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From: TSS ()
----- Original Message ----- ##################### Bovine Spongiform Encephalopathy ##################### Posted on Sun, Jul. 31, 2005 Texas case demonstrates weaknesses in testing of food supply for disease By Barry Shlachter Star-Telegram Staff Writer Public confidence wasn't enhanced last week when the USDA announced that a private veterinarian had "forgotten" about a brain tissue sample he took in April. It came from another cow now suspected of having had the brain-wasting disease bovine spongiform encephalopathy. Definitive tests are under way. Watchdog groups awarded barely passing marks to the department for its handling of the Texas cow, which turned up dead at a Waco packing plant Nov. 15. The USDA finally confirmed the mad cow case June 10 after multiple tests. "USDA gets a D or D minus," said Caroline Smith Dewaal of the Washington-based advocacy group, Center for Science in the Public Interest. "The best thing that came out of this is the work of the inspector general." It was the department's in-house watchdog, Inspector General Phyllis Fong, who skirted the USDA hierarchy by ordering retesting with a different method more than six months after a routine second-round test, known as the immunohistochemistry (IHC) test, proved negative for BSE. Agriculture Secretary Mike Johanns, who assumed office in January, has said he neither knew about nor authorized the retesting by the National Veterinary Services Laboratories in Ames, Iowa. Just why Fong, a lawyer who grew up in Hawaii, acted in such a forthright, hierarchy-dodging manner has puzzled many involved in the industry. In a statement, her office said the retesting was prompted by a review of "voluminous records" showing an unusual pattern of conflicting test results in the case. Industry sources say there is speculation that she responded to concern expressed in scientific circles. Two early tests, one reportedly conducted at Texas A&M University and a second in Ames, produced conflicting results -- one inconclusive, one negative. Unbeknownst to Fong and the public, Ames researchers had also used an experimental rapid version of the IHC test on brain tissue from the Texas cow. That proved positive for BSE, but staff members thought the result was technically flawed and the USDA didn't disclose until just recently that the Ames lab had conducted the experimental test. Months later, Fong stepped in and ordered more tests. A "Western blot" test proved positive, as did later tests at a lab in Weybridge, England. Finally in June, two days after the Weybridge lab confirmed the mad cow case, a chastened USDA announced that in addition to the routine IHC test, it was adopting the Western blot procedure whenever an initial "BioRad" screening test points to possible BSE. In addition, backup tests will now be conducted at Britain's national veterinary laboratory in Weybridge when earlier test results conflict or are inconclusive. All this sounded familiar to Consumers Union. In February, the nonprofit public interest group that publishes Consumer Reports urged the USDA to take those same steps in regard to the Texas cow, noted Michael Hansen, a senior researcher with the organization. In hindsight, the March 18 response to Consumers Union by Jere Dick, associate deputy administrator of USDA's animal health policy and programs, smeared egg clearly across the department's collective face. Referring to the Texas cow, Dick wrote: "We are confident in the expertise of USDA's laboratory technicians conducting BSE testing and do not feel that such confirmatory testing by the Weybridge laboratory is generally necessary, nor would the use of the Western blot test have enhanced the result of our November 2004 testing." The opposite turned out to be true. Sen. Tom Harkin, D-Iowa, noted the obvious in a July 7 letter to Johanns: "Without the prompting of the [inspector general's office] , we still would not know that the brain sample in question tests BSE-positive." While Americans should have no misgivings about eating steaks and hamburgers, Harkin said, "there is a limit to how much of its own errors, inconsistencies and lack of transparency USDA can reasonably expect consumers to abide and still have confidence in the safety of beef." Food supply kept safe All the criticism might obscure the fact that meat from diseased livestock has been kept out of the food supply. BSE is carried by hard-to-destroy protein prions, which scientists believe can cause a rare human disease -- variant