive by the National Veterinary Services Laboratory in Ames, Iowa.
"We will continue our efforts to monitor the disease by actively testing animals in Units 34 and 19," Mower said. "We also encourage all hunters statewide to submit their animals for testing." The department personally informs hunters if the tests are positive. Hunters will be able to see the complete list of test results as they become available on the department website: .
This season, hunters who kill animals in a "control area" of Unit 34 are required to submit their animals for testing and observe special regulations affecting which body parts of a deer or elk can be removed from the unit. Hunting seasons continue in that area into January. Chronic wasting disease is a fatal neurological illness that afflicts deer, elk and moose. There is no evidence of CWD being transmitted to humans or livestock. The disease causes animals to become emaciated, display abnormal behavior, and lose control of bodily functions. To date, it has been found in captive and wild deer, elk, and moose in 8 states and 2 Canadian provinces.
The origin of CWD in New Mexico is unknown. It has been found in 12 wild deer and 2 wild elk since 2002, when the disease was first discovered east of Las Cruces. All of the CWD-positive deer and elk in New Mexico were from the southern Sacramento Mountains southeast of Cloudcroft and areas surrounding the Organ Mountains near Las Cruces.
For more information about CWD in New Mexico, including special regulations and how hunters can assist in research and prevention, visit . More information about CWD also can be found on the Chronic Wasting Disease Alliance site at or on the Colorado Division of Wildlife site at
CWD control map New Mexico (30 Aug 2005) .
-- Terry S Singeltary
[A clearer map but only up to July 2004, is at
- MHJ.Mod]
[see also: Chronic wasting disease, cervids - USA (NM) 20050628.1827 2003 --- Chronic wasting disease, cervids - USA (NM) (02) 20030217.0414 Chronic wasting disease, cervids - USA (NM) 20030207.0328 2002 --- Chronic wasting disease, cervids - USA (New Mexico) (02) 20020620.4548 Chronic wasting disease, cervids - USA (New Mexico) 20020619.4535]
......................mhj/pg/sh
*##########################################################* ************************************************************ ProMED-mail makes every effort to verify the reports that are posted, but the accuracy and completeness of the information, and of any statements or opinions based thereon, are not guaranteed. The reader assumes all risks in using information posted or archived by ProMED-mail. ISID and its associated service providers shall not be held responsible for errors or omissions or held liable for any damages incurred as a result of use or reliance upon posted or archived material. ************************************************************ Please support ProMED-mail by donating to the 2005 Internet- a-thon at ************************************************************ Visit ProMED-mail's web site at . Send all items for posting to: promed@promedmail.org (NOT to an individual moderator). If you do not give your full name and affiliation, it may not be posted. Send commands to subscribe/unsubscribe, get archives, help, etc. to: majordomo@promedmail.org. For assistance from a human being send mail to: owner-promed@promedmail.org. ############################################################
----- Original Message ----- From: "Terry S. Singeltary Sr." To: Cc: Sent: Wednesday, October 04, 2006 10:56 AM Subject: Re: PRO/AH/EDR> CJD (new var.) update 2006 (10)
Greetings ProMED,
Thank you for that kind correction from last months CJD ProMED posting, and further to the data on such atypical BSE cases in the USA, are as follows ;
4 October 2006 - The final minutes (154 KB) of the 93rd SEAC meeting held on 6 July 2006 have been published.
snip...
The committee will hold a reserved business session in the afternoon to allow discussion of unpublished studies on BSE in cattle. This is in accordance with the SEAC Code of Practice. Short summaries of the open and reserved business discussions will be posted on the SEAC website next week.
snip...
. The Chair noted that recent reports described two cases of BSE in cattle in the United States of America (USA) as being similar to atypical cases of BSE found in a number of European countries. The Chair suggested that the term "atypical BSE", used in the USA report, is potentially confusing and that this would be discussed under any other business. Dr Danny Matthews (Veterinary Laboratories Agency [VLA]) explained that data from western blots of the USA cases resembled that of a small number of atypical cases of BSE in France. A study of the French cases had shown the condition to be transmissible to mice by intracerebral (ic) inoculation with the neuropathological phenotype maintained on transmission3. Claims have been made about the existence of atypical cases of BSE in other countries but these have yet to be confirmed. No study has yet examined the tissue distribution of abnormal prion protein (PrPSc) or infectivity in such atypical cases of BSE. ...
snip...
http://www.seac.gov.uk/minutes/final93.pdf
[Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)
snip...
3. WHY still now only partial ruminant feed ban, with the fact that now we seem to have 3 cases of nvCJD to humans i.e. humanbovineTSE that were responsible from blood, and the fact the last 2 mad cows documented in the USA were that of an Atypical strain, would it not seem prudent to remove blood as well from ruminant feed ?
Page 4 of 98
8/3/2006
WOULD it not seem prudent to improve and expand the SRM list now? as per your own thinking ;
If transmission occurs, tissue distribution comparisons will be made between cattle infected with the atypical BSE isolate and the U.S. BSE isolate. Differences in tissue distribution could require new regulations regarding specific risk material (SRM) removal.
FULL text ;
Research Project: Study of Atypical Bse Location: Virus and Prion Diseases of Livestock Project Number: 3625-32000-073-07 Project Type: Specific C/A Start Date: Sep 15, 2004 End Date: Sep 14, 2007
Objective:
The objective of this cooperative research project with Dr. Maria Caramelli from the Italian BSE Reference Laboratory in Turin, Italy, is to conduct comparative studies with the U.S. bovine spongiform encephalopathy (BSE) isolate and the atypical BSE isolates identified in Italy. The studies will cover the following areas: 1. Evaluation of present diagnostics tools used in the U.S. for the detection of atypical BSE cases. 2. Molecular comparison of the U.S. BSE isolate and other typical BSE isolates with atypical BSE cases. 3. Studies on transmissibility and tissue distribution of atypical BSE isolates in cattle and other species.
Approach:
This project will be done as a Specific Cooperative Agreement with the Italian BSE Reference Laboratory, Istituto Zooprofilattico Sperimentale del Piemonte, in Turin, Italy. It is essential for the U.S. BSE surveillance program to analyze the effectiveness of the U.S diagnostic tools for detection of atypical cases of BSE. Molecular comparisons of the U.S. BSE isolate with atypical BSE isolates will provide further characterization of the U.S. BSE isolate. Transmission studies are already underway using brain homogenates from atypical BSE cases into mice, cattle and sheep. It will be critical to see whether the atypical BSE isolates behave similarly to typical BSE isolates in terms of transmissibility and disease pathogenesis. If transmission occurs, tissue distribution comparisons will be made between cattle infected with the atypical BSE isolate and the U.S. BSE isolate. Differences in tissue distribution could require new regulations regarding specific risk material (SRM) removal.
http://www.ars.usda.gov/research/projects/projects.htm?ACCN_NO=408490
Page 5 of 98 8/3/2006
HOWEVER, JAPAN has already shown infectivity in tissues other than CNS in there atypical TSE in cattle, so why should we wait, and expose many to this agent needlessly, since the last two mad cows in the USA were also atypical TSE ? PrPSc distribution of a natural case of bovine spongiform encephalopathy
Yoshifumi Iwamaru, Yuka Okubo, Tamako Ikeda, Hiroko Hayashi, Mori- kazu Imamura, Takashi Yokoyama and Morikazu Shinagawa Priori Disease Research Center, National Institute of Animal Health, 3-1-5 Kannondai, Tsukuba 305-0856 Japan gan@affrc.go.jp
Abstract ... snip...full text 98 pages ;
http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf
Published online before print February 17, 2004, 10.1073/pnas.0305777101
Medical Sciences Identification of a second bovine amyloidotic spongiform encephalopathy: Molecular similarities with sporadic Creutzfeldt-Jakob disease
http://www.pnas.org/cgi/content/abstract/0305777101v1
J Gen Virol 85 (2004), 2471-2478; DOI 10.1099/vir.0.79889-0 Characterization of two distinct prion strains derived from bovine spongiform encephalopathy transmissions to inbred mice
http://vir.sgmjournals.org/cgi/content/abstract/85/8/2471
[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and Requirement for the Disposition of Non-Ambulatory Disabled Cattle
03-025IFA 03-025IFA-2
http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf
SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 1997 TO 2004. SPORADIC CJD CASES TRIPLED;
please note 'TYPE UNKNOWN' ???
http://www.cjdsurveillance.com/resources-casereport.html
kind regards,
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518
----- Original Message ----- From: "ProMED-mail" To: Sent: Monday, October 02, 2006 10:22 AM Subject: PRO/AH/EDR> CJD (new var.) update 2006 (10)
CJD (NEW VAR.) UPDATE 2006 (10) ******************************* A ProMED-mail post
ProMED-mail is a program of the International Society for Infectious Diseases
[The definition of the designations deaths, definite cases, probable vCJD cases, and the case definitions can be found by accessing the Department of Health website, or by reference to a previous ProMED-mail post in this thread (for example, CJD (new var.) - UK: update March 2002 20020305.3693).
Data on vCJD cases from other parts of the world are now included in these updates whenever available.
Also, data on other forms of CJD (sporadic, iatrogenic, familial and GSS) are now included when they have some relevance to the incidence and etiology of vCJD. - Mod.CP]
In this update: [1] UK: Department of Health monthly CJD statistics, Mon 2 Oct 2006 [2] Germany: characterization of atypical BSE: correction
****** [1] UK: vCJD and CJD statistics Date: Mon 2 Oct 2006 From: ProMED-mail Source: UK Department of Health, Monthly Creutzfeldt-Jakob Disease Statistics, Mon 2 Oct 2006 [edited]
The Department of Health is today [Mon 2 Oct 2006] issuing the latest information about the numbers of known cases of Creutzfeldt-Jakob disease. This includes cases of variant Creutzfeldt-Jakob disease [abbreviated in ProMED-mail as CJD (new var.) or vCJD], the form of the disease thought to be linked to BSE (bovine spongiform encephalopathy).
Definite and probable CJD cases in the UK, as at Fri 29 Sep 2006: ----------------------------------------------------------------- Summary of vCJD cases - deaths ------------------------------ Deaths from definite vCJD (confirmed): 112 Deaths from probable vCJD (without neuropathological confirmation): 44 Deaths from probable vCJD (neuropathological confirmation pending): 0 Number of deaths from definite or probable vCJD (as above): 156
Summary of vCJD cases - alive ----------------------------- Number of probable vCJD cases still alive: 6
Total ----- Number of definite or probable vCJD (dead and alive): 162 (The next table will be published on Mon 6 Nov 2006).
Since the previous monthly statistics were released on Mon 4 Sep 2006, the total number of deaths from definite vCJD remains unchanged at 156, and the overall total number of definite or probable vCJD cases (dead and alive) also remains unchanged at 162 for the second month in succession.
These data are consistent with the view that the vCJD outbreak in the UK is in decline. The total number of deaths due to vCJD in the UK remains 156. The peak number of deaths was 28 in the year 2000, followed by 20 in 2001, 17 in 2002, 18 in 2003, and 9 in 2004, 5 in 2005. The number of deaths due to definite or probable vCJD in the UK during the first 9 months of 2006 remains 3.
Totals for all types of CJD cases in the UK in 2005 and 2006 ------------------------------------------------------------ As of 29 Sep 2006, in the UK in the year 2005, there were 122 referrals of suspected CJD, and there were 65 deaths from sporadic CJD, 6 from familial CJD, 3 from iatrogenic CJD, 6 GSS (Gerstmann-Straussler-Scheinker) syndrome cases, and 5 deaths from vCJD.
The corresponding figures so far for the 1st 9 months of 2006 are: 81 referrals, 41 deaths from sporadic CJD, 3 from vCJD, 4 from familial CJD, 3 from GSS and one from iatrogenic CJD.
During the period 1995, when vCJD was first diagnosed, up to the present there have been 937 deaths from all forms of CJD including 156 deaths attributable to definite or probable vCJD.
[These data are accessible via
. - Mod.CP]
-- ProMED-mail
****** [2] Correction Date: Tue 5 Sep 2006 From: "Terry S Singeltary Sr"
Characterization of atypical BSE in Germany: correction ------------------------------------------------------- [In the Moderator's comment accompanying the abstract of the paper entitled "Atypical BSE in Germany-Proof of transmissibility and biochemical characterization'" by A Buschmannaet et al, (see part [2] of CJD (new var.) update 2006 (09) 20060904.2519) it was wrongly implied that Terry S Singeltary Sr endorsed the conclusions of the paper, whereas his comments were intended merely to highlight the conclusions of the paper. Namely that the atypical cases suggested the possible existence of sporadic BSE cases in bovines and perhaps the BSE epidemic in the UK could have also been initiated by an intraspecies transmission from a sporadic BSE case. I apologize for inadvertently misrepresenting Terry's views. - Mod.CP]
Terry S Singeltary Sr has written the following. "In fact I disagree with the spontaneous/sporadic BSE/TSE theory, IF this is what the authors of this paper meant by 'sporadic BSE' to mean. For one thing, it has never been proven. IF atypical BSE i.e. BASE is so similar to some sporadic CJDs, then how did they all of a sudden become spontaneous? Could it not be so simple as an atypical BSE i.e. BASE was transmitted the same way most of all of the other BSE cattle were i.e. feed of just an atypical source, thus causing atypical strain? Why would these animals not develop an atypical BSE i.e. BASE from the same oral route? WHAT about an atypical strain mutating to become infectious via a lateral or horizontal mode in the bovine, as with CWD and scrapie? Also, please explain to me how a distinct synthetic prion, of a strain that is supposedly unlike any other we have ever seen, how can this explain 6 different documented phenotypes of sporadic CJD to date?
It's like trying to explain away all the 6 phenotypes of sporadic CJD with the spontaneous theory, it's just not scientific. OR, if you render an atypical TSE of what ever phenotype, in what ever species, of the atypical strain and feed it to another whatever species, nothing happens x 1 x 2 x 3 x 4 etc passage? This all has been proven?
Please show me these transmission studies? What Prusiner and Soto produced in vitro did not look like any natural field TSE, and as far as this in vitro TSE being infectious, well this was questionable too. If this was the case, then why does CWD not spontaneously happen in geographical areas where it has never been documented, OR with scrapie, as in scrapie free New Zealand? If TSE were to arise spontaneously, I don't see how the scientific arena can dictate which animal TSE can arise spontaneously, and which ones cannot, without any scientific evidence to support this to date, and by even suggesting this in this study, was not scientific. The words sporadic and spontaneous are very confusing in the world literature of human and animal TSE and, in my opinion, should not be used as terminology of any TSE."
-- Terry S Singeltary Sr
[see also: CJD (new var.) update 2006 (09) 20060904.2519 CJD (new var.) update 2006 (08) 20060807.2207 CJD (new var.) update 2006 (07) 20060703.1831 CJD (new var.) - Netherlands: 2nd case 20060623.1741 CJD (new var.) update 2006 (06) 20060605.1566 CJD (new var.) update 2006 (05) 20060508.1332 CJD (new var.) update 2006 (04) 20060404.1005 CJD (new var.) update 2006 (03) 20060306.0728 CJD (new var.) - UK: 3rd transfusion-related case 20060209.0432 CJD (new var.) update 2006 (02) 20060206.0386 CJD (new var.) update 2006 (01) 20060111.0101 CJD (new var.) update 2006 20060111.0101 2005 --- CJD (new var.) update 2005 (12) 20051209.3547 CJD (new var.) update 2005 (11) 20051108.3270 CJD (new var.) update 2005 (10) 20051006.2916 CJD (new var.) update 2005 (09) 20050905.2627 CJD (new var.) update 2005 (08) 20050801.2237 CJD (new var.) update 2005 (07) 20050703.1889 CJD (new var.) update 2005 (06) 20050607.1584 CJD (new var.) update 2005 (05) 20050505.1243 CJD (new var.) update 2005 (04) 20050405.0982 CJD (new var.) update 2005 (03) 20050308.0687 CJD (new var.) update 2005 (02) 20050211.0467 CJD (new var.) - UK: update 2005 (01) 20050111.0095 2004 --- CJD, genetic susceptibility 20041112.3064 CJD (new var.) - UK: update 2004 (14) 20041206.3242 CJD (new var.) - UK: update 2004 (13) 20041103.2977 CJD (new var.) - UK: update 2004 (12) 20041023.2871 CJD (new var.) - UK: update 2004 (11) 20041008.2758 CJD (new var.) - UK: update 2004 (10) 20040909.2518 CJD (new var.) - UK: update 2004 (09) 20040809.2199 CJD (new var.) - UK: update 2004 (08) 20040806.2150 CJD (new var.) - UK: update 2004 (07) 20040706.1807 CJD (new var.) - UK: update 2004 (06) 20040608.1535 CJD (new var.) - UK: update 2004 (05) 20040510.1262 CJD (new var.) - UK: update 2004 (04) 20040406.0937 CJD (new var.) - UK: update 2004 (03) 20040314.0713 CJD (new var.) - UK: update 2004 (02) 20040202.0400 CJD (new var.) - UK: update 2004 (01) 20040106.0064 CJD (new var.) - France: 8th case 20041022.2864 CJD (new var.) - France: 9th case 20041123.3138 CJD (new var.), blood supply - UK 20040318.0758 CJD (new var.), carrier frequency study - UK 20040521.1365 2003 --- CJD (new var.) - UK: update 2003 (13) 20031216.3072 CJD (new var.) - UK: update 2003 (01) 20030108.0057 2002 --- CJD (new var.) - UK: update Dec 2002 20021207.5997 CJD (new var.) - UK: update Jan 2002 20020111.3223 2001 --- CJD (new var.), incidence & trends - UK (02) 20011124.2875 CJD (new var.), incidence & trends - UK 20011115.2816 CJD (new var.) - UK: reassessment 20011029.2671 CJD (new var.) - UK: update Oct 2001 20011005.2419 CJD (new var.) - UK: regional variation (02) 20010907.2145 CJD (new var.) - UK: update Sep 2001 20010906.2134 CJD (new var.) - UK: update Aug 2001 20010808.1872 CJD (new var.) - UK: 9th Annual Report 20010628.1231 CJD (new var.) - UK: update June 2001 20010622.1188 CJD (new var.) - UK: update 3 Jan 2001 20010104.0025]
................cp/sh
*##########################################################* ************************************************************ ProMED-mail makes every effort to verify the reports that are posted, but the accuracy and completeness of the information, and of any statements or opinions based thereon, are not guaranteed. The reader assumes all risks in using information posted or archived by ProMED-mail. ISID and its associated service providers shall not be held responsible for errors or omissions or held liable for any damages incurred as a result of use or reliance upon posted or archived material. ************************************************************ Become a ProMED-mail Premium Subscriber at
************************************************************ Visit ProMED-mail's web site at . Send all items for posting to: promed@promedmail.org (NOT to an individual moderator). If you do not give your full name and affiliation, it may not be posted. Send commands to subscribe/unsubscribe, get archives, help, etc. to: majordomo@promedmail.org. For assistance from a human being send mail to: owner-promed@promedmail.org. ############################################################
2006 mad cow protein to deer
e) "Big Jim's" BBB Deer Ration, Big Buck Blend, Recall # V-104-6;
every wonder why they are so big......mad cow protein ;
http://www.fda.gov/bbs/topics/enforce/2006/ENF00963.html
2003D-0186 Guidance for Industry: Use of Material From Deer and Elk In Animal Feed
EMC 7 Terry S. Singeltary Sr. Vol #: 1
-------- Original Message --------
Subject: DOCKET-- 03D-0186 -- FDA Issues Draft Guidance on Use of Material From Deer and Elk in Animal Feed; Availability Date: Fri, 16 May 2003 11:47:37 -0500 From: "Terry S. Singeltary Sr." To: fdadockets@oc.fda.gov
Greetings FDA,
i would kindly like to comment on;
Docket 03D-0186
FDA Issues Draft Guidance on Use of Material From Deer and Elk in Animal Feed; Availability
Several factors on this apparent voluntary proposal disturbs me greatly, please allow me to point them out;
1. MY first point is the failure of the partial ruminant-to-ruminant feed ban of 8/4/97. this partial and voluntary feed ban of some ruminant materials being fed back to cattle is terribly flawed. without the _total_ and _mandatory_ ban of all ruminant materials being fed back to ruminants including cattle, sheep, goat, deer, elk and mink, chickens, fish (all farmed animals for human/animal consumption), this half ass measure will fail terribly, as in the past decades...
2. WHAT about sub-clinical TSE in deer and elk? with the recent findings of deer fawns being infected with CWD, how many could possibly be sub-clinically infected. until we have a rapid TSE test to assure us that all deer/elk are free of disease (clinical and sub-clinical), we must ban not only documented CWD infected deer/elk, but healthy ones as well. it this is not done, they system will fail...
3. WE must ban not only CNS (SRMs specified risk materials), but ALL tissues. recent new and old findings support infectivity in the rump or ass muscle. wether it be low or high, accumulation will play a crucial role in TSEs.
4. THERE are and have been for some time many TSEs in the USA. TME in mink, Scrapie in Sheep and Goats, and unidentified TSE in USA cattle. all this has been proven, but the TSE in USA cattle has been totally ignored for decades. i will document this data below in my references.
5. UNTIL we ban all ruminant by-products from being fed back to ALL ruminants, until we rapid TSE test (not only deer/elk) but cattle in sufficient numbers to find (1 million rapid TSE test in USA cattle annually for 5 years), any partial measures such as the ones proposed while ignoring sub-clinical TSEs and not rapid TSE testing cattle, not closing down feed mills that continue to violate the FDA's BSE feed regulation (21 CFR 589.2000) and not making freely available those violations, will only continue to spread these TSE mad cow agents in the USA. I am curious what we will call a phenotype in a species that is mixed with who knows how many strains of scrapie, who knows what strain or how many strains of TSE in USA cattle, and the CWD in deer and elk (no telling how many strains there), but all of this has been rendered for animal feeds in the USA for decades. it will get interesting once someone starts looking in all species, including humans here in the USA, but this has yet to happen...
6. IT is paramount that CJD be made reportable in every state (especially ''sporadic'' cjd), and that a CJD Questionnaire must be issued to every family of a victim of TSE. only checking death certificates will not be sufficient. this has been proven as well (see below HISTORY OF CJD -- CJD QUESTIONNAIRE)
7. WE must learn from our past mistakes, not continue to make the same mistakes...
REFERENCES
Oral transmission and early lymphoid tropism of chronic wasting disease PrPres in mule deer fawns (Odocoileus hemionus ) Christina J. Sigurdson1, Elizabeth S. Williams2, Michael W. Miller3, Terry R. Spraker1,4, Katherine I. O'Rourke5 and Edward A. Hoover1
Department of Pathology, College of Veterinary Medicine and Biomedical Sciences, Colorado State University, Fort Collins, CO 80523- 1671, USA1 Department of Veterinary Sciences, University of Wyoming, 1174 Snowy Range Road, University of Wyoming, Laramie, WY 82070, USA 2 Colorado Division of Wildlife, Wildlife Research Center, 317 West Prospect Road, Fort Collins, CO 80526-2097, USA3 Colorado State University Veterinary Diagnostic Laboratory, 300 West Drake Road, Fort Collins, CO 80523-1671, USA4 Animal Disease Research Unit, Agricultural Research Service, US Department of Agriculture, 337 Bustad Hall, Washington State University, Pullman, WA 99164-7030, USA5
Author for correspondence: Edward Hoover.Fax +1 970 491 0523. e-mail ehoover@lamar.colostate.edu
Mule deer fawns (Odocoileus hemionus) were inoculated orally with a brain homogenate prepared from mule deer with naturally occurring chronic wasting disease (CWD), a prion-induced transmissible spongiform encephalopathy. Fawns were necropsied and examined for PrP res, the abnormal prion protein isoform, at 10, 42, 53, 77, 78 and 80 days post-inoculation (p.i.) using an immunohistochemistry assay modified to enhance sensitivity. PrPres was detected in alimentary-tract-associated lymphoid tissues (one or more of the following: retropharyngeal lymph node, tonsil, Peyer's patch and ileocaecal lymph node) as early as 42 days p.i. and in all fawns examined thereafter (53 to 80 days p.i.). No PrPres staining was detected in lymphoid tissue of three control fawns receiving a control brain inoculum, nor was PrPres detectable in neural tissue of any fawn. PrPres-specific staining was markedly enhanced by sequential tissue treatment with formic acid, proteinase K and hydrated autoclaving prior to immunohistochemical staining with monoclonal antibody F89/160.1.5. These results indicate that CWD PrP res can be detected in lymphoid tissues draining the alimentary tract within a few weeks after oral exposure to infectious prions and may reflect the initial pathway of CWD infection in deer. The rapid infection of deer fawns following exposure by the most plausible natural route is consistent with the efficient horizontal transmission of CWD in nature and enables accelerated studies of transmission and pathogenesis in the native species.
snip...
These results indicate that mule deer fawns develop detectable PrP res after oral exposure to an inoculum containing CWD prions. In the earliest post-exposure period, CWD PrPres was traced to the lymphoid tissues draining the oral and intestinal mucosa (i.e. the retropharyngeal lymph nodes, tonsil, ileal Peyer's patches and ileocaecal lymph nodes), which probably received the highest initial exposure to the inoculum. Hadlow et al. (1982) demonstrated scrapie agent in the tonsil, retropharyngeal and mesenteric lymph nodes, ileum and spleen in a 10-month-old naturally infected lamb by mouse bioassay. Eight of nine sheep had infectivity in the retropharyngeal lymph node. He concluded that the tissue distribution suggested primary infection via the gastrointestinal tract. The tissue distribution of PrPres in the early stages of infection in the fawns is strikingly similar to that seen in naturally infected sheep with scrapie. These findings support oral exposure as a natural route of CWD infection in deer and support oral inoculation as a reasonable exposure route for experimental studies of CWD.
snip...
http://vir.sgmjournals.org/cgi/content/full/80/10/2757
Subject: MAD DEER/ELK DISEASE AND POTENTIAL SOURCES Date: Sat, 25 May 2002 18:41:46 -0700 From: "Terry S. Singeltary Sr." Reply-To: BSE-L To: BSE-L
8420-20.5% Antler Developer For Deer and Game in the wild Guaranteed Analysis Ingredients / Products Feeding Directions
snip...
_animal protein_
http://www.surefed.com/deer.htm
BODE'S GAME FEED SUPPLEMENT #400 A RATION FOR DEER NET WEIGHT 50 POUNDS 22.6 KG.
snip...
_animal protein_
http://www.bodefeed.com/prod7.htm
Ingredients
Grain Products, Plant Protein Products, Processed Grain By-Products, Forage Products, Roughage Products 15%, Molasses Products, __Animal Protein Products__, Monocalcium Phosphate, Dicalcium Pyosphate, Salt, Calcium Carbonate, Vitamin A Acetate with D-activated Animal Sterol (source of Vitamin D3), Vitamin E Supplement, Vitamin B12 Supplement, Riboflavin Supplement, Niacin Supplement, Calcium Panothenate, Choline Chloride, Folic Acid, Menadione Soduim Bisulfite Complex, Pyridoxine Hydorchloride, Thiamine Mononitrate, d-Biotin, Manganous Oxide, Zinc Oxide, Ferrous Carbonate, Calcium Iodate, Cobalt Carbonate, Dried Sacchoromyces Berevisiae Fermentation Solubles, Cellulose gum, Artificial Flavors added.
http://www.bodefeed.com/prod6.htm ===================================
MORE ANIMAL PROTEIN PRODUCTS FOR DEER
Bode's #1 Game Pellets A RATION FOR DEER F3153
GUARANTEED ANALYSIS Crude Protein (Min) 16% Crude Fat (Min) 2.0% Crude Fiber (Max) 19% Calcium (Ca) (Min) 1.25% Calcium (Ca) (Max) 1.75% Phosphorus (P) (Min) 1.0% Salt (Min) .30% Salt (Max) .70%
Ingredients
Grain Products, Plant Protein Products, Processed Grain By-Products, Forage Products, Roughage Products, 15% Molasses Products, __Animal Protein Products__, Monocalcium Phosphate, Dicalcium Phosphate, Salt, Calcium Carbonate, Vitamin A Acetate with D-activated Animal Sterol (source of Vitamin D3) Vitamin E Supplement, Vitamin B12 Supplement, Roboflavin Supplement, Niacin Supplement, Calcium Pantothenate, Choline Chloride, Folic Acid, Menadione Sodium Bisulfite Complex, Pyridoxine Hydrochloride, Thiamine Mononitrate, e - Biotin, Manganous Oxide, Zinc Oxide, Ferrous Carbonate, Calcium Iodate, Cobalt Carbonate, Dried Saccharyomyces Cerevisiae Fermentation Solubles, Cellulose gum, Artificial Flavors added.
FEEDING DIRECTIONS Feed as Creep Feed with Normal Diet
http://www.bodefeed.com/prod8.htm
INGREDIENTS
Grain Products, Roughage Products (not more than 35%), Processed Grain By-Products, Plant Protein Products, Forage Products, __Animal Protein Products__, L-Lysine, Calcium Carbonate, Salt, Monocalcium/Dicalcium Phosphate, Yeast Culture, Magnesium Oxide, Cobalt Carbonate, Basic Copper Chloride, Manganese Sulfate, Manganous Oxide, Sodium Selenite, Zinc Sulfate, Zinc Oxide, Sodium Selenite, Potassium Iodide, Ethylenediamine Dihydriodide, Vitamin E Supplement, Vitamin A Supplement, Vitamin D3 Supplement, Mineral Oil, Mold Inhibitor, Calcium Lignin Sulfonate, Vitamin B12 Supplement, Menadione Sodium Bisulfite Complex, Calcium Pantothenate, Riboflavin, Niacin, Biotin, Folic Acid, Pyridoxine Hydrochloride, Mineral Oil, Chromium Tripicolinate
DIRECTIONS FOR USE
Deer Builder Pellets is designed to be fed to deer under range conditions or deer that require higher levels of protein. Feed to deer during gestation, fawning, lactation, antler growth and pre-rut, all phases which require a higher level of nutrition. Provide adequate amounts of good quality roughage and fresh water at all times.
http://www.profilenutrition.com/Products/Specialty/deer_builder_pellets.html ===================================================
DEPARTMENT OF HEALTH & HUMAN SERVICES PUBLIC HEALTH SERVICE FOOD AND DRUG ADMINISTRATION
April 9, 2001 WARNING LETTER
01-PHI-12 CERTIFIED MAIL RETURN RECEIPT REQUESTED
Brian J. Raymond, Owner Sandy Lake Mills 26 Mill Street P.O. Box 117 Sandy Lake, PA 16145 PHILADELPHIA DISTRICT
Tel: 215-597-4390
Dear Mr. Raymond:
Food and Drug Administration Investigator Gregory E. Beichner conducted an inspection of your animal feed manufacturing operation, located in Sandy Lake, Pennsylvania, on March 23, 2001, and determined that your firm manufactures animal feeds including feeds containing prohibited materials. The inspection found significant deviations from the requirements set forth in Title 21, code of Federal Regulations, part 589.2000 - Animal Proteins Prohibited in Ruminant Feed. The regulation is intended to prevent the establishment and amplification of Bovine Spongiform Encephalopathy (BSE) . Such deviations cause products being manufactured at this facility to be misbranded within the meaning of Section 403(f), of the Federal Food, Drug, and Cosmetic Act (the Act).
Our investigation found failure to label your swine feed with the required cautionary statement "Do Not Feed to cattle or other Ruminants" The FDA suggests that the statement be distinguished by different type-size or color or other means of highlighting the statement so that it is easily noticed by a purchaser.
In addition, we note that you are using approximately 140 pounds of cracked corn to flush your mixer used in the manufacture of animal feeds containing prohibited material. This flushed material is fed to wild game including deer, a ruminant animal. Feed material which may potentially contain prohibited material should not be fed to ruminant animals which may become part of the food chain.
The above is not intended to be an all-inclusive list of deviations from the regulations. As a manufacturer of materials intended for animal feed use, you are responsible for assuring that your overall operation and the products you manufacture and distribute are in compliance with the law. We have enclosed a copy of FDA's Small Entity Compliance Guide to assist you with complying with the regulation... blah, blah, blah...tss
http://www.fda.gov/foi/warning_letters/g1115d.pdf
SNIP...FULL TEXT ;
http://www.fda.gov/ohrms/dockets/dailys/03/oct03/100203/100203.htm
TONS Products manufactured from 02/01/2005 until 06/06/2006 Date: August 6, 2006 at 6:16 pm PST PRODUCT a) CO-OP 32% Sinking Catfish, Recall # V-100-6; b) Performance Sheep Pell W/Decox/A/N, medicated, net wt. 50 lbs, Recall # V-101-6; c) Pro 40% Swine Conc Meal -- 50 lb, Recall # V-102-6; d) CO-OP 32% Sinking Catfish Food Medicated, Recall # V-103-6;
*********************************
e) "Big Jim's" BBB Deer Ration, Big Buck Blend, Recall # V-104-6;
*********************************
f) CO-OP 40% Hog Supplement Medicated Pelleted, Tylosin 100 grams/ton, 50 lb. bag, Recall # V-105-6; g) Pig Starter Pell II, 18% W/MCDX Medicated 282020, Carbadox -- 0.0055%, Recall # V-106-6; h) CO-OP STARTER-GROWER CRUMBLES, Complete Feed for Chickens from Hatch to 20 Weeks, Medicated, Bacitracin Methylene Disalicylate, 25 and 50 Lbs, Recall # V-107-6; i) CO-OP LAYING PELLETS, Complete Feed for Laying Chickens, Recall # 108-6; j) CO-OP LAYING CRUMBLES, Recall # V-109-6; k) CO-OP QUAIL FLIGHT CONDITIONER MEDICATED, net wt 50 Lbs, Recall # V-110-6; l) CO-OP QUAIL STARTER MEDICATED, Net Wt. 50 Lbs, Recall # V-111-6; m) CO-OP QUAIL GROWER MEDICATED, 50 Lbs, Recall # V-112-6 CODE Product manufactured from 02/01/2005 until 06/06/2006 RECALLING FIRM/MANUFACTURER Alabama Farmers Cooperative, Inc., Decatur, AL, by telephone, fax, email and visit on June 9, 2006. FDA initiated recall is complete.
REASON
Animal and fish feeds which were possibly contaminated with ruminant based protein not labeled as "Do not feed to ruminants".
VOLUME OF PRODUCT IN COMMERCE
125 tons
DISTRIBUTION AL and FL
END OF ENFORCEMENT REPORT FOR AUGUST 2, 2006
###
http://www.fda.gov/bbs/topics/enforce/2006/ENF00963.html
snip...end...TSS
Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518
***UPDATE JANUARY 20, 2007
Increase of chronic-wasting in wild deer in Alberta & Saskatchewan
Jan, 15 2007 - 3:30 PM
EDMONTON - More cases of deadly chronic-wasting disease in wild deer are showing up in Saskatchewan and Alberta.
Saskatchewan officials say already 30 deer shot by hunters this fall have tested positive for the fatal disease.
In Alberta, there have been four cases since the fall in areas near the Saskatchewan boundary.
Officials in both provinces report some of the cases are showing up in new areas - a finding that suggests the disease may be spreading.
Scientists and wildlife officials are working to contain the disease before it ravages the deer population and jumps to other species, such as caribou.
Tests on about 450 white-tailed deer shot this fall in northwestern Ontario have so far come back negative. (BN, ccg)
http://www.630ched.com/news/news_local.cfm?cat=7428218912&rem=56168&red=80121823aPBIny&wids=410&gi=1&gm=news_local.cfm
SASKATCHEWAN
CWD Sample Totals Total Heads Submitted: 4280 As of January 9, 2007, hunters have turned in 1717 heads from white-tailed deer; 2447 heads from mule deer and 116 elk heads to test for CWD. Total Head Tested: So far 2821 heads have been tested with 26 confirmed positive results for CWD.
http://www.se.gov.sk.ca/fishwild/cwd_info.htm
ALBERTA
Monthly and Cumulative CWD Testing Totals in 2006
*Antelope, reindeer, moose **All results are negative, except for four wild mule deer detected in February 2006, one wild mule deer detected in March 2006, two wild mule deer detected in April 2006 and three wild mule deer confirmed by the CFIA detected in April 2006; TOTAL = 10 (9 cases from Alberta and 1 from Saskatchewan during the winter programs).
http://www1.agric.gov.ab.ca/$department/deptdocs.nsf/all/cpv9448
http://www1.agric.gov.ab.ca/$department/deptdocs.nsf/all/agdex3594
Subject: REPORT OF THE COMMITTEE ON CAPTIVE WILDLIFE AND ALTERNATIVE LIVESTOCK annual report (CWD) 2006 Date: January 15, 2007 at 7:37 pm PST
REPORT OF THE COMMITTEE ON CAPTIVE WILDLIFE AND ALTERNATIVE LIVESTOCK annual report (CWD) 2006
snip...
Drs. Dean Goeldner and Tom Gidlewski, VS, APHIS, USDA, presented the APHIS-VS chronic wasting disease (CWD) program update. CWD has been discovered in free-ranging cervids in 11 states and 41 captive cervid herds in nine states. There are currently four infected elk herds and one infected white-tailed deer herd that have chosen to remain under quarantine instead of depopulate. In 2006, the CWD program depopulated one elk herd in the endemic area which turned out to be infected as well as a chronically infected white-tailed deer herd and a mixed elk and white-tailed deer herd for a total of approximately 110 animals. For the last three years, the program has paid for testing about 15,000 captive cervids per year. Demand for testing is expected to increase with the implementation of the program. The first infected free-ranging white-tailed deer was found in northwest Kansas in 2006. On the positive side, New York found no additional positive free-ranging cervids in 2006 but West Virginia found four additional animals in Hampshire County. Wisconsin continues to aggressively battle CWD with over 100,000 animals submitted for testing since 2000 and over 650 positive deer identified. The infected area appears to be largely limited to the original counties. Interestingly, the number of deer in the Wisconsin endemic area does not appear to be decreasing despite the large number of animals that have been removed. Colorado has stopped culling deer in "hot spots" as they believe that it was not very successful. Alberta,
Canada continues to find more positive white-tailed deer adjacent to the infected Saskatchewan areas.
Appropriate tissue collection and submission for CWD diagnosis includes obex, medial retropharygeal lymph nodes and palatine tonsils. Submission of an ear with the official eartag attached or submission of fresh tissue accompanied by an appropriately executed chain of evidence document will allow DNA comparison in the event of a positive diagnosis. Archiving herd blood samples on special collection cards is also a way to compare DNA in the event of a positive diagnosis in the future. All positive cases are verified by two pathologists and the presumptive positive tissues are completely retested at the National Veterinary Services Laboratory (NVSL). Rectal biopsy continues to be examined as a tool for CWD ante-mortem diagnosis. Hundreds of animals have been examined and the results look promising. Larger numbers need to be examined in order to make final conclusions. Retrospective epidemiologic analysis and transgenic mouse research in 2006 still support the theory that CWD does not appear to affect people or non-cervids animals.
APHIS received approximately $18.5 million in appropriated CWD funding in FY 2006 including $2.44 million in congressional earmarks. The FY 2007 appropriations have not been passed by Congress; the president’s budget requests $15.4 million for CWD. On July 21, 2006, APHIS published its final CWD rule. The final rule added moose and all Cervus species to the previously announced deer and elk species covered in the herd certification program. It expanded the term "captive" to "farmed and captive", maintained a five-year surveillance standard for surveillance, clarified that two positive official tests are needed for a CWD diagnosis, reduced the minimum testing age to 12 months, adjusted commingling buffers, eliminated the 48-hour exemption for short-term commingling, changed the identification (ID) requirement to one official ID and one ID unique within the herd, and added the reporting of escapes and disappearances. Grandfathering of state programs will be accomplished through Memorandum of Understanding (MOUs) with the states followed by reviews of state programs for consistency with federal requirements. The interstate movement requirements maintained a "ramping up" process to reach the five year surveillance standard. An exemption was created for direct movement to slaughter. A permit will be required for interstate movement of research animals and two IDs will be required for wild cervids captured for translocation and release. Subsequent to publication of the rule, three petitions were received from organizations representing state agencies and officials challenging the interstate movement provisions in the rule and requesting a stay in the rule’s implementation. The petitions challenged the scientific basis for initially allowing the interstate movement of animals with only one or two years of surveillance. They also took issue with the federal preemption language in the rule. According to USDA legal counsel, federal preemption on interstate movement is implicit in all APHIS regulations; it was made explicit in this case in response to a comment on the proposed rule. Nevertheless, APHIS believes the petitions merit further consideration. On September 8, 2006, APHIS published a notice of delay of implementation for the rule. The petitions will be published soon for public comment. APHIS intends to resolve the issues quickly so that a final rule can be implemented as the state-federal-industry program it is intended to be.