Creutzfeldt-Jakob -- if found in beef. The food supply has been protected by a ban for human consumption of tissue most prone to prion contamination, including beef brain, scrapings from the spinal column, and a small section of the intestine. USDA's animal health officials were dealing with a highly unusual situation, said Keith Belk, a Colorado State University animal science professor who closely follows the BSE issue. "There clearly were some mistakes made, but this was a pretty unique cow," Belk said. "She had the disease, and it incubated for a long time. But it apparently received a light dose of prions that were very sporadically spread" in the brain tissue. The tissue sample, a pie-slice-shaped sliver taken from the obex, a slight bulge in the brain stem, had so few BSE prions at some points that a British expert, Dr. Danny Matthews of Weybridge, said tests easily could have missed them. Matthews has said BSE is becoming rarer and more difficult to detect because of effective bans on tainted cattle feeds, which are believed to spread the disease. Some of the missteps, ironically, were caused by USDA staff doing exactly what they were supposed to do -- faithfully following measures that go far beyond international standards, Belk said in a call from Fort Collins, Colo. "They appeared to have made some significant errors, which unfortunately made them appear foolish," Belk said. "Most scientists around the world would have argued that they should have run both the Western blot and IHC tests. I think they would have liked to have done so; but because of policy in place, their hands were tied." Under lab rules in effect in November, the USDA staff in Ames was not permitted to carry out any other type of test after it received a negative result from the second round of testing, using the IHC method. Communication miscues haven't helped the USDA's image in its handling of the Texas case. In June, the USDA finally confirmed that the yellow to cream-colored cow had arrived dead at a meatpacking plant, not a dog food plant as originally reported. And it had not been a downer -- a live but nonwalking animal -- as described by officials for nearly seven months. Making matters worse, the initial description of the cow as an Angus was wrong, and some of its high-risk body parts were tossed in a bin with those of other cattle, creating the need to run a number of DNA tests. Dewaal also criticized the successful effort to keep the name and location of the ranch that produced the Brahman cross-bred cow secret. The ranch has been speculated to be somewhere in Southeast Texas. "Who are they protecting?" she asked. "The only person it protects is the rancher." But officials like Carla Everett, a spokeswoman with the Texas Animal Health Commission, say that maintaining confidentiality is necessary to prevent such operations from being needlessly stigmatized and to ensure future cooperation from ranches to which other diseased animals might be traced. Owning up Although chastened, the USDA said it had no choice but to treat the Texas cow case as it had. "It was handled within our protocol with few exceptions," said Jim Rogers, a spokesman for the department's Animal and Plant Health Inspection Service. But the department is owning up to some mistakes. Johanns has acknowledged that some things should have been done differently, Rogers said. Among them: Animal parts from different cattle should not have been commingled as they were at the Waco pet food plant; the Ames laboratory should not have run an experimental test while running the official test; and the lab made a verbal disclosure, but it should have presented a written report. Whatever steps the USDA takes, it could find itself in a no-win situation, Rogers said. In November, Western blot was not part of the testing protocol because it was not deemed appropriate in that situation. "Let's go back in time and say we had conducted Western blot," Rogers said. "We would have critics saying we had stepped outside our protocol." In fact, the National Cattlemen's Beef Association publicly criticized the inspector general for stepping out of the testing routine -- just before it became clear that her irregular action had turned up the positive BSE finding. Reinforcing experts' description of the Texas cow as an unusual case, Rogers noted that after Fong requested that Western blot be used on the brain sample, "they tested it 10 times -- five of which were negative and five positive." The results could be far different depending on what part of the sample was examined