Dr. Robert Kunkle, National Animal Disease Center (NADC), Agricultural Research Center (ARS), USDA, presented a time-specific Committee paper entitled "Experimental Transmission of Chronic Wasting Disease (CWD) of Elk (Cervus elapus nelsoni), White-tailed Deer
(Odocoileus virginianus), and Mule Deer (Odocoileus hemionus hemionus) to White-tailed Deer by Intracerebral Route. This paper is included in its entirety in these proceedings.
Dr. Michael Miller, Senior Wildlife Veterinarian, Colorado Division of Wildlife, provided an overview of recent progress in understanding various aspects of chronic wasting disease (CWD) epidemiology, diagnosis, and control. Dr. Miller used the occurrence of CWD in a moose to hypothesize that the potential natural host range of CWD may be predicted based on similarities between the native prion protein of known hosts (deer, wapiti, and moose) and other cervid species. He also reviewed findings related to CWD transmission and showed that simulation models of epidemic dynamics based on relatively simple transmission assumptions suggest that CWD is likely to persist in wild deer populations and depress population performance over time. Dr. Miller next described highlights of a new study on PrPCWD distribution in experimentally-infected mule deer that demonstrated marked genetic effects on CWD progression but not susceptibility in this species, and discussed the potential implications for CWD epidemiology. He then shared preliminary data on use of rectal mucosa biopsy to detect CWD infections in live white-tailed and mule deer, which suggest that rectal biopsy likely will be a useful herd screening test and surveillance tool provided PrP genotype data are available for sampled individuals. Dr. Miller concluded his presentation with a brief summary of unsuccessful attempts to control CWD in north central Colorado, emphasizing the challenges and obstacles that likely make eradication of CWD from the wild infeasible given present technology.
snip...
Dr. Keith Rohr presented a resolution on "The use of the ELISA test to diagnose Chronic Wasting Disease in Captive Wildlife". After discussion and modification to the original submission, the resolution was passed by the Committee and will be referred to the Committee on Nominations and Resolutions. Resolution passed by the Committee and referred to the Committee on Nominations and Resolution.
Experimental Transmission of Chronic Wasting Disease (CWD) of Elk (Cervus elaphus nelsoni), White-tailed Deer (Odocoileus virginianus), and Mule Deer (Odocoileus hemionus hemionus) to White-tailed Deer by Intracerebral Route
R.A. Kunkle, A.N. Hamir, J.M. Miller, J.A. Richt, J.J. Greenlee
National Animal Disease Center
Agriculture Research Center
United States Department of Agriculture
S.M. Hall
National Veterinary Services Laboratories, USDA-VS-APHIS
Animal and Plant Health Inspection Service
Veterinary Services
United States Department of Agriculture
E.S. Williams
Wyoming Veterinary Laboratory
University of Wyoming
Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy (TSE) affecting elk, white-tailed deer, and mule deer. Intra-species transmission of CWD is readily accomplished via oral administration of CWD-affected brain, and while the exact mode of natural transmission is unclear, horizontal transmission within species has been demonstrated.
The TSE’s are prion-associated diseases. Different prion strains are associated with variations in clinical course and pathology in susceptible animal hosts. To determine the potential existence of CWD pathotype strain differences, groups of five white-tailed deer were inoculated by intracerebral route (IC) with 1 ml of 10% (wt/vol) brain homogenates derived from CWD-affected elk, white-tailed deer, or mule deer. Two non-inoculated deer served as negative controls. All deer were homozygous at PrP gene polymorphic sites 95 (glutamine) and 138 (serine). Deer homozygous (glycine/glycine) or heterozygous (glycine/serine) at codon 96 were approximately equally divided between treatment groups. One deer from each treatment group was euthanized 10 months post-inoculation (PI); findings for these three deer were similar and included limited or mild spongiform encephalopathy (SE) and immunohistochemical (IHC) detection of prion in lymphoid tissue follicles and in the CNS, especially in subependymal areas. All remaining deer were euthanized at the terminal stage of disease. The clinical course of CWD appeared similar between groups. The survival period did not differ between groups, ranging from 14 to 26 months, with an average mean of 20 months. The severity of SE and magnitude of IHC staining appeared proportional to incubation period. Microscopic lesions in the CNS were typical of previously reported CWD SE, including the presence of cerebral florid plaques. IHC staining was multifocally extensive to diffuse, and was perineuronal, subependymal, and neuropil associated. Staining was pronounced in the midbrain, but relatively sparse in the hippocampus. Differences in histopathologic and IHC findings between groups were not noted. Negative control deer sacrificed at 26 months PI did not have SE and were IHC negative. The composite findings indicate the clinical course and pathology of CWD in IC challenged white-tailed deer was not influenced by species of the inoculum source or by PrP gene polymorphism at codon 96 in recipients.
http://www.usaha.org/committees/reports/2006/report-cwal-2006.pdf
UNITED STATES ANIMAL HEALTH ASSOCIATION – 2006
RESOLUTION NUMBER: 13 APPROVED
SOURCE: COMMITTEE ON CAPTIVE WILDLIFE
AND ALTERNATIVE LIVESTOCK
SUBJECT MATTER: THE USE OF THE ENZYME LINKED IMMUNOSORBENT ASSAY (ELISA) TEST TO DIAGNOSE CHRONIC WASTING DISEASE IN CAPTIVE WILDLIFE
DATES: MINNEAPOLIS, MINNESOTA – OCTOBER 12-18, 2006
BACKGROUND INFORMATION:
The enzyme-linked immunosorbent assay (ELlSA) for chronic wasting disease (CWD) is approved and licensed for free roaming mule deer, white tailed deer and elk. There is ample data indicating essentially equal sensitivity and specificity of ELISA tests compared to immunohistochemistry (IHC). The ELISA test can be done with faster turnaround times and is more efficient for the laboratory and requires fewer personnel than IHC. The ELISA test positives can be confirmed by IHC conducted by laboratory personnel who are experienced in identifying the obex and lymph node tissue to ensure proper tissue submission. More timely laboratory results are needed for producers to move animal product, to verify CWD status and for proper disposal of potentially CWD positive animals.
RESOLUTION:
The United States Animal Health Association (USAHA) requests that the United States Department of Agriculture (USDA), Animal and Plant Health Inspection Service (APHIS), Veterinary Services (VS) approve the USDA licensed enzyme-linked immunosorbent assay (ELISA) test for use on cervid species within the captive wildlife industry.
http://www.usaha.org/committees/resolutions/2006/resolution13-2006.pdf
Drs. Dean Goeldner and Tom Gidlewski, VS-APHIS-USDA provided updated information to the committee regarding APHIS-VS efforts directed at chronic wasting disease (CWD). CWD has been discovered in free-ranging cervids in 11 states and in 41 captive cervid herds in nine states. There are currently four infected elk herds and one infected white-tailed deer herd that have chosen to remain under quarantine instead of depopulate.
In 2006, the CWD program depopulated one elk herd in the endemic area, which turned out to be infected, as well as a chronically infected white-tailed deer herd and a mixed elk and white-tailed deer herd for a total of approximately 110 animals. For the last three years, the program has paid for testing about 15,000 captive cervids per year. Demand for testing is expected to increase with the implementation of the program.
Rectal biopsy continues to be examined as a tool for CWD ante-mortem diagnosis. Hundreds of animals have been examined and the results look promising. Larger numbers need to be examined in order to make final conclusions.
On July 21, 2006, APHIS published its final CWD rule. Subsequently three petitions were received from organizations representing state agencies and officials challenging the interstate movement provisions in the rule and requesting a stay in the rule’s implementation. Believing the petitions merit further consideration, APHIS published a notice of delay of implementation for the rule on September 8, 2006. The petitions will be published soon for public comment.
APHIS received approximately $18.5 million in appropriated CWD funding in FY 2006 including $2.44 million in congressional earmarks. The FY 2007 appropriations have not been passed by Congress; the president’s budget requests $15.4 million for CWD. APHIS again made $5 million in CWD cooperative agreement funding available to state wildlife agencies in FY 2006. The formula for distributing the funds was revised after consultation with the Association of Fish and Wildlife Agencies. Forty-nine states applied for and received funding. APHIS also provided $750,000 for tribal CWD activities, the funding going to the Native American Fish and Wildlife Society and 20 individual tribes. After internal discussions, it was decided to leave the state and tribal wildlife cooperative agreements for CWD on a fiscal year basis, rather than moving them to a calendar year basis in FY 2007 with other VS agreements.
As some states reduce the amount of hunter-killed surveillance for CWD, APHIS is urging those states to utilize targeted and road-killed surveillance to increase the likelihood of detecting the disease.
Dr. Michael Miller, Senior Wildlife Veterinarian with the Colorado Division of Wildlife, provided a brief and lucid overview of recent progress and remaining needs in understanding various aspects of chronic wasting disease (CWD) epidemiology, diagnosis, and control. Dr. Miller alerted committee members to several upcoming research publications on CWD epidemic dynamics, host range, prion distribution patterns, management efforts, and new antemortem diagnostic approaches, and posed five questions that he considers most important to answer in future research: "Can we reliably predict the host range of CWD using non-experimental approaches?" "Why is CWD transmission so efficient?" "Can CWD foci arise from natural exposure to the scrapie agent?" "Does CWD affect populations and ecosystems?" "What can we reasonably do to control CWD?"
The remainder of Dr. Miller’s presentation focused on opportunities for improving the efficiency of surveillance to detect new CWD foci in free-ranging wildlife by using structured, non-random sampling approaches. This strategy has been adopted by the World Organziation for Animal Health (OIE) and accepted internationally as the standard for bovine spongiform encephalopathy (BSE) surveillance. As outlined in the OIE International Animal Health Code ("Surveillance for bovine spongiform encephalopathy", Appendix 3.8.4, OIE 2006, http://www.oie.int/eng/normes/mcode/en_chapitre_3.8.4.htm), the structured, non-random
sampling strategy developed for BSE uses a point-based quota system that weights sample sources based on the likelihood of detecting disease in that subpopulation. This contrasts to the standard random sampling paradigm presently used as the basis for most CWD surveillance programs, wherein all sample sources are assumed equal with respect to disease detection probability and epidemiological "value." According to Dr. Miller, the main advantage of OIE’s structured, non-random sampling approach is that it would allow agencies to combine several survey approaches (e.g., targeted surveillance, vehicle-kill surveys, and harvest surveys) in a straightforward and epidemiologically meaningful way, adding value for "high risk" samples (e.g., cervids showing clinical sighs of CWD). Dr. Miller suggested that we already have sufficient knowledge about CWD epidemiology to adopt this approach to ongoing surveillance. Critical elements would be defining target populations based on biological criteria, choosing desired prevalence thresholds for detection of foci, establishing a timeframe for sample collection based on natural disease course, calculating "point" quotas per target population based on prevalence thresholds, and assigning values to subpopulation (source) samples based on likelihood of infection as reflected published field data (e.g., males > females, middle-aged individuals > young, clinical suspects > vehicle kills > harvested animals). Dr. Miller concluded by pointing out that although the most immediate applications of this structured, non-random sampling surveillance approach might be in CWD surveillance, similar strategies could be devised to improve the efficiency of surveillance for detecting foci of other diseases like bovine tuberculosis or avian influenza in free-ranging wildlife populations.
Dr. Kevin Keel, Southeastern Cooperative Wildlife Disease Study reported that on September 9, 2005 the West Virginia Division of Natural Resources announced that a two-and-a-half year-old buck was determined to be positive for CWD. This deer was found in Hampshire County in close proximity to Virginia, Maryland and Pennsylvania. Special collection teams were dispatched to Hampshire County to collect deer within five miles of the index case. Of the 195 deer sampled from September 14 to October 14, four additional deer were found to be positive for CWD. Subsequently, during the first three days of the firearms seasons, 1,016 hunter-killed deer were sampled from throughout Hampshire County; however, none of the hunter-killed deer tested positive. A second special collection of 80 deer within one mile of the known positives led to the detection of four additional deer that were positive for CWD.
Initial observations suggested that the distribution of hunter-killed samples was relatively uniform throughout the County. However an evaluation of the number of hunter-killed samples at close proximity to the known positives revealed the statistical limitations of the sample size. The 256 samples collected within five miles of known positives are sufficient to detect CWD at a prevalence of 1.2% with a 95% confidence interval. However, the 17 hunter-killed samples collected within one mile of known positives were only sufficient to detect CWD at a prevalence of 16% or greater with a 95% confidence interval. The special collections resulted in the sampling of 146 deer in the one-mile margin. The total of 166 deer sampled within this region would be 95% certain to detect CWD at a prevalence of 1.5% or greater. The data available suggest that CWD is currently confined to a very small region in Hampshire County.
http://www.usaha.org/committees/reports/2006/report-wd-2006.pdf
Chronic Wasting Disease
Chronic Wasting Disease (CWD), a TSE, is a fatal neurological disease of
farmed and wild deer and elk in North America. CWD is generally similar to BSE,
and is thought to be caused by a similar type of infectious prion protein. CWD
differs from BSE in a number of significant ways, however, including the types of
tissues involved and the fact that it is contagious among animals in a herd. A study
under experimental conditions suggested that CWD may be transmissible through
contaminated environments long after infectious animals were no longer present.110
Since 1997, CWD has been detected in captive cervid herds in nine states:
Colorado, Kansas, Minnesota, Montana, Nebraska, New York, Oklahoma, South
Dakota, and Wisconsin. As of July 2006, there were six known positive captive
herds in the United States: four elk herds in Colorado, and one deer herd each in
Wisconsin and Minnesota. Federal and state policy is to depopulate (destroy) these
herds (see below). CWD has been detected in wild cervids in 11 states: Colorado,
Illinois, Kansas, Nebraska, New Mexico, New York, South Dakota, Utah, West
Virginia, Wisconsin, and Wyoming.111 It has also been found in Canada and the
Republic of Korea.
The Centers for Disease Control and Prevention (CDC) says regarding the
potential for CWD transmission to humans:
It is generally prudent to avoid consuming food derived from any animal with
evidence of a TSE (a “transmissible spongiform encephalopathy,” or prion
disease such as BSE and CWD). To date, there is no evidence that CWD has
been transmitted or can be transmitted to humans under natural conditions.
However, there is not yet strong evidence that such transmissions could not
occur. To further assess the possibility that the CWD agent might occasionally
cause disease in humans, additional epidemiologic and laboratory studies could
be helpful. Such studies include molecular characterization and strain typing of
the agents causing CWD in deer and elk and CJD (the human form of prion
disease) in potentially exposed patients. Ongoing national surveillance for CJD
and other neurological cases will remain important for continuing to assess the
risk, if any, of CWD transmission to humans.112
CRS-50
113 Ibid.
114 FDA, Guidance for Industry #158: Use of Material from Deer and Elk in Animal Feed,
Sept. 15, 2003, at [http://www.fda.gov/cvm/Documents/guide158.pdf].
115 See ARS research website to search for CWD studies at [http://www.ars.usda.gov/
Research/Research.htm].
116 USDA/ DOI, Plan for Assisting States, Federal Agencies, and Tribes in Managing
Chronic Wasting Disease in Wild and Captive Cervids, June 26, 2002.
117 71 Federal Register 41682.
With regard to the potential for CWD transmission to cattle, possibly causing
BSE or a related disease that could pose a food safety hazard, USDA says:
During the approximately two decades of monitoring, researchers have not found
any evidence that CWD can be transmitted to domestic cattle under natural
conditions. Ongoing experiments involving oral exposure and contact exposure
on heavily CWD contaminated sites have not resulted in infection of cattle.
These experiments, however, require additional time before they are completed.
CWD has been experimentally transmitted by artificial means to mice, ferrets,
mink, goats, squirrel monkeys, and calves.113
FDA prohibits the feeding of rendered deer and elk to ruminants. In addition,
FDA prohibits the use of known-CWD positive animals in any animal feeds, and
recommends against the use of rendered deer and elk material considered high-risk
in any animal feeds.114
Activities related to CWD control are also conducted by USDA’s Agricultural
Research Service (ARS) and Cooperative State Research, Education and Extension
Service (CSREES)115 and several agencies in the Department of the Interior (DOI).
In recognition that CWD is being found in more areas, and that resource limitations
and program inconsistencies exist among the states, a national CWD Task Force was
formed in 2002 “to ensure that federal and state agencies cooperate in the
development and implementation of an effective national CWD program.”116 This
task force, initiated between USDA, DOI, and state wildlife and agriculture agencies,
produced the strategic plan (see footnote) which, among other things, states that the
primary federal role will be to provide coordination and assistance with research,
surveillance, disease management, diagnostic testing, technology, communications,
information, education, and funding for state CWD programs. The task force has
working groups with action plans organized around most of these topics, though
there have been concerns about delays in its implementation.
APHIS, whose regulations govern cooperative programs to control animal
diseases, had published a final rule in the July 21, 2006, Federal Register to establish
a captive herd certification program for CWD, and rules on interstate movement of
cervids.117 The rule was to take effect on October 19, 2006, but APHIS published (in
the September 8, 2006 Federal Register) an indefinite delay in this effective date.
The agency had received petitions from the Association of Fish and Wildlife
Agencies, the U. S. Animal Health Association, and the National Assembly of State
Animal Health Officials raising concerns about the rule, such as whether federal
CRS-51
118 Chronic Wasting Disease Alliance, “CWD Update,” September 13, 2006, at
http://www.cwd-info.org/index.php/fuseaction/news.detail/ID/31747682f9f71e0d5d12e12a8f322288
119 Progress Report on the Plan for Assisting States, Federal Agencies and Tribes in
Managing Chronic Wasting Disease in Wild and Captive Herds (October 2002-September
2003), May 2004.
120 Cornell Feline Health Center, “Mad Cow Disease and Cats,” accessed Sept. 20, 2006, at
[http://www.vet.cornell.edu/fhc/resources/madcow.htm].
interstate movement regulations should preempt state requirements for importation,
and the scientific basis underlying federal interstate movement requirements.118
According to the 2004 CWD task force’s progress report, total federal spending
for CWD approximates $25 million annually, of which nearly $20 million are USDA
funds. The report estimates state spending to be roughly $15-20 million per year.119
Several bills addressing research, surveillance, or control of CWD were introduced
in the 108th Congress, including H.R. 2057; H.R. 2430; H.R. 2431; H.R. 2636; H.R.
3714, and S. 1036; S. 1366 and S. 2007. A hearing on CWD was held by the Senate
Environment and Public Works Committee, Fisheries, Wildlife and Water
Subcommittee on April 5, 2004. Much of the CWD interest in the 109th Congress
has been centered around the funding levels in USDA’s annual appropriation
measures.
SNIP...
http://ncseonline.org/NLE/CRSreports/06Oct/RL32199.pdf
IMPORT AND EXPORT
http://www.usaha.org/committees/reports/2006/report-ie-2006.pdf
Brain disease stalks Alberta deer Bigger populations make wasting more likely to spread in threat to Alberta's $100M-a-year hunting industry Hanneke Brooymans, The Edmonton Journal Published: Monday, January 15, 2007 WAINWRIGHT -- As the helicopter turns a corkscrew in the sky to get a better look, panicked deer bound away, bashing through brush and ice-glazed snow drifts.
Wildlife technician Traci Morgan peers out her window behind the pilot as the chopper swirls downward on this survey flight. She presses the button of her mike: "Whitetails -- two does, four fawns," she says, marking them down on her clipboard.
"I can't believe the number of critters out there," says Lyle Fullerton, another staffer with Alberta Sustainable Resource Development.
University of Alberta student Chris Garrett, left, and Gary Hornbeck, a consultant with Wildlife and Company, help in the battle against chronic wasting disease by attaching collars to wild Alberta deer. By day's end, Morgan tallies 939 deer and moose in a 55-square-kilometre area. That's surprisingly high, given that fish and wildlife officers had conducted a cull here in March 2005 to battle the spread of chronic wasting disease.
"We've probably never had as many deer in the province as we do now," Fullerton says later.
The denser the population, the more likely that chronic wasting disease will spread.
The brain-wasting disease has been found in wild deer in 11 American states and two provinces, first in Saskatchewan and then in Alberta in 2005. No jurisdiction has been able to halt its spread.
It's a prion disease like bovine spongiform encephalopathy -- mad cow disease -- and it is always fatal. It isn't known to infect humans, but the World Health Organization nevertheless has cautioned people not to eat the meat of affected animals.
If the disease spreads further, it could scare off recreational hunters from Alberta and out of province -- and each group spends about $50 million a year, said Vic Adamowicz, a University of Alberta professor of rural economy.
Scientists don't know yet what would happen to wild deer populations if the disease went unchecked. In an area of Colorado where up to 30 per cent of the deer are infected, scientists say it's plausible the population will die out within 35 years.
Alberta's already-threatened woodland caribou are another cause for concern. Could they catch the disease? No one knows for sure.
With test results pouring in from the current hunting season in Alberta, the number of wild deer testing positive for chronic wasting disease has crept up to 17 since the first case was found in late 2005. The most recent positive was confirmed Jan. 2.
Even more worrisome is where the cases are turning up.
Until now, the infected mule deer and white-tailed deer have been found along the Alberta/Saskatchewan border. One outbreak appeared around Empress and the South Saskatchewan River valley. The second "spark," as some biologists call it, is further north, around Chauvin.
Since the first Alberta case staggered into a farmyard in September 2005, a small rash of cases has spread further into the province.
One of the most recent was found near Edgerton, about 30 kilometres from the Saskatchewan border.
"That's the furthest into Alberta that a positive deer has been found," said Fullerton, an information officer with Sustainable Resource Development and a past executive director of the Alberta Fish and Game Association.
http://www.canada.com/edmontonjournal/news/story.html?id=665c8db6-093c-45ef-a4c7-a692d25098de
Hanneke Brooymans, The Edmonton Journal
Published: Monday, January 15, 2007 "That deer had the potential to infect other deer when it was living -- how long did it have the disease?"
The Edgerton case was perilously close to CFB Wainwright, which has an ecological reserve attached to its southern edge. The deer density there is extremely high, and the logistics of a cull would be nightmarish.
Further south, at the other outbreak area, the rash of positive cases follows upstream along the South Saskatchewan River valley. If the last case had travelled another 20 kilometres down the valley, it would have entered CFB Suffield. Another nightmare.
Fullerton loves to hunt and stocks his freezer with venison for the winter, but the growing deer population is no boon because of the risk of further spreading the wasting disease. "So what we're trying to do is get on it now, before it spreads out."
For now, that means deer surveys by helicopter at a cost of up to $35,000 per wildlife management unit, putting collars on deer and conducting genetic studies.
By analyzing the genetic differences among deer samples, David Coltman and his associates at the University of Alberta will try to predict where the disease will spread.
If two deer populations have similar genetic profiles, it means they're mixing and the disease is likely to spread between them, although no one knows exactly how it passes from one deer to the next.
The researchers hope to create a map of Alberta shaded in categories of high, medium and low risk.
The genetic studies will also show if pockets of deer populations are resistant to the disease. Although these animals do eventually get infected, they usually take longer to succumb. This information would be worked into the predictive model.
Chris Garrett adds to the projection work by attaching tracking collars to deer. The U of A master's student and his colleagues run traps to catch the deer.
Each 70-kilogram animal is wrestled to the ground and blindfolded for calming before the team hobbles its legs and takes a tissue sample by punching a hole in the ear for a tag. After looking at its teeth to determine the animal's age, they release it.
All of these studies will help Alberta Sustainable Resource Development develop a plan to try to stop the disease from spreading.
But the ministry isn't idle while waiting for the results. It conducts culls in a 10-kilometre radius around an area after a deer tests positive, and it thins herds in select locations.
From September 2005 to March 2006, wildlife officers shot 1,850 deer.
The department has also eased hunting restrictions along the Saskatchewan border to encourage recreational hunters to kill more deer. Hunters are required to turn in deer heads for testing in five wildlife management units in this area.
So far, 2,831 heads have been turned in this season. Four have tested positive.
Ninety-five heads are left to test, and more may arrive -- hunting season closes today for landowners in the areas where chronic wasting disease has been found.
Fullerton said the deer survey results, the positive tests and any data the U of A scientists can provide will be gathered to formulate the next plan of attack in the battle to contain chronic wasting disease.
http://www.canada.com/edmontonjournal/news/story.html?id=665c8db6-093c-45ef-a4c7-a692d25098de&p=2
Michael Samuel applauds the province's aggressive approach.
"If they can prevent the disease from getting into Alberta, that's the best thing," says Samuel, assistant unit leader with the U.S. Geological Survey co-operative wildlife research unit in Wisconsin. "Once it gets there, it's very difficult and costly to control."
But he doesn't know if the aggressive tactics will succeed. "I think nobody can promise that's going to work because no one's been successful yet."
hbrooymans@thejournal.canwest.com
http://www.canada.com/edmontonjournal/news/story.html?id=665c8db6-093c-45ef-a4c7-a692d25098de&p=3
WEB EXTRA: Game ranch near Swan River quarantined
Thu Jan 11 2007
A game ranch about 30 kilometres from Swan River is under quarantine by the Canadian Food Inspection Agency, after elk in a Saskatchewan ranch tested positive for chronic wasting disease.
"There's been no confirmed case (in Manitoba)," said Wayne Lees, chief veterinary officer for the province, on Wednesday.
Lees could not comment on the exact location of the Manitoban game ranch under quarantine.
The neurological disease is transmissable among deer and elk, and can cause small lesions in brains of infected animals. For infected creatures, the disease can lead to loss of bodily functions, abnormal behaviour and death.
"As part of any of these normal investigations, (inspectors) follow animals that travel in and out of the (elk) herd, and that's where the Manitoba connection comes in," said Lees.
"The investigation might take a number of weeks."
Lees said if any animals are found to have moved from the Saskatchewan ranch where animals tested positive for CWD, officials must conduct a 'trace-out' to ascertain the health of animals which may have come in contact with them.
Chronic wasting disease can be slow to develop, said Lees, and can take years for animals to show signs of illness.
"Animals that have been bought or sold from (the Saskatchewan) farm...(inspectors) determine the identity of those animals, and what's happened since," said Lees. "These investigations have to go back a number of years...and that's the reason these tracebacks can take some time. Several years of animal movements have to be checked out."
http://www.winnipegfreepress.com/subscriber/local/story/3838936p-4441931c.html
Revised: January 2007
Purpose:
To support the development of the game farm industry by delivering licensing and regulatory programs for domestic game farms.
Background:
All domestic farm operators are licensed and regulated according to The Domestic Game Farm Animal Regulations under The Animal Products Act. The act and regulations provide a framework for the Saskatchewan domestic game farm industry.
Impact:
Saskatchewan currently has 572 licensed domestic game farms with approximately 30,500 elk, 1,350 fallow deer, 7,400 white-tailed deer and 300 animals of other species being farmed. All domestic game farms are enrolled in Saskatchewan's Cervid Chronic Wasting Disease (CWD) Surveillance Program, with the additional option of enrolling in the Canadian CWD Voluntary Herd Certification Program.
Licensed Domestic Game Farm Operators
Contact:
To discuss Game Farm licensing: Rusty Hawryluk Room 202 - 3085 Albert Street Regina, SK S4S 0B1 Phone: 787-4682 Fax: 787-1315 Email: RHawryluk@agr.gov.sk.ca
To discuss tagging options or order unique identification tags for game farm species other than elk: Game Farm Statistics Clerk or Rusty Hawryluk Room 202 - 3085 Albert Street Regina, SK S4S 0B1 Phone: 787-7053 (Game Farm Statistics Clerk) Phone: 787-4606 (Game Farm Statistics Clerk) Phone: 787-4682 (Rusty Hawryluk) Fax: 787-1315
To obtain import or export permits: Crystal Lozinski Room 202 - 3085 Albert Street Regina, SK S4S 0B1 Phone: 787-6469 Fax: 787-1315 Email: CLozinski@agr.gov.sk.ca
To obtain export certificates: Rusty Hawryluk Room 202 - 3085 Albert Street Regina, SK S4S 0B1 Phone: 787-4682 Fax: 787-1315 Email: RHawryluk@agr.gov.sk.ca
To discuss tagging options or order unique identification tags for elk only: Saskatchewan Elk Breeders Association (SEBA) 381 Parkview Road Yorkton, SK S3N 2L4 Phone: 782-6500 (Maria Bartok) Fax: 782-6501 Email: seba@sasktel.net or maria@elkbreeders.sk.ca Regulatory related inquiries or concerns: Murray Phipps Provincial Game Farm Investigator 3830 Thatcher Avenue Saskatoon, SK S7K 2H6 Phone: 933-6138 Fax: 933-5715 Email: MPhipps@agr.gov.sk.ca For other information on game farming and related industries: Sherri Dobbs (development) Provincial Livestock Development Specialist Room 226 - 3085 Albert Street Regina, SK S4S 0B1 Phone: 787-4657 Fax: 787-9297 Email: SDobbs@agr.gov.sk.ca Kevin Augustine Provincial Game Farm Investigator 800 Central Avenue, Box 3003 Prince Albert, SK S6V 6G1 Phone: 953-2729 Fax: 953-2440 Email: KAugustine@agr.gov.sk.ca Renee Chartier Provincial Game Farm Inspector 3085 Albert Street Regina, SK S4S 0B1 Phone: 787-0514 Fax: 787-1315 Email: RChartier@agr.gov.sk.ca Murray Feist (nutrition & technology transfer) Feed Industry Development Specialist Agriculture Knowledge Centre 45 Thatcher Avenue Moose Jaw, SK S6J 1L8 Phone: 694-3492 Fax: 694-3938 Email: MFeist@agr.gov.sk.ca Dick Johnson Provincial Manager, Inspections & Investigations 3830 Thatcher Avenue Saskatoon, SK S7K 2H6 Phone: 933-6191 Fax: 933-5715 Email: DJohnson@agr.gov.sk.ca Saskatchewan's Cervid Chronic Wasting Disease Surveillance Program Applications, Certificates & Inventory Records: Phone: 787-4264 (Mary Jane Laville) Physical Inventory & Herd Appointments by District: (see map)
1 Renee Chartier 787-0514 2 Renee Chartier 787-0514 3 Dave Augustine 778-8312 4 Bob Solomon 786-5712 5 Murray Phipps 933-6138 6 Kevin Augustine 953-2729 7 Mike Lessard 446-7404 8 Danny L'Heureux 236-5456
Collection & Submission of Samples: Phone: 787-6069 (Dr. LeeAnn Forsythe)
Notification of Discovery of Deaths: Phone: 787-6469 (Animal Health Unit)
Game Farm Inspection Districts:
http://www.agr.gov.sk.ca/docs/programs_services/GF_Inspect_Program.asp
From: TSS (216-119-163-189.ipset45.wt.net) Subject: CWD aka MAD DEER/ELK TO HUMANS ??? Date: September 30, 2002 at 7:06 am PST
From: "Belay, Ermias" To: Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias" Sent: Monday, September 30, 2002 9:22 AM Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Dear Sir/Madam, In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.
That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: 404-639-3091). Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated.
Ermias Belay, M.D. Centers for Disease Control and Prevention
-----Original Message----- From: Sent: Sunday, September 29, 2002 10:15 AM To: rr26k@nih.gov; rrace@niaid.nih.gov; ebb8@CDC.GOV Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Sunday, November 10, 2002 6:26 PM ......snip........end..............TSS
also,
A. Aguzzi - Chronic Wasting Disease (CWD) also needs to be addressed. Most serious because of rapid horizontal spread and higher prevalence than BSE in UK, up to 15% in some populations. Also may be a risk to humans - evidence that it is not dangerous to humans is thin.
http://www.tseandfoodsafety.org/activities/bse_conference_basel_april_02/2summary_of_conference.htm
Chronic Wasting Disease and Potential Transmission to Humans Ermias D. Belay,* Ryan A. Maddox,* Elizabeth S. Williams,† Michael W. Miller,‡ Pierluigi Gambetti,§ and Lawrence B. Schonberger* *Centers for Disease Control and Prevention, Atlanta, Georgia, USA; †University of Wyoming, Laramie, Wyoming, USA; ‡Colorado Division of Wildlife, Fort Collins, Colorado, USA; and §Case Western Reserve University, Cleveland, Ohio, USA
Suggested citation for this article: Belay ED, Maddox RA, Williams ES, Miller MW, Gambetti P, Schonberger LB. Chronic wasting disease and potential transmission to humans. Emerg Infect Dis [serial on the Internet]. 2004 Jun [date cited]. Available from: http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm
--------------------------------------------------------------------------------
Chronic wasting disease (CWD) of deer and elk is endemic in a tri-corner area of Colorado, Wyoming, and Nebraska, and new foci of CWD have been detected in other parts of the United States. Although detection in some areas may be related to increased surveillance, introduction of CWD due to translocation or natural migration of animals may account for some new foci of infection. Increasing spread of CWD has raised concerns about the potential for increasing human exposure to the CWD agent. The foodborne transmission of bovine spongiform encephalopathy to humans indicates that the species barrier may not completely protect humans from animal prion diseases. Conversion of human prion protein by CWD-associated prions has been demonstrated in an in vitro cell-free experiment, but limited investigations have not identified strong evidence for CWD transmission to humans. More epidemiologic and laboratory studies are needed to monitor the possibility of such transmissions.
snip...full text ;
http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm
Volume 12, Number 10–October 2006 Research Human Prion Disease and Relative Risk Associated with Chronic Wasting Disease Samantha MaWhinney,* W. John Pape,† Jeri E. Forster,* C. Alan Anderson,‡§ Patrick Bosque,‡¶ and Michael W. Miller# *University of Colorado at Denver and Health Sciences Center, Denver, Colorado, USA; †Colorado Department of Public Health and Environment, Denver, Colorado, USA; ‡University of Colorado School of Medicine, Denver, Colorado, USA; §Denver Veteran's Affairs Medical Center, Denver, Colorado, USA; ¶Denver Health Medical Center, Denver, Colorado, USA; and #Colorado Division of Wildlife, Fort Collins, Colorado, USA
Suggested citation for this article
The transmission of the prion disease bovine spongiform encephalopathy (BSE) to humans raises concern about chronic wasting disease (CWD), a prion disease of deer and elk. In 7 Colorado counties with high CWD prevalence, 75% of state hunting licenses are issued locally, which suggests that residents consume most regionally harvested game. We used Colorado death certificate data from 1979 through 2001 to evaluate rates of death from the human prion disease Creutzfeldt-Jakob disease (CJD). The relative risk (RR) of CJD for CWD-endemic county residents was not significantly increased (RR 0.81, 95% confidence interval [CI] 0.40–1.63), and the rate of CJD did not increase over time (5-year RR 0.92, 95% CI 0.73–1.16). In Colorado, human prion disease resulting from CWD exposure is rare or nonexistent. However, given uncertainties about the incubation period, exposure, and clinical presentation, the possibility that the CWD agent might cause human disease cannot be eliminated.
snip... full text ;
http://0-www.cdc.gov.mill1.sjlibrary.org/ncidod/EID/vol12no10/06-0019.htm
3:00 Afternoon Refreshment Break, Poster and Exhibit Viewing in the Exhibit Hall
3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse
Models Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western Reserve University
Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain discovered recently in Italy, and similar or different atypical BSE cases were also reported in other countries. The infectivity and phenotypes of these atypical BSE strains in humans are unknown. In collaboration with Pierluigi Gambetti, as well as Maria Caramelli and her co-workers, we have inoculated transgenic mice expressing human prion protein with brain homogenates from BASE or BSE infected cattle. Our data shows that about half of the BASE-inoculated mice became infected with an average incubation time of about 19 months; in contrast, none of the BSE-inoculated mice appear to be infected after more than 2 years. ***These results indicate that BASE is transmissible to humans and suggest that BASE is more virulent than classical BSE in humans.
6:30 Close of Day One
http://www.healthtech.com/2007/tse/day1.asp
SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype of 'UNKNOWN' strain growing. ...
http://www.cjdsurveillance.com/resources-casereport.html
There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.
He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf
JOURNAL OF NEUROLOGY
MARCH 26, 2003
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States
Email Terry S. Singeltary:
flounder@wt.net
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to
comment on the CDC's attempts to monitor the occurrence of emerging
forms of CJD. Asante, Collinge et al [1] have reported that BSE
transmission to the 129-methionine genotype can lead to an alternate
phenotype that is indistinguishable from type 2 PrPSc, the commonest
sporadic CJD. However, CJD and all human TSEs are not reportable
nationally. CJD and all human TSEs must be made reportable in every
state and internationally. I hope that the CDC does not continue to
expect us to still believe that the 85%+ of all CJD cases which are
sporadic are all spontaneous, without route/source. We have many TSEs in
the USA in both animal and man. CWD in deer/elk is spreading rapidly and
CWD does transmit to mink, ferret, cattle, and squirrel monkey by
intracerebral inoculation. With the known incubation periods in other
TSEs, oral transmission studies of CWD may take much longer. Every
victim/family of CJD/TSEs should be asked about route and source of this
agent. To prolong this will only spread the agent and needlessly expose
others. In light of the findings of Asante and Collinge et al, there
should be drastic measures to safeguard the medical and surgical arena
from sporadic CJDs and all human TSEs. I only ponder how many sporadic
CJDs in the USA are type 2 PrPSc?
http://www.neurology.org/cgi/eletters/60/2/176#535
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Singeltary, Sr et al. JAMA.2001; 285: 733-734.
http://jama.ama-assn.org/
TSS
=====================================================
MY greatest fear is of iCJD via CWD tainted humans in the surgical and medical arena x 1, 2, 3, 4, 5, passages etc. from someone that has been exposed to the cwd agent, but not gone clinical, and may never go clinical. WE must realize that the USA has the most documented TSE in different species than any other country in the world. all of which has been rendered and fed back to animals for human and animal consumption. the incubation period is what is fooling everyone. ...tss
Prion protein in cardiac
elaphus nelsoni)
Odocoileus virginianus)
wasting disease
snip...
infectivity in skeletal muscle of CWD-infected mule deer (Angers et al.,
2006) has raised the level of concern on the issue of potential
human health risks that might be encountered by consuming
prion-containing meat.
Prions in Skeletal Muscles of Deer with Chronic Wasting Disease
Rachel C. Angers,1* Shawn R. Browning,1*† Tanya S. Seward,2 Christina J. Sigurdson,4‡ Michael W. Miller,5 Edward A. Hoover,4 Glenn C. Telling1,2,3§
1Department of Microbiology, Immunology and Molecular Genetics, 2Sanders Brown Center on Aging, 3Department of Neurology, University of Kentucky, Lexington, KY 40536, USA. 4Department of Microbiology, Immunology and Pathology, Colorado State University, Fort Collins, CO 80523, USA. 5Colorado Division of Wildlife, Wildlife Research Center, Fort Collins, CO 80526, USA.
*These authors contributed equally to this work.
†Present address: Department of Infectology, Scripps Research Institute, 5353 Parkside Drive, RF-2, Jupiter, Florida, 33458, USA.
‡Present address: Institute of Neuropathology, University of Zurich, Schmelzbergstrasse 12, 8091 Zurich, Switzerland.
§To whom correspondence should be addressed: E-mail: gtell2@uky.edu
Prions are transmissible proteinaceous agents of mammals that cause fatal neurodegenerative diseases of the central nervous system (CNS). The presence of infectivity in skeletal muscle of experimentally infected mice raised the possibility that dietary exposure to prions might occur through meat consumption (1). Chronic wasting disease (CWD), an enigmatic and contagious prion disease of North American cervids, is of particular concern. The emergence of CWD in an increasingly wide geographic area and the interspecies transmission of bovine spongiform encephalopathy (BSE) to humans as variant Creutzfeldt Jakob disease (vCJD) have raised concerns about zoonotic transmission of CWD.