because of the erratic spread of BSE prions. Scientists believe that these submicroscopic flecks of protein spread BSE, a rare but always fatal disease discovered in 1986, through feed contaminated with rendered cattle parts. The infected Texas cow was born before such tainted feed was banned in the United States in 1997. Ninety-seven percent of beef now consumed by Americans comes from cattle born after the ban, according to the National Cattlemen's Beef Association. By comparison, Japan did not ban such feed until 2001, possibly contributing to the 20 BSE cases there -- and a heightened popular distrust of its food security program, Belk said. That lack of public confidence prompted testing of every animal going to slaughter and a continued reluctance to end a 19-month ban on U.S. beef imports. While such feed is banned for cattle, it can still be fed to pigs and chickens, which cannot contract BSE. But some scientists and consumer groups, fearing mislabeling of sacks or other human errors, have urged the Food and Drug Administration, which regulates the feed industry, to act on its own January 2004 recommendation and ban it for poultry and pigs, too. One U.S. case Of the 153 worldwide cases of a fatal human ailment believed caused by BSE-contaminated beef products, the only victim of variant Creutzfeldt-Jakob disease in the United States was a Florida woman who had lived in Britain when such tainted food was available. Six months after the first U.S. case in a Canadian-born dairy cow was discovered in December 2003, scientists said BSE was probably present at low levels in the U.S. herd, and the USDA launched a widespread surveillance effort. The beef industry notes that the Texas animal was the only cow found with BSE after 419,113 high-risk animals have been tested. In the most recent case, involving a cow that was euthanized after severe calving problems whose test samples were forgotten about, the carcass was incinerated. The Texas and Washington state cows were discovered before they entered the food chain, reinforcing the government and industry's insistence that world-class firewalls have kept U.S. consumers protected. After the Texas cow arrived dead at a Waco packinghouse, it was trucked across town to a dog food plant, where test samples were taken. The animal's body, kept segregated, was incinerated at Texas A&M University. Unfortunately, by then its parts were commingled with three other cattle, making the difficult task of tracing a suspect cow without a mandatory national animal identification program even harder. This was seen as another blunder by authorities. Hansen of Consumers Union complained that little is known publicly about the 419,113 tested cattle -- or many others with central nervous system problems that were never examined. He cited an August 2004 audit by the inspector general's office as saying that only a fraction of the highest-risk cattle -- erratic-acting animals that tested negative for rabies -- were not given the rapid BioRad test for BSE. In fiscal 2003, for example, 108 Texas cattle were tested and found clean for rabies, but only 29 of this high-risk group were checked for BSE. In South Dakota, 81 tested cattle were negative for rabies, but none were given BSE tests. Rogers conceded that there was a "mix-up" in states where testing guidelines were not clearly understood. The confusion has been remedied, and by late September, all animal inspectors understood that such high-risk cattle must be tested for BSE, he said. Call for tracking Chief among complaints by watchdog groups like Consumers Union and the Center for Science in the Public Interest is that the United States -- unlike the European Union, Japan, Canada, Australia and New Zealand -- still has no mandatory national identification program for tracking cattle. An ID program would help quickly trace the origins of a contaminated cow. As the mad-cow story developed this summer, the National Cattlemen's Beef Association got a head start on Johanns by saying it was starting its own animal ID system and hoped to have it operational in January 2007. The association is seeking to have a system that would eventually be taken over as the national program. It envisions one that would ensure confidentiality of certain proprietary data that cattle operations and others want withheld for competitive reasons. "For example, Cargill doesn't want Tyson to know where they purchase cattle," said Belk of Colorado State, referring to major beef processing companies. And ranchers, some of whom have run cattle for