To test whether skeletal muscle of diseased cervids contained prion infectivity, Tg(CerPrP)1536 mice (2) expressing cervid prion protein (CerPrP), were inoculated intracerebrally with extracts prepared from the semitendinosus/semimembranosus muscle group of CWD-affected mule deer or from CWD-negative deer. The availability of CNS materials also afforded direct comparisons of prion infectivity in skeletal muscle and brain. All skeletal muscle extracts from CWD-affected deer induced progressive neurological dysfunction in Tg(CerPrP)1536 mice with mean incubation times ranging between 360 and ~490 d, whereas the incubation times of prions from the CNS ranged from ~230 to 280 d (Table 1). For each inoculation group, the diagnosis of prion disease was confirmed by the presence of PrPSc in the brains of multiple infected Tg(CerPrP)1536 mice (see supporting online material for examples). In contrast, skeletal muscle and brain material from CWD-negative deer failed to induce disease in Tg(CerPrP)1536 mice (Table 1) and PrPSc was not detected in the brains of sacrificed asymptomatic mice as late as 523 d after inoculation (supporting online material).
Our results show that skeletal muscle as well as CNS tissue of deer with CWD contains infectious prions. Similar analyses of skeletal muscle BSE-affected cattle did not reveal high levels of prion infectivity (3). It will be important to assess the cellular location of PrPSc in muscle. Notably, while PrPSc has been detected in muscles of scrapie-affected sheep (4), previous studies failed to detect PrPSc by immunohistochemical analysis of skeletal muscle from deer with natural or experimental CWD (5, 6). Since the time of disease onset is inversely proportional to prion dose (7), the longer incubation times of prions from skeletal muscle extracts compared to matched brain samples indicated that prion titers were lower in muscle than in CNS where infectivity titers are known to reach high levels. Although possible effects of CWD strains or strain mixtures on these incubation times cannot be excluded, the variable 360 to ~490 d incubation times suggested a range of prion titers in skeletal muscles of CWD-affected deer. Muscle prion titers at the high end of the range produced the fastest incubation times that were ~30% longer than the incubation times of prions from the CNS of the same animal. Since all mice in each inoculation group developed disease, prion titers in muscle samples producing the longest incubation times were higher than the end point of the bioassay, defined as the infectious dose at which half the inoculated mice develop disease. Studies are in progress to accurately assess prion titers.
While the risk of exposure to CWD infectivity following consumption of prions in muscle is mitigated by relatively inefficient prion transmission via the oral route (8), these
/ www.sciencexpress.org / 26 January 2006 / Page 1 / 10.1126/science.1122864
results show that semitendinosus/semimembranosus muscle, which is likely to be consumed by humans, is a significant source of prion infectivity. Humans consuming or handling meat from CWD-infected deer are therefore at risk to prion exposure.
CWD
Human health implications
14. Epidemiological data on possible CWD infection of humans are
very limited. The possibility that clinical symptoms of CWD in
humans differ from those of Creutzfeldt-Jakob Disease (CJD)
cannot be excluded. There is no significant difference between the
prevalence of CJD in CWD endemic areas and other areas of the
world. However, because CJD surveillance in the USA is relatively
recent, not all CJD cases may have been identified. Additionally,
detection of a small increase in prevalence of such a rare disease
is very difficult. Investigation of six cases of prion disease in young
people ((> 54 years of age) diagnosed
with sporadic CJD, no link with consumption of venison from CWD
endemic areas was found. No causal link was found in an
investigation of three men with neurological illnesses who were
known to partake in “wild game feasts”. Only one of these subjects
was found to have a prion disease and this was also
indistinguishable from sporadic CJD.
snip...
http://www.seac.gov.uk/statements/statement180105.pdf
10) HUMAN HEALTH CONCERNS
217. To date there are no known cases of human prion disease attributable to CWD
transmitted to humans (Belay et al., 2004). While limited epidemiological investigations to
date have not shown any links between CWD and humans with spongiform encephalopathies
CWD Review / Dr Debra Bourne / October 2004 / For SEAC / Page 45 of 66
this data must be considered along with a caveat: “because CWD is a relatively new TSE, it is
unlikely that enough people have consumed enough CWD-affected cervids to result in a
clinically or pathologically recognizable disease attributable to CWD, especially considering
the very long incubation periods characteristic of TSE diseases.” (Race et al., 2002)
Epidemiological investigations
218. Epidemiological investigations have failed to show any links between cases of prion
disease in unusually young people or in hunters in the USA and CWD (CDC, 2003). Two
major epidemiological investigations have been carried out, one on cases of CJD in unusually
young individuals in the USA, the second on a group of men from Wisconsin who developed
neurological diseases.
219. The first study (Belay et al., 2001) focused on three individuals, two 28 years of age
and the third 30 years old, diagnosed with CJD in the USA between 1st January 1997 and 31st
May 2000, and without any established risk factors for CJD (family history, receipt of human
growth hormone, receipt of grafts of dura mater or cornea, or previous neurological surgery)
and concluded that there was no strong evidence for a causal link with CWD. None of the
individuals had travelled to Europe (therefore a link with BSE was unlikely). Two of the
individuals were hunters who regularly consumed game meat while the third (case 1) had, as a
young child, regularly consumed venison from animals hunted by family members and on two
occasions from a family friend. Two of the individuals (cases 1 and 2) had undergone tonsillar
surgery as children; the third had never received any surgical treatment. One individual (case
1) had eaten venison mainly hunted in Maine, occasionally hunted in New Jersey, and, on two
occasions at about six years old, elk meat which had probably been harvested in Wyoming.
The second person (case 2) had hunted cervids mainly in Utah, but had harvested an elk in
southwestern Wyoming on one occasion (less than three years before onset of clinical signs)
and had hunted in British Columbia on one occasion nine years before onset of illness. The
third person (case 3) had hunted close to home and never in Colorado or Wyoming although
the plant where he took his carcasses for processing did also process some elk from Colorado
each year. The clinical signs, duration of illness and histopathological findings for the three
individuals showed no obvious similarities to one another. One individual was
methionine/methionine homozygous at codon 129 of the PRNP (case 1), one was
homozygous for valine at this gene (case 2) and the third (case 3) was heterozygous
methionine/valine. Immunohistochemistry revealed strong staining with a “synaptic” pattern
in the first individual and weak staining with a “synaptic” pattern in the second case; in case
3, based on a brain biopsy sample obtained at an early point in the illness, staining was
questionable and possibly showed a synaptic pattern. Cases 2 and 3 showed a “Type 1”
immunoblot pattern, this test had not been carried out for case 1. It was noted that none of
these three individuals had a definite history of consumption of venison from the geographical
areas in which CWD was known to be endemic in Colorado and Wyoming, and no CWD had
been identified in 299 deer and sampled from the area in which most of the venison consumed
by patient 1 had originated, nor in 404 deer and 196 elk sampled from the area in which most
of the venison consumed by patient 2 had originated, nor in 138 deer samples from the area in
which most of the venison consumed by patient 1 had originated. Additionally, there was no
homogeneity in phenotypic expression of the disease and all three possible options for coding
at codon 129 of the PRNP gene were represented. Since a survey had indicated that
approximately 40% of blood donors in the USA consumed venison from wild cervids, it was
considered most likely that coincidence explained why three of the four young (30 years old
or younger) individuals with sporadic CJD reported in the USA after March 1996 had
consumed such meat (Belay et al., 2001).
CWD Review / Dr Debra Bourne / October 2004 / For SEAC / Page 46 of 66
220. The second major epidemiological investigation centred around three men from
Wisconsin and Minnesota who had died from degenerative neurological illnesses and who
had participated in “wild game feasts” in northern Wisconsin. Full investigation including
examination of fixed brain tissue confirmed CJD in only one of the three individuals. Wild
game eaten during the feasts was harvested mainly in Wisconsin but also in areas of
Colorado, Wyoming and Montana; CWD was not known to be endemic in the areas where the
game was hunted at the time that the game was harvested. Further investigations of other
possible attendees of the feasts revealed 34 participants, all male, of whom a total of seven
were deceased, including the three individuals in the initial investigation. Causes of death in
the other four deceased individuals were not attributed to nor associated with any
degenerative neurological disorder and no signs or symptoms associated with a degenerative
neurological disorder were noted for any of the remaining living participants of the feasts. It
was noted that only one case of CJD had occurred among known participants at the feasts,
that this case was consistent with the commonest form of sporadic CJD, that this individual
had only participated in one feast and that it was unlikely that he had consumed CWDinfected
venison at the feasts “because venison and other game from outside Wisconsin that
was served at these feasts did not originate from known CWD-endemic areas.” Limitations of
the investigations were noted to include reliance on recall of events from up to 25 years
previously and the fact that not all participants in the feasts could be contacted and
interviewed. However, those who were interviewed agreed in their recall of events (CDC,
2003).
221. It is important to recognise that the limited epidemiological investigations that have
been carried out are not able to rule out the possibility that CWD might play a role in causing
illness in humans (CDC, 2003).
222. Three further cases of prion disease in young humans in the USA have been
investigated for possible links to CWD (Belay et al., 2004). The first case was a 25-year-old
man who died in 2001 after about 22 months of illness. Gerstmann-Sträussler-Scheinker
syndrome (GSS) was diagnosed by analysis of the prion gene, with a P102L mutation
together with valine at codon 129 in the mutant allele. It was noted that the disease had
occurred at an unusually young age, even for GSS, and the possibility that exposure to CWDinfected
venison contributed to early onset of the disease could not be ruled out; the patient’s
grandfather had regularly hunted in southeastern Wyoming, around the known CWD-endemic
area, and had given venison to the patient’s family. Two other cases of prion disease occurred
in individuals of 26 and 28 years of age, from adjacent counties, and with onset of illness only
months apart, therefore an environmental source of infection was investigated. However,
these two individuals were finally diagnosed with different prion diseases: sporadic CJD in
one case and GSS in the other, indicating that a common cause was unlikely. In the first case
CJD was confirmed from autopsy samples (by histopathology, immunohistochemistry and
immunoblotting); the individual had no history of hunting nor of regular consumption of
venison, and although he may have eaten venison originating from the Upper Peninsula of
Michigan while at college CWD has never been detected in deer from Michigan.
Phenotypically this individual fit the “MM2 sporadic CJD” phenotype described by Parchi et
al. (1999). In the other case post mortem immunohistochemistry revealed prion deposition
which was consistent with GSS and a GSS P102L mutation was detected in a blood sample
from one parent (appropriate samples were not available from the affected patient); this
individual may possibly have eaten venison from Michigan on one occasion at about two
years of age (Belay et al., 2004).
223. A further three cases of CJD in individuals of 54 to 66 years old who were deer and
elk hunters (two individuals) or ate wild-harvested venison (one individual) have been
CWD Review / Dr Debra Bourne / October 2004 / For SEAC / Page 47 of 66
investigated. There was no evidence that any of these individuals had hunted in known CWD
endemic areas; information available indicated hunting or eating venison from Washington
State and Pennsylvania. Two individuals were V/V at codon 129 the third was M/M; they
were considered to fit known subtypes of sporadic CJD (MM1, VV1 and VV2 subtypes as
described by Parchi et al. (1999)). Further investigations were also made on the only two
nonfamilial cases of CJD in individuals with a history of eating venison from the known
CWD-endemic areas. One was reported to have eaten venison from two deer harvested in an
area with endemic CWD, but both deer had been tested and not found to be CWD-positive;
the patient’s illness was consistent with the CJD subtype MM1. The other individual grew up
in a CWD-endemic area and ate locally-harvested venison; her disease fit the MM1 CJD
phenotype and no atypical neurological features were noted (Belay et al., 2004).
224. Additional epidemiological notes are that the incidence and age distribution of CJD in
Colorado and Wyoming, where CWD is thought to have been endemic for decades, are
similar to those found in other areas of the USA. In Wyoming, seven cases of CJD have been
reported between 1979 and 2000 with an average annual age-adjusted CJD death rate of 0.8
per million and no cases reported in humans less than 55 years old. In Colorado in the same
period 67 cases of CJD have been reported, with an average annual age-adjusted CJD death
rate of 1.2 per million (Belay et al., 2004).
225. In summary, there is no evidence of an increase in incidence of CJD in Colorado and
Wyoming, nor have epidemiological investigations carried out so far found any evidence of a
link between CWD and cases of CJD in persons in the USA (Belay et al., 2001; CDC, 2003;
Belay et al., 2004).
Laboratory studies
226. There is evidence from an in vitro cell-free system that there may be a considerable
“species barrier” reducing the probability that CWD will affect humans. It was shown that
PrPres associated with chronic wasting disease (PrPCWD) from elk, mule deer or white-tailed
deer was able to readily induce substantial conversion of recombinant cervid PrPsen
molecules form any of these three species to the protease-resistant state. In the same system,
CWD-associated PrPres was shown to convert human PrPsen but at a much lower efficiency:
more than 14-fold lower efficiency than inter-cervid conversion reactions and more than
fivefold
lower than conversion of human PrPsen by PrPres from the brains of humans with CJD
(Raymond et al., 2000). While encouraging, interpretation of this study is complicated by the
fact that conversion of human PrPC by PrPBSE and PrPSc from sheep were of similar efficacy,
both being more than 10-fold less efficient compared with corresponding homologous
conversions) and one of these appears to be orally transmissible to humans (BSE) while the
other (scrapie) appears not to be (Raymond et al., 2000). In previous experiments PrPBSE had
showed 10-fold greater conversion efficacy for bovine PrPsen than for human codon 129-M
(methionine) PrPsen and 30-fold greater conversion efficacy than for human codon 129-V
(valine) PrPsen, while ovine PrPSc showed five-fold greater conversion efficacy for ovine
PrPsen than for human 129-M PrPsen and eight-fold greater conversion than for human 129-
V PrPsen (Raymond et al., 1997).
227. Results of recent work in transgenic mice expressing human PrP (see paragraph 71), in
which transmission of CWD from elk by intracerebral inoculation failed, was considered to
“strongly suggest” a species barrier to transmission of elk CWD to humans (Kong et al.,
2004).
CWD Review / Dr Debra Bourne / October 2004 / For SEAC / Page 48 of 66
Potential risk from consuming cervid products
Velvet antler
228. Limited studies to date indicate risk from this product may be very low. No CWDspecific
PrP accumulation was detected in a sample of velvet from an elk stag which
developed clinical CWD about three months later; there were severe brain lesions and
extensive CWD-specific PrP staining in both the brain and peripheral lymphoid tissue of the
stag (Kahn et al., 2004).
Consumption of venison and other parts of the animal
229. PrPCWD has not been detected in muscle tissue from infected cervids (Spraker et al.,
2002c). However, it has been recommended by the World Health Organisation that no parts or
products of any animal know to be CWD-positive should be consumed (WHO, 2000). Public
health authorities in the USA and Canada have indicated agreement with this (Canadian Food
Inspection Agency, 2003; Chronic Wasting Disease Alliance, 2004). It has been suggested
that if a harvested cervid is being tested for CWD, the test results should be awaited before
the meat is eaten (Wisconsin Department of Agriculture, Trade and Consumer Protection,
2002). Authorities in North America have widely advised that (a) tissues likely to contain the
greatest amount of CWD agent in infected cervids, including the brain, spinal cord, lymph
nodes, spleen, tonsils and eyes, should not be consumed from any harvested deer; (b) meat
should be boned out and fat and connective tissue removed (which would also remove lymph
nodes); and (c) hunters should avoid eating meat from deer or elk which look sick or which
test positive for CWD (Buege, 2002; Chronic Wasting Disease Alliance, 2004; Williams et
al., 2002; Wisconsin Department of Agriculture, Trade and Consumer Protection, 2002;
Belay et al., 2004).
Potential risk from handling and processing cervids
230. In order to minimise any potential risk from exposure to the agent of CWD, hunters,
meat processors and taxidermists handling cervid carcasses are advised to wear latex or
rubber gloves when handling or dressing cervids from CWD-endemic areas, to minimise
handling of brain and spinal cord, and to thoroughly wash knives and other implements after
use on deer or elk carcasses (Belay, 2004; Williams et al., 2002). It has been suggested that
the risk of “build-up” of infectious CWD agent in a venison processing plant would be
unlikely (Buege, 2002).
Potential risk from disposal of carcasses and subsequent contamination of
ground/water/air
231. In 2002, a risk analysis was produced on disposal of deer from Wisconsin in
municipal solid landfills. It was noted that it is not known how much infected material a
human (or animal) must consume or be exposed to in order to be infected with CWD. The
report took into account the probable species barrier for transmission to humans (Raymond et
al., 2000). It was noted that the CWD agent is hydrophobic and likely to adhere to organic
materials within a landfill, taking several months to move through the landfill, and that any
infectivity exiting the landfill would be captured in the landfill effluent. If effluent was
transferred to a wastewater plant (rather than recirculated in the landfill) the agent would be
expected to partition with the sludge fraction, which would be diluted greatly with other
solids and mixed with nine inches (22.5 cm) of topsoil, providing “an extremely large dilution
factor.” It was concluded that there was no significant risk to human health from disposing of
deer infected with CWD in properly constructed landfill sites (Olander, 2002).
snip...full text 66 pages ;
http://www.seac.gov.uk/papers/CWD-bournereview.pdf
SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype of 'UNKNOWN' strain growing. ...
http://www.cjdsurveillance.com/resources-casereport.html
HUMAN TSE USA 2005
Animal Prion Diseases Relevant to Humans (unknown types?) Thu Oct 27, 2005 12:05 71.248.128.109
About Human Prion Diseases / Animal Prion Diseases Relevant to Humans
Bovine Spongiform Encephalopathy (BSE) is a prion disease of cattle. Since 1986, when BSE was recognized, over 180,000 cattle in the UK have developed the disease, and approximately one to three million are likely to have been infected with the BSE agent, most of which were slaughtered for human consumption before developing signs of the disease. The origin of the first case of BSE is unknown, but the epidemic was caused by the recycling of processed waste parts of cattle, some of which were infected with the BSE agent and given to other cattle in feed. Control measures have resulted in the consistent decline of the epidemic in the UK since 1992. Infected cattle and feed exported from the UK have resulted in smaller epidemics in other European countries, where control measures were applied later.
Compelling evidence indicates that BSE can be transmitted to humans through the consumption of prion contaminated meat. BSE-infected individuals eventually develop vCJD with an incubation time believed to be on average 10 years. As of November 2004, three cases of BSE have been reported in North America. One had been imported to Canada from the UK, one was grown in Canada, and one discovered in the USA but of Canadian origin. There has been only one case of vCJD reported in the USA, but the patient most likely acquired the disease in the United Kingdom. If current control measures intended to protect public and animal health are well enforced, the cattle epidemic should be largely under control and any remaining risk to humans through beef consumption should be very small. (For more details see Smith et al. British Medical Bulletin, 66: 185. 2003.)
Chronic Wasting Disease (CWD) is a prion disease of elk and deer, both free range and in captivity. CWD is endemic in areas of Colorado, Wyoming, and Nebraska, but new foci of this disease have been detected in Nebraska, South Dakota, New Mexico, Wisconsin, Mississippi Kansas, Oklahoma, Minnesota, Montana, and Canada. Since there are an estimated 22 million elk and deer in the USA and a large number of hunters who consume elk and deer meat, there is the possibility that CWD can be transmitted from elk and deer to humans. As of November 2004, the NPDPSC has examined 26 hunters with a suspected prion disease. However, all of them appeared to have either typical sporadic or familial forms of the disease. The NPDPSC coordinates with the Centers for Disease Control and state health departments to monitor cases from CWD-endemic areas. Furthermore, it is doing experimental research on CWD transmissibility using animal models. (For details see Sigurdson et al. British Medical Bulletin. 66: 199. 2003 and Belay et al. Emerging Infectious Diseases. 10(6): 977. 2004.)
http://www.cjdsurveillance.com/abouthpd-animal.html
Interspecies Transmission of Chronic Wasting Disease Prions to
Squirrel Monkeys (Saimiri sciureus)
Richard F. Marsh,1? Anthony E. Kincaid,2 Richard A. Bessen,3 and Jason C. Bartz4*
Department of Animal Health and Biomedical Sciences, University of Wisconsin, Madison 537061; Department of
Physical Therapy2 and Department of Medical Microbiology and Immunology,4 Creighton University, Omaha,
Nebraska 68178; and Department of Veterinary Molecular Biology, Montana
State University, Bozeman, Montana 597183
Received 3 May 2005/Accepted 10 August 2005
Chronic wasting disease (CWD) is an emerging prion disease of deer and elk. The risk of CWD transmission to humans following exposure to CWD-infected tissues is unknown. To assess the susceptibility of nonhuman primates to CWD, two squirrel monkeys were inoculated with brain tissue from a CWD-infected mule deer. The CWD-inoculated squirrel monkeys developed a progressive neurodegenerative disease and were euthanized at 31 and 34 months postinfection. Brain tissue from the CWD-infected squirrel monkeys contained the abnormal
isoform of the prion protein, PrP-res, and displayed spongiform degeneration. This is the first reported transmission of CWD to primates.
snip...
JOURNAL OF VIROLOGY, Nov. 2005, p. 13794-13796 Vol. 79, No. 21
0022-538X/05/$08.00!0 doi:10.1128/JVI.79.21.13794-13796.2005
Copyright © 2005, American Society for Microbiology. All Rights Reserved.
=============================================
The EMBO Journal, Vol. 19, No. 17 pp. 4425-4430, 2000 © European Molecular Biology Organization
Evidence of a molecular barrier limiting susceptibility of humans, cattle and sheep to chronic wasting disease
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
1NIAID/NIH Rocky Mountain Laboratories, Hamilton, MT 59840, 3USDA/ARS/ADRU, Pullman, WA 99164-7030, 4USDA/ARS/ABADRL, Laramie, WY 82071, 5Colorado Division of Wildlife, Wildlife Research Center, Fort Collins, CO 80526-2097, 6Department of Veterinary Sciences, University of Wyoming, Laramie, WY 82070, USA and 2ID-Lelystad, Institute for Animal Science and Health, Lelystad, The Netherlands 7Corresponding author e-mail: bcaughey@nih.gov Received June 7, 2000; revised July 3, 2000; accepted July 5, 2000.
Abstract
Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy (TSE) of deer and elk, and little is known about its transmissibility to other species. An important factor controlling interspecies TSE susceptibility is prion protein (PrP) homology between the source and recipient species/genotypes. Furthermore, the efficiency with which the protease-resistant PrP (PrP-res) of one species induces the in vitro conversion of the normal PrP (PrP-sen) of another species to the protease-resistant state correlates with the cross-species transmissibility of TSE agents. Here we show that the CWD-associated PrP-res (PrPCWD) of cervids readily induces the conversion of recombinant cervid PrP-sen molecules to the protease-resistant state in accordance with the known transmissibility of CWD between cervids. In contrast, PrPCWD-induced conversions of human and bovine PrP-sen were much less efficient, and conversion of ovine PrP-sen was intermediate. These results demonstrate a barrier at the molecular level that should limit the susceptibility of these non-cervid species to CWD.
snip...
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 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.
snip...
http://www.emboj.org/current.shtm
Subject: Infectious Prions in the Saliva and Blood of Deer with Chronic Wasting Disease
Date: October 5, 2006 at 1:45 pm PST
Infectious Prions in the Saliva
and Blood of Deer with Chronic
Wasting Disease
Candace K. Mathiason,1 Jenny G. Powers,3 Sallie J. Dahmes,4 David A. Osborn,5 Karl V. Miller,5
Robert J. Warren,5 Gary L. Mason,1 Sheila A. Hays,1 Jeanette Hayes-Klug,1 Davis M. Seelig,1
Margaret A. Wild,3 Lisa L. Wolfe,6 Terry R. Spraker,1,2 Michael W. Miller,6 Christina J. Sigurdson,1
Glenn C. Telling,7 Edward A. Hoover1*
A critical concern in the transmission of prion diseases, including chronic wasting disease (CWD) of cervids, is the potential presence of prions in body fluids. To address this issue directly, we exposed cohorts of CWD-nai¨ve deer to saliva, blood, or urine and feces from CWD-positive deer. We found infectious prions capable of transmitting CWD in saliva (by the oral route) and in blood (by transfusion). The results help to explain the facile transmission of CWD among cervids and prompt caution concerning contact with body fluids in prion infections.
SNIP...
Deer cohorts 1 (blood), 2 (saliva), and 3
(urine and feces) were electively euthanized at
18 months pi to permit whole-body examination
for PrPCWD. The greatest scrutiny was directed
toward those tissues previously established
to have highest frequency of PrPCWD deposition
in infected deer and generally regarded
as the most sensitive indicators of infection—
medulla oblongata and other brainstem regions,
tonsil, and retropharyngeal lymph node. We
found unequivocal evidence of PrPCWD in brain
and lymphoid tissue of all six tonsil biopsy–
positive deer in cohorts 1 (blood) and 2 (saliva),
whereas all deer in cohorts 3 and 5 were negative
for PrPCWD in all tissues (Table 2 and
Figs. 1 and 2).
The transmission of CWD by a single blood
transfusion from two symptomatic and one
asymptomatic CWDþ donor is important in at
least three contexts: (i) It reinforces that no tissue
from CWD-infected cervids can be considered
free of prion infectivity; (ii) it poses the
possibility of hematogenous spread of CWD,
such as through insects; and (iii) it provides a
basis for seeking in vitro assays sufficiently
sensitive to demonstrate PrPCWD or alternate
prion protein conformers in blood—one of the
grails of prion biology and epidemiology.
The identification of blood-borne prion
transmission has been sought before with mixed
results (9–11). Bovine spongiform encephalopathy
and scrapie have been transmitted to naBve
sheep through the transfer of 500 ml of blood
or buffy coat white blood cells from infected
sheep (12, 13). In addition, limited but compelling
evidence argues for the transmission of variant
Creutzfeldt-Jakob disease (vCJD) through blood
from asymptomatic donors (14–16). Even in
sporadic CJD, PrPres has been found in periph-
eral organs of some patients (17). The present
work helps establish that prion diseases can be
transmitted through blood.
The presence of infectious CWD prions in
saliva may explain the facile transmission of
CWD. Cervid-to-cervid interactions (SOM text),
especially in high density and captive situations,
would be expected to facilitate salivary crosscontact
(11, 18, 19). Salivary dissemination of
prions may not be limited to CWD. Proteaseresistant
prion protein has been demonstrated in
the oral mucosa, taste buds, lingual epithelium,
vomeronasal organ, and olfactory mucosa of
hamsters infected with transmissible mink
encephalopathy (19) and ferrets infected with
CWD (20). Although no instance of CWD
transmission to humans has been detected, the
present results emphasize the prudence of using
impervious gloves during contact with saliva or
blood of cervids that may be CWD-infected.
Environmental contamination by excreta
from infected cervids has traditionally seemed
the most plausible explanation for the dissemination
of CWD (21). However, we could not
detect PrPCWD in cohort 3 deer inoculated repeatedly
with urine and feces from CWDþ deer and examined up to 18 months pi (Table 2).
There are several reasons to view this negative
finding cautiously, including small sample size,
elective preclinical termination, and potential
variation in individual susceptibility that may
be associated with the 96 G/S polymorphism in
the PRNP gene (7, 22). Although no genotype
of white-tailed deer is resistant to CWD infection,
PRNP genotypes S/S or G/S at codon 96
appear to have reduced susceptibility manifest
by longer survival (7). Both deer in cohort 3
(urine and feces) were subsequently shown to
be of the PRNP 96 G/S genotype. Thus, it is
possible, although we think unlikely, that these
deer had a prolonged incubation period (918
months pi) before the amplification of PrPCWD
became detectable in tissues. Recent studies
have shown that PrPres is poorly preserved
after incubation with intestinal or fecal content
(23, 24). Further research using cervid and surrogate
cervid PrP transgenic mice (25) are indicated
to continue to address the presence of
infectious CWD prions in excreta of CWDþ deer and to
provide a more substantial basis for
reconsideration of the assumption that excreta
are the chief vehicle for CWDdissemination and
transmission.
The results reported here provide a plausible
basis for the efficient transmission of CWD in
nature. We demonstrate that blood and saliva in
particular are able to transmit CWD to naBve deer
and produce incubation periods consistent with
those observed in naturally acquired infections
(3, 26). The time from exposure to first detection
of PrPCWD by tonsil biopsy was variable—as
short as 3 months but as long as 18 months (likely
underestimates due to sampling frequency).
The results also reinforce a cautious view of the
exposure risk presented by body fluids, excreta,
and all tissues from CWDþ cervids. ...
SNIP...END
http://www.sciencemag.org/cgi/content/abstract/314/5796/133
http://www.sciencemag.org/
While it is early in the investigation of these events, New York officials have documented several facts: (1) the owner of the index captive herd was also a wildlife rehabilitator, including white-tailed deer, and that wild deer were commingled with his captive herd and subsequently released back to the wild; (2) he was also a taxidermist, possibly having handled animals that originated in CWD-endemic regions in other states; and (3) he had recently sold captive stock to additional cervid farms within New York (the state has quarantined these herds).
http://www.nwhc.usgs.gov/publications/wildlife_health_bulletins/WHB_05_01.jsp
What Every Taxidermist Should Know
About Chronic Wasting Disease (CWD)
snip...
What actions have been taken to prevent the spread of CWD?
The movement of high-risk carcass parts (brain, spinal cord, lymph tissues) is a potential avenue through which CWD could be spread from infected areas. Investigations in New York indicate that the infection could have been spread by a taxidermist who accepted specimens from CWD-positive states, allowed rehabilitated fawns access to the taxidermy workshop and spread potentially infectious curing salt waste as a fence line weed killer on his deer
Updated 8/24/06 1 of 1
farm. Several states, including Pennsylvania, have developed regulations to prohibit the importation of high-risk carcass parts from CWD endemic states. As of April 1, 2006 Pennsylvania’s importation ban prohibits the importation of high-risk carcass parts from Colorado, Illinois, Kansas, Nebraska, New Mexico, New York (CWD containment area only), South Dakota, Utah, West Virginia (Hampshire County only), Wisconsin, Wyoming and the Canadian provinces of Alberta and Saskatchewan. Pennsylvanians hunting in CWD-positive areas should get their animals tested and should leave high-risk carcass parts in the area where the animal was hunted.
snip...
http://www.pgc.state.pa.us/pgc/lib/pgc/taxidermists_cwd.pdf
CWD AND ENVIRONMENTAL FACTORS i.e. saliva, fecal shedding and fecal-oral transmission is likely
http://p079.ezboard.com/fwolftracksproductionsfrm2.showMessage?topicID=592.topic
From: TSS (216-119-163-189.ipset45.wt.net) Subject: CWD aka MAD DEER/ELK TO HUMANS ??? Date: September 30, 2002 at 7:06 am PST
From: "Belay, Ermias" To: Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias" Sent: Monday, September 30, 2002 9:22 AM Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Dear Sir/Madam, In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.
That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: 404-639-3091). Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated.
Ermias Belay, M.D. Centers for Disease Control and Prevention
-----Original Message----- From: Sent: Sunday, September 29, 2002 10:15 AM To: rr26k@nih.gov; rrace@niaid.nih.gov; ebb8@CDC.GOV Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Sunday, November 10, 2002 6:26 PM ......snip........end..............TSS
also,
A. Aguzzi - Chronic Wasting Disease (CWD) also needs to be addressed. Most serious because of rapid horizontal spread and higher prevalence than BSE in UK, up to 15% in some populations. Also may be a risk to humans - evidence that it is not dangerous to humans is thin.
http://www.tseandfoodsafety.org/activities/bse_conference_basel_april_02/2summary_of_conference.htm
PLUS, i like to never forget this, but just what the hell would human cwd look like??? which one, or all of the 6 documented phenotypes of the sporadic CJD might cwd be envolved with, first passage, 2nd, 3rd etc, direct route and or friendly fire??? will they all look the same pathologically???
HOUND STUDY (CSE???)
http://www.priondata.org/data/A_deerdog.html#Hound
HOUND STUDY
AS implied in the Inset 25 we must not _ASSUME_ that transmission of BSE to other species will invariably present pathology typical of a scrapie-like disease.
snip...
http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf
Image] Research letters Volume 352, Number 9134 [Image] 3 October 1998 [Previous] [Next]
[Image][Image]Simultaneous occurrence of spongiform encephalopathy in a man and his cat in Italy [Image]
Gianluigi Zanusso, Ettore Nardelli, Anna Rosati, GianMaria Fabrizi, Sergio Ferrari, Antonella Carteri, Franco De Simone, Nicola Rizzuto, Salvatore Monaco
Transmissible spongiform encephalopathies (TSE) encompass inherited, acquired, and sporadic mammalian neurological disorders, and are characterised by the conversion of the cellular prion protein (PrP) in an insoluble and protease-resistant isoform (PrPres). In human TSE, four types of PrPres have been identified according to size and glycoform ratios, which may represent different prion strains. Type-1 and type-2 PrPres are associated with sporadic Creutzfeldt-Jakob disease (CJD), type 3 with iatrogenic CJD, and type 4 with variant CJD.1,2 There is evidence that variant CJD is caused by the bovine spongiform encephalopathy (BSE)-prion strain.2-4 The BSE strain has been identified in three cats with feline spongiform encephalopathy (FSE), a prion disease which appeared in 1990 in the UK.5 We report the simultaneous occurrence of sporadic CJD in a man and a new variety of FSE in his cat.
A 60-year-old man, with no unusual dietary habits, was admitted in November, 1993, because of dysarthria, cerebellar ataxic gait, visual agnosia, and myoclonus. An electroencephalogram (EEG) showed diffuse theta-delta activity. A brain magnetic resonance imaging scan was unremarkable. 10 days later, he was speechless and able to follow only simple commands. Repeat EEGs showed periodic triphasic complexes. 2 weeks after admission, he was mute, akinetic, and unable to swallow. He died in early January, 1994.
His 7-year-old, neutered, female shorthaired cat presented in November, 1993, with episodes of frenzy, twitching of its body, and hyperaesthesia. The cat was usually fed on canned food and slept on its owner's bed. No bites from the cat were recalled. In the next few days, the cat became ataxic, with hindquarter locomotor dysfunction; the ataxia got worse and there was diffuse myoclonus. The cat was killed in mid-January, 1994.
No pathogenic mutations in the patient's PrP gene were found. The patient and the cat were methionine homozygous at codon 129. Histology of the patient's brain showed neocortical and cerebellar neuronal loss, astrocytosis, and spongiosis (figure A). PrP immunoreactivity showed a punctate pattern and paralleled spongiform changes (figure B). The cat's brain showed mild and focal spongiosis in deeper cortical layers of all four lobes (figure C), vacuolated cortical neurons (figure D), and mild astrogliosis. The cerebellar cortex and the dentate nucleus were gliosed. Immunoreactive PrP showed a punctate pattern in neocortex, allocortex, and caudate nucleus (figure E). Western blot analysis of control and affected human and cat brain homogenates showed 3 PrP bands of 27-35 kDa. After digestion with proteinase K and deglycosylation, only samples from the affected patient and cat showed type-1 PrPres, with PrP glycoform ratios comparable to those observed in sporadic CJD1 (details available from author).
[Image]
Microscopic sections of patient and cat brains
A: Occipital cortex of the patient showing moderate spongiform degeneration and neuronal loss (haematoxylin and eosin) and B: punctate perineuronal pattern of PrP immunoreactivity; peroxidase immunohistochemistry with monoclonal antibody 3F4. C: cat parietal cortex showing mild spongiform degeneration (haematoxylin and eosin).D: vacuolated neurons (arrow, haematoxylin and eosin), E: peroxidase immunohistochemistry with antibody 3F4 shows punctate perineuronal deposition of PrP in temporal cortex.
This study shows a spatio-temporal association between human and feline prion diseases. The clinical features of the cat were different from previously reported cases of FSE which were characterised by gradual onset of behavioural changes preceding locomotor dysfunction and ataxia.5 Neuropathological changes were also at variance with the diffuse spongiosis and vacuolation of brainstem neurons, seen in FSE.5 The synaptic pattern of PrP deposition, similar in the cat and in the patient, was atypical for a BSE-related condition. Evidence of a new type of FSE was further provided by the detection of a type-1 PrPres, other than the BSE-associated type 4.2 Taken together, our data suggest that the same agent strain of sporadic CJD was involved in the patient and in his cat.
It is unknown whether these TSE occurred as the result of horizontal transmission in either direction, infection from an unknown common source, or the chance occurrence of two sporadic forms.
1 Parchi P, Castellani R, Capellari S, et al. Molecular basis of phenotypic variablity in sporadic Creutzfeldt-Jakob disease. Ann Neurol 1996; 39: 767-78 [PubMed].
2 Collinge J, Sidle KCL, Meads J, Ironside J, Hill AF. Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD. Nature 1996; 383: 685-90 [PubMed].
3 Bruce ME, Will RG, Ironside JW, et al. Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent. Nature 1997; 389: 498-501 [PubMed].
4 Hill AF, Desbruslais M, Joiner S, et al. The same prion strain causes vCJD and BSE. Nature 1997; 389: 448-50 [PubMed].
5 Pearson GR, Wyatt JM, Henderson JP, Gruffydd-Jones TJ. Feline spongiform encephalopathy: a review. Vet Annual 1993; 33: 1-10.
------------------------------------------------------------------------ Sezione di Neurologie Clinica, Dipartimento di Scienze Neurologiche e della Visione, Università di Verona, Policlinico Borgo Roma, 37134 Verona, Italy (S Monaco; e mail rizzuto@Gorgorna.univr.it); and Istituto Zooprofilattico Sperimentale della Lombardia e dell' Emilia, Brescia
=========================================
PET FOODS MAD CATS AND MAD DOGS BSE/TSEs
worse still, there is serious risk the media could get to hear of such a meeting...
snip...
Crushed heads (which inevitably involve brain and spinal cord material) are used to a limited extent but will also form one of the constituent raw materials of meat and bone meal, which is used extensively in pet food manufacturer...
http://www.bseinquiry.gov.uk/files/yb/1989/03/17004001.pdf
2. The Parliamentary Secretary said that he was concerned about the possibility that countries in which BSE had not yet been detected could be exporting raw meat materials (in particular crushed heads) contaminated with the disease to the UK for use in petfood manufacture...
snip...
YOU explained that imported crushed heads were extensively used in the petfood industry...
http://www.bseinquiry.gov.uk/files/yb/1989/04/14001001.pdf
In particular I do not believe one can say that the levels of the scrapie agent in pet food are so low that domestic animals are not exposed...
http://www.bseinquiry.gov.uk/files/yb/1989/04/24003001.pdf
40. When Sir Richard Southwood gave oral evidence to the Agriculture Select Committee, he said:
'The case of the cat is the first case of spongiform encephalopathy ever in a cat. Well, it appears to be - but if poor moggie got a bit of the staggers, you took it along to the vet and said it was not very happy, it would be put to sleep in the past and no one would have paid £300 to have a post mortem undertaken. So it is just possible that these were occurring before and were in fact caught from moggie being fed scrapie offal from sheep. I cannot say.'[46]
NEXT, ask yourself how many cats and dogs have ever been tested for TSE in the USA ???
Research Project: Transmission, Differentiation, and Pathobiology of Transmissible Spongiform Encephalopathies Location: Virus and Prion Diseases of Livestock
Title: Identification and Characterization of U.S. Bse Cases
Authors
Hall, S - USDA, APHIS, VS, NVSL Richt, Juergen Davis, A - USDA, APHIS, VS, NVSL Kluge, J - USDA, APHIS, VS, NVSL Simmons, M - VETERINARY LABS AGENCY,UK Stack, M - VETERINARY LABS AGENCY,UK Spencer, Y - VETERINARY LABS AGENCY,UK
Submitted to: Meeting Abstract Publication Type: Abstract Publication Acceptance Date: April 25, 2006 Publication Date: May 28, 2006 Citation: Hall, S.M., Richt, J., Davis, A., Kluge, J., Simmons, M., Stack, M., Spencer, Y. 2006. Identification and characterization of U.S. BSE cases [abstract]. Prion Diseases of Domestic Livestock. p. 25.