generations, are worried about consumer lawsuits, he said. "If you can determine where something comes from, it opens the door to liability," Belk said. Johanns had announced that the government's mandatory ID system won't be up and running until 2009. "I think the system is flawed as long as we don't have a national mandatory system for tracking cows," said Dewaal, who noted that Canada went from a voluntary to a mandatory system in one year. "We may be better at finding the one infected animal but not others similarly exposed to the disease. "And we are surprised by the extraordinary delay." Consumers, ever more concerned about food safety, are putting money where their mouths are and demanding beef traceability. In Texas, chains such as United Market Street, Whole Foods and Central Market are offering source-verified beef -- meat whose herd or ranch origin is spelled out to consumers. In doing so, the market will push for an animal ID system to be implemented before 2009, Belk predicted. And source verifiability might be needed to re-establish lost export markets, he said. And such traceability carries plenty of benefits. Some systems could go beyond a mandatory animal ID system, Belk said, providing useful information: Is the animal free of antibiotics? Was it given vitamin E for a certain antioxidant level? The producer could also receive feedback on whether livestock management techniques led to more tender cuts, for example. "It's not all related to safety," Belk said. BSE timeline • Nov. 11, 2004 A no longer productive 12-year-old beef cow is sold at a Texas auction. • Nov. 15, 2004 A cattle buyer ships the cow and others to H&B Packing Co. in Waco, where it arrives dead. The animal is transported across town to Champion Pet Food Co., where its head is severed and shipped to a laboratory at Texas A&M University in College Station. • Nov. 18, 2004 Results of two Bio Rad rapid tests are announced by the U.S. Department of Agriculture as "inconclusive," meaning positive for this error-prone, sensitive method of determining bovine spongiform encephalopthy, or BSE. This prompts rounds of confirmatory tests. • Nov. 23, 2004 Results are negative on brain tissue sent to the National Veterinary Services Laboratories in Ames, Iowa, for two immunohistochemistry (IHC) tests. A positive result four days earlier from an experimental rapid IHC test is not released because researchers believe that it was technically flawed. • June 10, 2005 The USDA announces that a Western blot test ordered by the department's inspector general without Agriculture Secretary Mike Johann's knowledge is positive for BSE. • June 24, 2005 Britain's national veterinary laboratory gets positive results using both IHC and Western blot tests. Johanns also discloses British confirmation that the Ames' experimental IHC test was positive for BSE. • July 11, 2005 Sixty-seven cattle from the infected cow's herd test negative for BSE with the Bio-Rad method. Investigators are still trying to track down the infected cow's last two calves. SOURCE: USDA http://www.dfw.com/mld/dfw/news/12269630.htm Date: July 29, 2005 at 11:16 am PST NEW STRAIN OF TSE USA CATTLE OR JUST INCOMPETENCE IN TESTING??? http://www.usda.gov/wps/portal/usdahome?contentidonly=true&contentid=2005/07 IN CONFIDENCE PERCEPTION OF UNCONVENTENTIONAL SLOW VIRUS DISEASES OF ANIMALS IN THE USA 1985 The Stetsonville outbreak (farmer's name: Brecke). In addition to the Many mink ranches now feed a commerical pelleted diet. Brecke was equipped snip... Dead mink go for rendering but are used only in poultry feed. In the fall at pelting time the skinned carcasses of the mink are placed in Sections from the brains of the two Brecke TME inoculated cattle were Wilbur Clarke (reference the Mission, Texas scrapie transmission I was given confidential access to sections from the Clarke scrapie-cattle Transmission Studies Mule deer transmissions of CWD were by intracerebral inoculation and {the following was written but with a single line marked through it ''first resulted in a more rapidly progressive clinical disease with repeated snip... Appendix 3 VISIT TO USA - DR A E WRATHALL - INFO OH BSE AND SCRAPIE 1. Dr Clark lately of the Scrapie Research Unit, Mission Texas has Expt A Expt B Expt C Diagnosis in A, B, C was by histopath. No reports on SAT were given. 2. Dr Warren Foote indicated success so far in eliminating scrapie in 3. Prof. A Robertson gave a brief account of BSE. The US approach was to 5. Scrapie agent was reported to have been isolated from a solitary 6. A western blotting diagnostic technique (? on PrP) shows some promise. 