Technical Abstract: Bovine Spongiform Encephalopathy (BSE) surveillance has been ongoing in the USA since the early 1990¿s and initial testing was done at the USDA, National Veterinary Services Laboratories (NVSL) utilizing routine histopathology exclusively. In 1995, the immunohistochemistry (IHC) test was incorporated into surveillance testing in addition to routine histopathology. By 1999 virtually all BSE screening was performed by IHC and by 2001 the NVSL had switched to an automated IHC procedure. In 2002 and 2003 the NVSL tested about 20,000 high risk animals each year by IHC. In December, 2003 an animal was identified by IHC as positive for BSE (Case 1); this animal was determined to be imported from Canada. After this animal was identified, in June 2004 the USDA began its enhanced surveillance program as a shared effort between selected state veterinary diagnostic laboratories and NVSL, as part of the National Animal Health Laboratory Network. The plan called for testing as many targeted high risk animals as possible in a 12-18 month period. From June 1, 2004 through March 21, 2006, over 650,000 animals have been tested (Bio-Rad ELISA). Of those tested, two animals (Cases 2 and 3) have been identified as positive for BSE. While all three cases were strongly positive by Bio-Rad ELISA, Cases 2 and 3 have common features which are distinct from Case 1. Definitive spongiform changes in the obex, strong immunohistochemical reactions, and Western blot patterns similar to European BSE cases were observed in Case 1. In contrast, Cases 2 and 3 did not contain definitive histological lesions of BSE and the IHC staining was less intense than Case 1. In addition, Cases 2 (approximately 12 years) and Case 3 (approximately ten years) were older animals while Case 1 was 6.5 years old. Western blot analysis, PrP**Sc from Case 1 showed molecular features similar to typical BSE isolates, whereas PrP**Sc from Cases 2 and 3 revealed an unusual molecular PrP**Sc pattern: molecular mass of the unglycosylated and monoglycosylated isoform was higher than that of typical BSE isolates. Case 1 contained more PrP**Sc per brain tissue mg equivalent compared with Cases 2 and 3 using antibody 6H4. In Western Blot analysis, Case 2 and Case 3 were strongly positive with antibody P4, while Case 1 was negative or weakly positive with P4.
http://www.ars.usda.gov/research/publications/publications.htm?seq_no_115=194279
Differences in tissue distribution could require new regulations regarding specific risk material (SRM) removal.
snip...end
full text 33 PAGES ;
http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf
http://www.bseinquiry.gov.uk/files/yb/1988/10/00001001.pdf
It was, however, performed in the USA in 1979, when it was shown that cattle inoculated with the scrapie agent endemic in the flock of Suffolk sheep at the United States Department of Agriculture in Mission, Texas, developed a TSE quite unlike BSE. 32 The findings of the initial transmission, though not of the clinical or neurohistological examination, were communicated in October 1988 to Dr Watson, Director of the CVL, following a visit by Dr Wrathall, one of the project leaders in the Pathology Department of the CVL, to the United States Department of Agriculture. 33 The results were not published at this point, since the attempted transmission to mice from the experimental cow brain had been inconclusive. The results of the clinical and histological differences between scrapie-affected sheep and cattle were published in 1995. Similar studies in which cattle were inoculated intracerebrally with scrapie inocula derived from a number of scrapie-affected sheep of different breeds and from different States, were carried out at the US National Animal Disease Centre. 34 The results, published in 1994, showed that this source of scrapie agent, though pathogenic for cattle, did not produce the same clinical signs of brain lesions characteristic of BSE.
http://www.bseinquiry.gov.uk/
FULL TEXT ;
http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf
THE SEVEN SCIENTIST REPORT ***
http://www.fda.gov/ohrms/dockets/dockets/02n0273/02n-0273-EC244-Attach-1.pdf
Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)
http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf
[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and Requirement for the Disposition of Non-Ambulatory Disabled Cattle
03-025IFA 03-025IFA-2 Terry S. Singeltary
9/13/2005
http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518
CHRONIC WASTING DISEASE, CERVIDS - USA (NEW MEXICO) (02) ******************************************************** A ProMED-mail post
ProMED-mail is a program of the International Society for Infectious Diseases
Sponsored in part by Elsevier, publisher of Infectious Disease Gateway Alert
Date: Tue, 13 Dec 2005 11:53:52 -0600 From: Terry S Singeltary Sr Source: NM Press Release, 9 Dec 2005 [edited]
2 elk killed in the southern Sacramento Mountains of south east New Mexico have tested positive for chronic wasting disease (CWD), the Department of Game and Fish announced. The animals were the first elk in New Mexico to test positive for CWD since the disease was first discovered in mule deer in 2002.
Both CWD-afflicted elk were killed in an area 10 to 15 miles southeast of Cloudcroft in Game Management Unit 34, the same general area where the state's most recent case of CWD was detected in a mule deer. One of the elk -- a mature male -- was taken on 3 Oct 2005 by a hunter and showed no symptoms of the disease. The other elk -- a yearling female -- was in very poor condition and unable to stand when a Department of Game and Fish conservation officer found it 1 Oct 2005. Testing and verification of the samples required about 2 months. Future testing is expected to occur more quickly as the Department of Game and Fish and the Veterinary Diagnostic Services in the New Mexico Department of Agriculture further implement recently achieved in-state CWD testing capabilities.
"The range in which the disease is found appears to be expanding, so finding it in more animals in that area is not surprising," said Kerry Mower, the department's lead wildlife disease biologist. "But it is disappointing to find our first cases of CWD in free-ranging elk."
Brain stem samples from the 2 elk were among more than 100 taken from deer and elk in Unit 34 this year and sent to the Veterinary Diagnostic Services Laboratory in Albuquerque. The Albuquerque laboratory's "presumptive positive" samples from the 2 elk were confirmed as CWD-positive by the National Veterinary Services Laboratory in Ames, Iowa.
"We will continue our efforts to monitor the disease by actively testing animals in Units 34 and 19," Mower said. "We also encourage all hunters statewide to submit their animals for testing." The department personally informs hunters if the tests are positive. Hunters will be able to see the complete list of test results as they become available on the department website: .
This season, hunters who kill animals in a "control area" of Unit 34 are required to submit their animals for testing and observe special regulations affecting which body parts of a deer or elk can be removed from the unit. Hunting seasons continue in that area into January. Chronic wasting disease is a fatal neurological illness that afflicts deer, elk and moose. There is no evidence of CWD being transmitted to humans or livestock. The disease causes animals to become emaciated, display abnormal behavior, and lose control of bodily functions. To date, it has been found in captive and wild deer, elk, and moose in 8 states and 2 Canadian provinces.
The origin of CWD in New Mexico is unknown. It has been found in 12 wild deer and 2 wild elk since 2002, when the disease was first discovered east of Las Cruces. All of the CWD-positive deer and elk in New Mexico were from the southern Sacramento Mountains southeast of Cloudcroft and areas surrounding the Organ Mountains near Las Cruces.
For more information about CWD in New Mexico, including special regulations and how hunters can assist in research and prevention, visit . More information about CWD also can be found on the Chronic Wasting Disease Alliance site at or on the Colorado Division of Wildlife site at
CWD control map New Mexico (30 Aug 2005) .
-- Terry S Singeltary
[A clearer map but only up to July 2004, is at
- MHJ.Mod]
[see also: Chronic wasting disease, cervids - USA (NM) 20050628.1827 2003 --- Chronic wasting disease, cervids - USA (NM) (02) 20030217.0414 Chronic wasting disease, cervids - USA (NM) 20030207.0328 2002 --- Chronic wasting disease, cervids - USA (New Mexico) (02) 20020620.4548 Chronic wasting disease, cervids - USA (New Mexico) 20020619.4535]
......................mhj/pg/sh
*##########################################################* ************************************************************ ProMED-mail makes every effort to verify the reports that are posted, but the accuracy and completeness of the information, and of any statements or opinions based thereon, are not guaranteed. The reader assumes all risks in using information posted or archived by ProMED-mail. ISID and its associated service providers shall not be held responsible for errors or omissions or held liable for any damages incurred as a result of use or reliance upon posted or archived material. ************************************************************ Please support ProMED-mail by donating to the 2005 Internet- a-thon at ************************************************************ Visit ProMED-mail's web site at . Send all items for posting to: promed@promedmail.org (NOT to an individual moderator). If you do not give your full name and affiliation, it may not be posted. Send commands to subscribe/unsubscribe, get archives, help, etc. to: majordomo@promedmail.org. For assistance from a human being send mail to: owner-promed@promedmail.org. ############################################################
----- Original Message ----- From: "Terry S. Singeltary Sr." To: Cc: Sent: Wednesday, October 04, 2006 10:56 AM Subject: Re: PRO/AH/EDR> CJD (new var.) update 2006 (10)
Greetings ProMED,
Thank you for that kind correction from last months CJD ProMED posting, and further to the data on such atypical BSE cases in the USA, are as follows ;
4 October 2006 - The final minutes (154 KB) of the 93rd SEAC meeting held on 6 July 2006 have been published.
snip...
The committee will hold a reserved business session in the afternoon to allow discussion of unpublished studies on BSE in cattle. This is in accordance with the SEAC Code of Practice. Short summaries of the open and reserved business discussions will be posted on the SEAC website next week.
snip...
. The Chair noted that recent reports described two cases of BSE in cattle in the United States of America (USA) as being similar to atypical cases of BSE found in a number of European countries. The Chair suggested that the term "atypical BSE", used in the USA report, is potentially confusing and that this would be discussed under any other business. Dr Danny Matthews (Veterinary Laboratories Agency [VLA]) explained that data from western blots of the USA cases resembled that of a small number of atypical cases of BSE in France. A study of the French cases had shown the condition to be transmissible to mice by intracerebral (ic) inoculation with the neuropathological phenotype maintained on transmission3. Claims have been made about the existence of atypical cases of BSE in other countries but these have yet to be confirmed. No study has yet examined the tissue distribution of abnormal prion protein (PrPSc) or infectivity in such atypical cases of BSE. ...
snip...
http://www.seac.gov.uk/minutes/final93.pdf
[Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)
snip...
3. WHY still now only partial ruminant feed ban, with the fact that now we seem to have 3 cases of nvCJD to humans i.e. humanbovineTSE that were responsible from blood, and the fact the last 2 mad cows documented in the USA were that of an Atypical strain, would it not seem prudent to remove blood as well from ruminant feed ?
Page 4 of 98
8/3/2006
WOULD it not seem prudent to improve and expand the SRM list now? as per your own thinking ;
If transmission occurs, tissue distribution comparisons will be made between cattle infected with the atypical BSE isolate and the U.S. BSE isolate. Differences in tissue distribution could require new regulations regarding specific risk material (SRM) removal.
FULL text ;
Research Project: Study of Atypical Bse Location: Virus and Prion Diseases of Livestock Project Number: 3625-32000-073-07 Project Type: Specific C/A Start Date: Sep 15, 2004 End Date: Sep 14, 2007
Objective:
The objective of this cooperative research project with Dr. Maria Caramelli from the Italian BSE Reference Laboratory in Turin, Italy, is to conduct comparative studies with the U.S. bovine spongiform encephalopathy (BSE) isolate and the atypical BSE isolates identified in Italy. The studies will cover the following areas: 1. Evaluation of present diagnostics tools used in the U.S. for the detection of atypical BSE cases. 2. Molecular comparison of the U.S. BSE isolate and other typical BSE isolates with atypical BSE cases. 3. Studies on transmissibility and tissue distribution of atypical BSE isolates in cattle and other species.
Approach:
This project will be done as a Specific Cooperative Agreement with the Italian BSE Reference Laboratory, Istituto Zooprofilattico Sperimentale del Piemonte, in Turin, Italy. It is essential for the U.S. BSE surveillance program to analyze the effectiveness of the U.S diagnostic tools for detection of atypical cases of BSE. Molecular comparisons of the U.S. BSE isolate with atypical BSE isolates will provide further characterization of the U.S. BSE isolate. Transmission studies are already underway using brain homogenates from atypical BSE cases into mice, cattle and sheep. It will be critical to see whether the atypical BSE isolates behave similarly to typical BSE isolates in terms of transmissibility and disease pathogenesis. If transmission occurs, tissue distribution comparisons will be made between cattle infected with the atypical BSE isolate and the U.S. BSE isolate. Differences in tissue distribution could require new regulations regarding specific risk material (SRM) removal.
http://www.ars.usda.gov/research/projects/projects.htm?ACCN_NO=408490
Page 5 of 98 8/3/2006
HOWEVER, JAPAN has already shown infectivity in tissues other than CNS in there atypical TSE in cattle, so why should we wait, and expose many to this agent needlessly, since the last two mad cows in the USA were also atypical TSE ? PrPSc distribution of a natural case of bovine spongiform encephalopathy
Yoshifumi Iwamaru, Yuka Okubo, Tamako Ikeda, Hiroko Hayashi, Mori- kazu Imamura, Takashi Yokoyama and Morikazu Shinagawa Priori Disease Research Center, National Institute of Animal Health, 3-1-5 Kannondai, Tsukuba 305-0856 Japan gan@affrc.go.jp
Abstract ... snip...full text 98 pages ;
http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf
Published online before print February 17, 2004, 10.1073/pnas.0305777101
Medical Sciences Identification of a second bovine amyloidotic spongiform encephalopathy: Molecular similarities with sporadic Creutzfeldt-Jakob disease
http://www.pnas.org/cgi/content/abstract/0305777101v1
J Gen Virol 85 (2004), 2471-2478; DOI 10.1099/vir.0.79889-0 Characterization of two distinct prion strains derived from bovine spongiform encephalopathy transmissions to inbred mice
http://vir.sgmjournals.org/cgi/content/abstract/85/8/2471
[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and Requirement for the Disposition of Non-Ambulatory Disabled Cattle
03-025IFA 03-025IFA-2
http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf
SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 1997 TO 2004. SPORADIC CJD CASES TRIPLED;
please note 'TYPE UNKNOWN' ???
http://www.cjdsurveillance.com/resources-casereport.html
kind regards,
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518
----- Original Message ----- From: "ProMED-mail" To: Sent: Monday, October 02, 2006 10:22 AM Subject: PRO/AH/EDR> CJD (new var.) update 2006 (10)
CJD (NEW VAR.) UPDATE 2006 (10) ******************************* A ProMED-mail post
ProMED-mail is a program of the International Society for Infectious Diseases
[The definition of the designations deaths, definite cases, probable vCJD cases, and the case definitions can be found by accessing the Department of Health website, or by reference to a previous ProMED-mail post in this thread (for example, CJD (new var.) - UK: update March 2002 20020305.3693).
Data on vCJD cases from other parts of the world are now included in these updates whenever available.
Also, data on other forms of CJD (sporadic, iatrogenic, familial and GSS) are now included when they have some relevance to the incidence and etiology of vCJD. - Mod.CP]
In this update: [1] UK: Department of Health monthly CJD statistics, Mon 2 Oct 2006 [2] Germany: characterization of atypical BSE: correction
****** [1] UK: vCJD and CJD statistics Date: Mon 2 Oct 2006 From: ProMED-mail Source: UK Department of Health, Monthly Creutzfeldt-Jakob Disease Statistics, Mon 2 Oct 2006 [edited]
The Department of Health is today [Mon 2 Oct 2006] issuing the latest information about the numbers of known cases of Creutzfeldt-Jakob disease. This includes cases of variant Creutzfeldt-Jakob disease [abbreviated in ProMED-mail as CJD (new var.) or vCJD], the form of the disease thought to be linked to BSE (bovine spongiform encephalopathy).
Definite and probable CJD cases in the UK, as at Fri 29 Sep 2006: ----------------------------------------------------------------- Summary of vCJD cases - deaths ------------------------------ Deaths from definite vCJD (confirmed): 112 Deaths from probable vCJD (without neuropathological confirmation): 44 Deaths from probable vCJD (neuropathological confirmation pending): 0 Number of deaths from definite or probable vCJD (as above): 156
Summary of vCJD cases - alive ----------------------------- Number of probable vCJD cases still alive: 6
Total ----- Number of definite or probable vCJD (dead and alive): 162 (The next table will be published on Mon 6 Nov 2006).
Since the previous monthly statistics were released on Mon 4 Sep 2006, the total number of deaths from definite vCJD remains unchanged at 156, and the overall total number of definite or probable vCJD cases (dead and alive) also remains unchanged at 162 for the second month in succession.
These data are consistent with the view that the vCJD outbreak in the UK is in decline. The total number of deaths due to vCJD in the UK remains 156. The peak number of deaths was 28 in the year 2000, followed by 20 in 2001, 17 in 2002, 18 in 2003, and 9 in 2004, 5 in 2005. The number of deaths due to definite or probable vCJD in the UK during the first 9 months of 2006 remains 3.
Totals for all types of CJD cases in the UK in 2005 and 2006 ------------------------------------------------------------ As of 29 Sep 2006, in the UK in the year 2005, there were 122 referrals of suspected CJD, and there were 65 deaths from sporadic CJD, 6 from familial CJD, 3 from iatrogenic CJD, 6 GSS (Gerstmann-Straussler-Scheinker) syndrome cases, and 5 deaths from vCJD.
The corresponding figures so far for the 1st 9 months of 2006 are: 81 referrals, 41 deaths from sporadic CJD, 3 from vCJD, 4 from familial CJD, 3 from GSS and one from iatrogenic CJD.
During the period 1995, when vCJD was first diagnosed, up to the present there have been 937 deaths from all forms of CJD including 156 deaths attributable to definite or probable vCJD.
[These data are accessible via
. - Mod.CP]
-- ProMED-mail
****** [2] Correction Date: Tue 5 Sep 2006 From: "Terry S Singeltary Sr"
Characterization of atypical BSE in Germany: correction ------------------------------------------------------- [In the Moderator's comment accompanying the abstract of the paper entitled "Atypical BSE in Germany-Proof of transmissibility and biochemical characterization'" by A Buschmannaet et al, (see part [2] of CJD (new var.) update 2006 (09) 20060904.2519) it was wrongly implied that Terry S Singeltary Sr endorsed the conclusions of the paper, whereas his comments were intended merely to highlight the conclusions of the paper. Namely that the atypical cases suggested the possible existence of sporadic BSE cases in bovines and perhaps the BSE epidemic in the UK could have also been initiated by an intraspecies transmission from a sporadic BSE case. I apologize for inadvertently misrepresenting Terry's views. - Mod.CP]
Terry S Singeltary Sr has written the following. "In fact I disagree with the spontaneous/sporadic BSE/TSE theory, IF this is what the authors of this paper meant by 'sporadic BSE' to mean. For one thing, it has never been proven. IF atypical BSE i.e. BASE is so similar to some sporadic CJDs, then how did they all of a sudden become spontaneous? Could it not be so simple as an atypical BSE i.e. BASE was transmitted the same way most of all of the other BSE cattle were i.e. feed of just an atypical source, thus causing atypical strain? Why would these animals not develop an atypical BSE i.e. BASE from the same oral route? WHAT about an atypical strain mutating to become infectious via a lateral or horizontal mode in the bovine, as with CWD and scrapie? Also, please explain to me how a distinct synthetic prion, of a strain that is supposedly unlike any other we have ever seen, how can this explain 6 different documented phenotypes of sporadic CJD to date?
It's like trying to explain away all the 6 phenotypes of sporadic CJD with the spontaneous theory, it's just not scientific. OR, if you render an atypical TSE of what ever phenotype, in what ever species, of the atypical strain and feed it to another whatever species, nothing happens x 1 x 2 x 3 x 4 etc passage? This all has been proven?
Please show me these transmission studies? What Prusiner and Soto produced in vitro did not look like any natural field TSE, and as far as this in vitro TSE being infectious, well this was questionable too. If this was the case, then why does CWD not spontaneously happen in geographical areas where it has never been documented, OR with scrapie, as in scrapie free New Zealand? If TSE were to arise spontaneously, I don't see how the scientific arena can dictate which animal TSE can arise spontaneously, and which ones cannot, without any scientific evidence to support this to date, and by even suggesting this in this study, was not scientific. The words sporadic and spontaneous are very confusing in the world literature of human and animal TSE and, in my opinion, should not be used as terminology of any TSE."