7. Results of a questionnaire sent to 33 states on the subject of the 6/33 wished to develop it 8/33 had few sheep and were neutral 33 end...TSS full text 33 PAGES ; http://www.bseinquiry.gov.uk/files/yb/1988/10/00001001.pdf http://www.bseinquiry.gov.uk/ 1: J Infect Dis. 1994 Apr;169(4):814-20. Cutlip RC, Miller JM, Race RE, Jenny AL, Katz JB, Lehmkuhl HD, DeBey BM, USDA, Agriculture Research Service, National Animal Disease Center, Ames, IA To determine if sheep scrapie agent(s) in the United States would induce a MeSH Terms: Substances: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ui SNIP... http://www.bseinquiry.gov.uk/files/yb/1988/10/00001001.pdf 12/10/76 snip... A The Present Position with respect to Scrapie Scrapie is a natural disease of sheep and goats. It is a slow The field problem has been reviewed by a MAFF working group It is clear that scrapie in sheep is important commercially and Recently the question has again been brought up as to whether Whether true or not. the hypothesis that these agents might be snip... 76/10.12/4.6 http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf Statement on Texas Cow With Central Nervous System Symptoms FDA, which is responsible for the safety of animal feed, immediately began FDA's investigation showed that the animal in question had already been Cattle with central nervous system symptoms are of particular interest FDA is sending a letter to the firm summarizing its findings and informing To protect the U.S. against BSE, FDA works to keep certain mammalian protein Under the current regulation, the material from this Texas cow is not FDA is committed to protecting the U.S. from BSE and collaborates closely #### http://www.fda.gov/bbs/topics/news/2004/NEW01061.html .1 gram is lethal; THE TEXAS GONZALES/PURINA INCIDENT SHOWED THAT 5.5 GRAMS OF FOR IMMEDIATE RELEASE FDA ANNOUNCES TEST RESULTS FROM TEXAS FEED LOT Today the Food and Drug Administration announced the results of tests FDA has determined that each animal could have consumed, at most and in It is important to note that the prohibited material was domestic in According to Dr. Bernard Schwetz, FDA's Acting Principal Deputy Despite this negligible risk, Purina Mills, Inc., is nonetheless FDA believes that Purina Mills has behaved responsibly by first This episode indicates that the multi-layered safeguard system put into FDA will continue working with USDA as well as State and local officials http://www.fda.gov/bbs/topics/NEWS/2001/NEW00752.html From: TSS (216-119-144-34.ipset24.wt.net) Risk of oral infection with bovine spongiform encephalopathy agent in Corinne Ida Lasmézas, Emmanuel Comoy, Stephen Hawkins, Christian Herzog, Published online January 27, 2005 http://www.thelancet.com/journal/journal.isa It is clear that the designing scientists must also have shared Mr Bradley’s surprise at the results because all the dose levels right down to 1 gram triggered infection. 6. It also appears to me that Mr Bradley’s answer (that it would take less grams) was probably given with the benefit of hindsight; particularly if one considers that later in the same answer Mr Bradley expresses his surprise could take as little of 1 gram of brain to cause BSE by the oral route same species. This information did not become available until the "attack experiment had been completed in 1995/96. This was a titration experiment designed to ascertain the infective dose. A range of dosages was used to that the actual result was within both a lower and an upper limit within the and the designing scientists would not have expected all the dose levels to infection. The dose ranges chosen by the most informed scientists at that ranged from 1 gram to three times one hundred grams. It is clear that the scientists must have also shared Mr Bradley’s surprise at the results dose levels right down to 1 gram triggered infection. Re: BSE .1 GRAM LETHAL NEW STUDY SAYS via W.H.O. Dr Maura Ricketts [BBC radio 4 FARM news] http://www.maddeer.org/audio/BBC4farmingtoday2_1_03.ram http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.htm To cattle: 1 gram of infected brain material (by oral ingestion) http://www.inspection.gc.ca/english/sci/bio/bseesbe.shtml Medical Sciences Cristina Casalone *, Gianluigi Zanusso , Pierluigi Acutis *, Sergio Ferrari *Centro di Referenza Nazionale per le Encefalopatie Animali, Istituto Transmissible spongiform encephalopathies (TSEs), or prion diseases, are ---------------------------------------------------------------------------- C.C. and