-- Terry S Singeltary Sr
[see also: CJD (new var.) update 2006 (09) 20060904.2519 CJD (new var.) update 2006 (08) 20060807.2207 CJD (new var.) update 2006 (07) 20060703.1831 CJD (new var.) - Netherlands: 2nd case 20060623.1741 CJD (new var.) update 2006 (06) 20060605.1566 CJD (new var.) update 2006 (05) 20060508.1332 CJD (new var.) update 2006 (04) 20060404.1005 CJD (new var.) update 2006 (03) 20060306.0728 CJD (new var.) - UK: 3rd transfusion-related case 20060209.0432 CJD (new var.) update 2006 (02) 20060206.0386 CJD (new var.) update 2006 (01) 20060111.0101 CJD (new var.) update 2006 20060111.0101 2005 --- CJD (new var.) update 2005 (12) 20051209.3547 CJD (new var.) update 2005 (11) 20051108.3270 CJD (new var.) update 2005 (10) 20051006.2916 CJD (new var.) update 2005 (09) 20050905.2627 CJD (new var.) update 2005 (08) 20050801.2237 CJD (new var.) update 2005 (07) 20050703.1889 CJD (new var.) update 2005 (06) 20050607.1584 CJD (new var.) update 2005 (05) 20050505.1243 CJD (new var.) update 2005 (04) 20050405.0982 CJD (new var.) update 2005 (03) 20050308.0687 CJD (new var.) update 2005 (02) 20050211.0467 CJD (new var.) - UK: update 2005 (01) 20050111.0095 2004 --- CJD, genetic susceptibility 20041112.3064 CJD (new var.) - UK: update 2004 (14) 20041206.3242 CJD (new var.) - UK: update 2004 (13) 20041103.2977 CJD (new var.) - UK: update 2004 (12) 20041023.2871 CJD (new var.) - UK: update 2004 (11) 20041008.2758 CJD (new var.) - UK: update 2004 (10) 20040909.2518 CJD (new var.) - UK: update 2004 (09) 20040809.2199 CJD (new var.) - UK: update 2004 (08) 20040806.2150 CJD (new var.) - UK: update 2004 (07) 20040706.1807 CJD (new var.) - UK: update 2004 (06) 20040608.1535 CJD (new var.) - UK: update 2004 (05) 20040510.1262 CJD (new var.) - UK: update 2004 (04) 20040406.0937 CJD (new var.) - UK: update 2004 (03) 20040314.0713 CJD (new var.) - UK: update 2004 (02) 20040202.0400 CJD (new var.) - UK: update 2004 (01) 20040106.0064 CJD (new var.) - France: 8th case 20041022.2864 CJD (new var.) - France: 9th case 20041123.3138 CJD (new var.), blood supply - UK 20040318.0758 CJD (new var.), carrier frequency study - UK 20040521.1365 2003 --- CJD (new var.) - UK: update 2003 (13) 20031216.3072 CJD (new var.) - UK: update 2003 (01) 20030108.0057 2002 --- CJD (new var.) - UK: update Dec 2002 20021207.5997 CJD (new var.) - UK: update Jan 2002 20020111.3223 2001 --- CJD (new var.), incidence & trends - UK (02) 20011124.2875 CJD (new var.), incidence & trends - UK 20011115.2816 CJD (new var.) - UK: reassessment 20011029.2671 CJD (new var.) - UK: update Oct 2001 20011005.2419 CJD (new var.) - UK: regional variation (02) 20010907.2145 CJD (new var.) - UK: update Sep 2001 20010906.2134 CJD (new var.) - UK: update Aug 2001 20010808.1872 CJD (new var.) - UK: 9th Annual Report 20010628.1231 CJD (new var.) - UK: update June 2001 20010622.1188 CJD (new var.) - UK: update 3 Jan 2001 20010104.0025]
................cp/sh
*##########################################################* ************************************************************ ProMED-mail makes every effort to verify the reports that are posted, but the accuracy and completeness of the information, and of any statements or opinions based thereon, are not guaranteed. The reader assumes all risks in using information posted or archived by ProMED-mail. ISID and its associated service providers shall not be held responsible for errors or omissions or held liable for any damages incurred as a result of use or reliance upon posted or archived material. ************************************************************ Become a ProMED-mail Premium Subscriber at
************************************************************ Visit ProMED-mail's web site at . Send all items for posting to: promed@promedmail.org (NOT to an individual moderator). If you do not give your full name and affiliation, it may not be posted. Send commands to subscribe/unsubscribe, get archives, help, etc. to: majordomo@promedmail.org. For assistance from a human being send mail to: owner-promed@promedmail.org. ############################################################
2006 mad cow protein to deer
e) "Big Jim's" BBB Deer Ration, Big Buck Blend, Recall # V-104-6;
every wonder why they are so big......mad cow protein ;
http://www.fda.gov/bbs/topics/enforce/2006/ENF00963.html
2003D-0186 Guidance for Industry: Use of Material From Deer and Elk In Animal Feed
EMC 7 Terry S. Singeltary Sr. Vol #: 1
-------- Original Message --------
Subject: DOCKET-- 03D-0186 -- FDA Issues Draft Guidance on Use of Material From Deer and Elk in Animal Feed; Availability Date: Fri, 16 May 2003 11:47:37 -0500 From: "Terry S. Singeltary Sr." To: fdadockets@oc.fda.gov
Greetings FDA,
i would kindly like to comment on;
Docket 03D-0186
FDA Issues Draft Guidance on Use of Material From Deer and Elk in Animal Feed; Availability
Several factors on this apparent voluntary proposal disturbs me greatly, please allow me to point them out;
1. MY first point is the failure of the partial ruminant-to-ruminant feed ban of 8/4/97. this partial and voluntary feed ban of some ruminant materials being fed back to cattle is terribly flawed. without the _total_ and _mandatory_ ban of all ruminant materials being fed back to ruminants including cattle, sheep, goat, deer, elk and mink, chickens, fish (all farmed animals for human/animal consumption), this half ass measure will fail terribly, as in the past decades...
2. WHAT about sub-clinical TSE in deer and elk? with the recent findings of deer fawns being infected with CWD, how many could possibly be sub-clinically infected. until we have a rapid TSE test to assure us that all deer/elk are free of disease (clinical and sub-clinical), we must ban not only documented CWD infected deer/elk, but healthy ones as well. it this is not done, they system will fail...
3. WE must ban not only CNS (SRMs specified risk materials), but ALL tissues. recent new and old findings support infectivity in the rump or ass muscle. wether it be low or high, accumulation will play a crucial role in TSEs.
4. THERE are and have been for some time many TSEs in the USA. TME in mink, Scrapie in Sheep and Goats, and unidentified TSE in USA cattle. all this has been proven, but the TSE in USA cattle has been totally ignored for decades. i will document this data below in my references.
5. UNTIL we ban all ruminant by-products from being fed back to ALL ruminants, until we rapid TSE test (not only deer/elk) but cattle in sufficient numbers to find (1 million rapid TSE test in USA cattle annually for 5 years), any partial measures such as the ones proposed while ignoring sub-clinical TSEs and not rapid TSE testing cattle, not closing down feed mills that continue to violate the FDA's BSE feed regulation (21 CFR 589.2000) and not making freely available those violations, will only continue to spread these TSE mad cow agents in the USA. I am curious what we will call a phenotype in a species that is mixed with who knows how many strains of scrapie, who knows what strain or how many strains of TSE in USA cattle, and the CWD in deer and elk (no telling how many strains there), but all of this has been rendered for animal feeds in the USA for decades. it will get interesting once someone starts looking in all species, including humans here in the USA, but this has yet to happen...
6. IT is paramount that CJD be made reportable in every state (especially ''sporadic'' cjd), and that a CJD Questionnaire must be issued to every family of a victim of TSE. only checking death certificates will not be sufficient. this has been proven as well (see below HISTORY OF CJD -- CJD QUESTIONNAIRE)
7. WE must learn from our past mistakes, not continue to make the same mistakes...
REFERENCES
Oral transmission and early lymphoid tropism of chronic wasting disease PrPres in mule deer fawns (Odocoileus hemionus ) Christina J. Sigurdson1, Elizabeth S. Williams2, Michael W. Miller3, Terry R. Spraker1,4, Katherine I. O'Rourke5 and Edward A. Hoover1
Department of Pathology, College of Veterinary Medicine and Biomedical Sciences, Colorado State University, Fort Collins, CO 80523- 1671, USA1 Department of Veterinary Sciences, University of Wyoming, 1174 Snowy Range Road, University of Wyoming, Laramie, WY 82070, USA 2 Colorado Division of Wildlife, Wildlife Research Center, 317 West Prospect Road, Fort Collins, CO 80526-2097, USA3 Colorado State University Veterinary Diagnostic Laboratory, 300 West Drake Road, Fort Collins, CO 80523-1671, USA4 Animal Disease Research Unit, Agricultural Research Service, US Department of Agriculture, 337 Bustad Hall, Washington State University, Pullman, WA 99164-7030, USA5
Author for correspondence: Edward Hoover.Fax +1 970 491 0523. e-mail ehoover@lamar.colostate.edu
Mule deer fawns (Odocoileus hemionus) were inoculated orally with a brain homogenate prepared from mule deer with naturally occurring chronic wasting disease (CWD), a prion-induced transmissible spongiform encephalopathy. Fawns were necropsied and examined for PrP res, the abnormal prion protein isoform, at 10, 42, 53, 77, 78 and 80 days post-inoculation (p.i.) using an immunohistochemistry assay modified to enhance sensitivity. PrPres was detected in alimentary-tract-associated lymphoid tissues (one or more of the following: retropharyngeal lymph node, tonsil, Peyer's patch and ileocaecal lymph node) as early as 42 days p.i. and in all fawns examined thereafter (53 to 80 days p.i.). No PrPres staining was detected in lymphoid tissue of three control fawns receiving a control brain inoculum, nor was PrPres detectable in neural tissue of any fawn. PrPres-specific staining was markedly enhanced by sequential tissue treatment with formic acid, proteinase K and hydrated autoclaving prior to immunohistochemical staining with monoclonal antibody F89/160.1.5. These results indicate that CWD PrP res can be detected in lymphoid tissues draining the alimentary tract within a few weeks after oral exposure to infectious prions and may reflect the initial pathway of CWD infection in deer. The rapid infection of deer fawns following exposure by the most plausible natural route is consistent with the efficient horizontal transmission of CWD in nature and enables accelerated studies of transmission and pathogenesis in the native species.
snip...
These results indicate that mule deer fawns develop detectable PrP res after oral exposure to an inoculum containing CWD prions. In the earliest post-exposure period, CWD PrPres was traced to the lymphoid tissues draining the oral and intestinal mucosa (i.e. the retropharyngeal lymph nodes, tonsil, ileal Peyer's patches and ileocaecal lymph nodes), which probably received the highest initial exposure to the inoculum. Hadlow et al. (1982) demonstrated scrapie agent in the tonsil, retropharyngeal and mesenteric lymph nodes, ileum and spleen in a 10-month-old naturally infected lamb by mouse bioassay. Eight of nine sheep had infectivity in the retropharyngeal lymph node. He concluded that the tissue distribution suggested primary infection via the gastrointestinal tract. The tissue distribution of PrPres in the early stages of infection in the fawns is strikingly similar to that seen in naturally infected sheep with scrapie. These findings support oral exposure as a natural route of CWD infection in deer and support oral inoculation as a reasonable exposure route for experimental studies of CWD.
snip...
http://vir.sgmjournals.org/cgi/content/full/80/10/2757
Subject: MAD DEER/ELK DISEASE AND POTENTIAL SOURCES Date: Sat, 25 May 2002 18:41:46 -0700 From: "Terry S. Singeltary Sr." Reply-To: BSE-L To: BSE-L
8420-20.5% Antler Developer For Deer and Game in the wild Guaranteed Analysis Ingredients / Products Feeding Directions
snip...
_animal protein_
http://www.surefed.com/deer.htm
BODE'S GAME FEED SUPPLEMENT #400 A RATION FOR DEER NET WEIGHT 50 POUNDS 22.6 KG.
snip...
_animal protein_
http://www.bodefeed.com/prod7.htm
Ingredients
Grain Products, Plant Protein Products, Processed Grain By-Products, Forage Products, Roughage Products 15%, Molasses Products, __Animal Protein Products__, Monocalcium Phosphate, Dicalcium Pyosphate, Salt, Calcium Carbonate, Vitamin A Acetate with D-activated Animal Sterol (source of Vitamin D3), Vitamin E Supplement, Vitamin B12 Supplement, Riboflavin Supplement, Niacin Supplement, Calcium Panothenate, Choline Chloride, Folic Acid, Menadione Soduim Bisulfite Complex, Pyridoxine Hydorchloride, Thiamine Mononitrate, d-Biotin, Manganous Oxide, Zinc Oxide, Ferrous Carbonate, Calcium Iodate, Cobalt Carbonate, Dried Sacchoromyces Berevisiae Fermentation Solubles, Cellulose gum, Artificial Flavors added.
http://www.bodefeed.com/prod6.htm ===================================
MORE ANIMAL PROTEIN PRODUCTS FOR DEER
Bode's #1 Game Pellets A RATION FOR DEER F3153
GUARANTEED ANALYSIS Crude Protein (Min) 16% Crude Fat (Min) 2.0% Crude Fiber (Max) 19% Calcium (Ca) (Min) 1.25% Calcium (Ca) (Max) 1.75% Phosphorus (P) (Min) 1.0% Salt (Min) .30% Salt (Max) .70%
Ingredients
Grain Products, Plant Protein Products, Processed Grain By-Products, Forage Products, Roughage Products, 15% Molasses Products, __Animal Protein Products__, Monocalcium Phosphate, Dicalcium Phosphate, Salt, Calcium Carbonate, Vitamin A Acetate with D-activated Animal Sterol (source of Vitamin D3) Vitamin E Supplement, Vitamin B12 Supplement, Roboflavin Supplement, Niacin Supplement, Calcium Pantothenate, Choline Chloride, Folic Acid, Menadione Sodium Bisulfite Complex, Pyridoxine Hydrochloride, Thiamine Mononitrate, e - Biotin, Manganous Oxide, Zinc Oxide, Ferrous Carbonate, Calcium Iodate, Cobalt Carbonate, Dried Saccharyomyces Cerevisiae Fermentation Solubles, Cellulose gum, Artificial Flavors added.
FEEDING DIRECTIONS Feed as Creep Feed with Normal Diet
http://www.bodefeed.com/prod8.htm
INGREDIENTS
Grain Products, Roughage Products (not more than 35%), Processed Grain By-Products, Plant Protein Products, Forage Products, __Animal Protein Products__, L-Lysine, Calcium Carbonate, Salt, Monocalcium/Dicalcium Phosphate, Yeast Culture, Magnesium Oxide, Cobalt Carbonate, Basic Copper Chloride, Manganese Sulfate, Manganous Oxide, Sodium Selenite, Zinc Sulfate, Zinc Oxide, Sodium Selenite, Potassium Iodide, Ethylenediamine Dihydriodide, Vitamin E Supplement, Vitamin A Supplement, Vitamin D3 Supplement, Mineral Oil, Mold Inhibitor, Calcium Lignin Sulfonate, Vitamin B12 Supplement, Menadione Sodium Bisulfite Complex, Calcium Pantothenate, Riboflavin, Niacin, Biotin, Folic Acid, Pyridoxine Hydrochloride, Mineral Oil, Chromium Tripicolinate
DIRECTIONS FOR USE
Deer Builder Pellets is designed to be fed to deer under range conditions or deer that require higher levels of protein. Feed to deer during gestation, fawning, lactation, antler growth and pre-rut, all phases which require a higher level of nutrition. Provide adequate amounts of good quality roughage and fresh water at all times.
http://www.profilenutrition.com/Products/Specialty/deer_builder_pellets.html ===================================================
DEPARTMENT OF HEALTH & HUMAN SERVICES PUBLIC HEALTH SERVICE FOOD AND DRUG ADMINISTRATION
April 9, 2001 WARNING LETTER
01-PHI-12 CERTIFIED MAIL RETURN RECEIPT REQUESTED
Brian J. Raymond, Owner Sandy Lake Mills 26 Mill Street P.O. Box 117 Sandy Lake, PA 16145 PHILADELPHIA DISTRICT
Tel: 215-597-4390
Dear Mr. Raymond:
Food and Drug Administration Investigator Gregory E. Beichner conducted an inspection of your animal feed manufacturing operation, located in Sandy Lake, Pennsylvania, on March 23, 2001, and determined that your firm manufactures animal feeds including feeds containing prohibited materials. The inspection found significant deviations from the requirements set forth in Title 21, code of Federal Regulations, part 589.2000 - Animal Proteins Prohibited in Ruminant Feed. The regulation is intended to prevent the establishment and amplification of Bovine Spongiform Encephalopathy (BSE) . Such deviations cause products being manufactured at this facility to be misbranded within the meaning of Section 403(f), of the Federal Food, Drug, and Cosmetic Act (the Act).
Our investigation found failure to label your swine feed with the required cautionary statement "Do Not Feed to cattle or other Ruminants" The FDA suggests that the statement be distinguished by different type-size or color or other means of highlighting the statement so that it is easily noticed by a purchaser.
In addition, we note that you are using approximately 140 pounds of cracked corn to flush your mixer used in the manufacture of animal feeds containing prohibited material. This flushed material is fed to wild game including deer, a ruminant animal. Feed material which may potentially contain prohibited material should not be fed to ruminant animals which may become part of the food chain.
The above is not intended to be an all-inclusive list of deviations from the regulations. As a manufacturer of materials intended for animal feed use, you are responsible for assuring that your overall operation and the products you manufacture and distribute are in compliance with the law. We have enclosed a copy of FDA's Small Entity Compliance Guide to assist you with complying with the regulation... blah, blah, blah...tss
http://www.fda.gov/foi/warning_letters/g1115d.pdf
SNIP...FULL TEXT ;
http://www.fda.gov/ohrms/dockets/dailys/03/oct03/100203/100203.htm
TONS Products manufactured from 02/01/2005 until 06/06/2006 Date: August 6, 2006 at 6:16 pm PST PRODUCT a) CO-OP 32% Sinking Catfish, Recall # V-100-6; b) Performance Sheep Pell W/Decox/A/N, medicated, net wt. 50 lbs, Recall # V-101-6; c) Pro 40% Swine Conc Meal -- 50 lb, Recall # V-102-6; d) CO-OP 32% Sinking Catfish Food Medicated, Recall # V-103-6;
*********************************
e) "Big Jim's" BBB Deer Ration, Big Buck Blend, Recall # V-104-6;
*********************************
f) CO-OP 40% Hog Supplement Medicated Pelleted, Tylosin 100 grams/ton, 50 lb. bag, Recall # V-105-6; g) Pig Starter Pell II, 18% W/MCDX Medicated 282020, Carbadox -- 0.0055%, Recall # V-106-6; h) CO-OP STARTER-GROWER CRUMBLES, Complete Feed for Chickens from Hatch to 20 Weeks, Medicated, Bacitracin Methylene Disalicylate, 25 and 50 Lbs, Recall # V-107-6; i) CO-OP LAYING PELLETS, Complete Feed for Laying Chickens, Recall # 108-6; j) CO-OP LAYING CRUMBLES, Recall # V-109-6; k) CO-OP QUAIL FLIGHT CONDITIONER MEDICATED, net wt 50 Lbs, Recall # V-110-6; l) CO-OP QUAIL STARTER MEDICATED, Net Wt. 50 Lbs, Recall # V-111-6; m) CO-OP QUAIL GROWER MEDICATED, 50 Lbs, Recall # V-112-6 CODE Product manufactured from 02/01/2005 until 06/06/2006 RECALLING FIRM/MANUFACTURER Alabama Farmers Cooperative, Inc., Decatur, AL, by telephone, fax, email and visit on June 9, 2006. FDA initiated recall is complete.
REASON
Animal and fish feeds which were possibly contaminated with ruminant based protein not labeled as "Do not feed to ruminants".
VOLUME OF PRODUCT IN COMMERCE
125 tons
DISTRIBUTION AL and FL
END OF ENFORCEMENT REPORT FOR AUGUST 2, 2006
###
http://www.fda.gov/bbs/topics/enforce/2006/ENF00963.html
snip...end...TSS
Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518
posted by Terry S. Singeltary Sr. at
9:01 PM
0 Comments:
Post a Comment
Subscribe to Post Comments [Atom]
<< Home