G.Z. contributed equally to this work. ||To whom correspondence should be addressed. E-mail: salvatore.monaco@mail.univr.it . www.pnas.org/cgi/doi/10.1073/pnas.0305777101 AS of March 31, 2005, there were 70 scrapie infected source flocks (Figure FULL TEXT ; http://www.aphis.usda.gov/vs/nahps/scrapie/monthly_report/monthly-report.htm WITH the MAY report, a scrapie case documented in a GOAT IN THE USA...TSS SCRAPIE USA UPDATE MAY 2005 AS of March 31, 2005, there were 70 scrapie infected source flocks (Figure FULL TEXT ; http://www.aphis.usda.gov/vs/nahps/scrapie/monthly_report/monthly-report.htm http://www.aphis.usda.gov/vs/nahps/cwd/cwd-distribution.html 5/9/2005: Negative Results for Chronic Wasting Disease in Captive Herd Learn 5/4/2005: DEC Announces Sampling Results for Chronic Wasting Disease Learn 4/29/2005: DEC Issues Emergency Regulations in Response to Discovery of 4/27/2005: Chronic Wasting Disease Found in Oneida County Deer. Learn More. 4/21/2005: DEC Releases Results of Tests for Chronic Wasting Disease. Learn 4/13/2005: DEC to Test For Chronic Wasting Disease in Hamilton County. Learn 4/8/2005: Chronic Wasting Disease Update: Test Results Reveal Three 4/5/2005: Chronic Wasting Disease Update. Learn More. 4/2/2005: Second Case of CWD Found in Oneida County Deer. Learn More. 3/31/2005: Positive Case of CWD Found in Oneida County Deer. Learn More. Transcript from March 31 Press Conference Regarding First Case of CWD in If you have difficulty opening the PDF files, please contact the Department i guess WE will never know due to the CJD Foundation INC. and there PHONE THEY don't want the rest of the world knowing any potential route and source JUST SAY NO TO ORAL CJD QUESTIONNAIRE, demand a written one with a copy TSS https://web01.aphis.usda.gov/regpublic.nsf/0/eff9eff1f7c5cf2b87256ecf000df08 Docket No. 03-080-1 -- USDA ISSUES PROPOSED RULE TO ALLOW LIVE ANIMAL http://www.fda.gov/ohrms/dockets/dockets/03n0312/03N-0312_emc-000001.txt Docket Management Docket: 02N-0273 - Substances Prohibited From Use in Animal Food or Feed; Animal Proteins Prohibited in Ruminant Feed Comment Number: EC -10 Accepted - Volume 2 PART 2 PDF]Freas, William TSS SUBMISSION File Format: PDF/Adobe Acrobat - Page 1. J Freas, William From: Sent: To: Subject: Terry S. Singeltary Sr. [flounder@wt.net] Monday, January 08,200l 3:03 PM freas ... http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_09.pdf Asante/Collinge et al, that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest _sporadic_ CJD; http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.htm Docket Management Docket: 96N-0417 - Current Good Manufacturing Practice http://www.fda.gov/ohrms/dockets/dailys/03/Mar03/031403/96N-0417-EC-2.htm Docket No. 2003N-0312 Animal Feed Safety System [TSS SUBMISSION http://www.fda.gov/ohrms/dockets/dockets/03n0312/03N-0312_emc-000001.txt # Docket No: 02-088-1 RE-Agricultural Bioterrorism Protection Act of Docket Management Docket: 02N-0276 - Bioterrorism Preparedness; Registration of Food http://www.fda.gov/ohrms/dockets/dockets/02n0276/02N-0276-EC-254.htm OTC External Analgesic Drug Products, ... EMC 7, Terry S. Singeltary Sr. www.fda.gov/ohrms/dockets/dailys/03/oct03/100203/100203.htm DOCKETS ENTERED on 2/5/03. ... EMC 4 Terry S. Singeltary Sr. Vol#: 2. 03N-0009 Federal Preemption of State & Local Medical Device Requireme. ... Docket: 02N-0370 - Neurological Devices; Classification of Human Dura Mater Comment Number: EC -1 Accepted - Volume 1 ... 00D-1662 Use of Xenotransplantation Products in Humans. http://www.fda.gov/ohrms/dockets/dailys/03/Jun03/060903/060903.htm 2003D-0186 01N-0423 Substances Prohibited from use in animal food/Feed Ruminant APE 5 National Renderers Association, Inc. Vol#: 2 APE 6 Animal Protein Producers Industry Vol#: 2 APE 7 Darling International Inc. Vol#: 2 EMC 1 Terry S. Singeltary Sr. Vol#: 3 http://www.fda.gov/ohrms/dockets/dailys/01/Oct01/101501/101501.htm Send Post-Publication Peer Review to journal: disease in the United States 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? LANCET INFECTIOUS DISEASE JOURNAL My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem. largely unsatisfied after being told that a close relative died from a rapidly progressive dementia compatible with spontaneous Creutzfeldt-Jakob disease (CJD). So he decided to gather hundreds of documents on transmissible spongiform encephalopathies (TSE) and realised that if Britons could get variant CJD from bovine spongiform encephalopathy (BSE), Americans might get a similar disorder from chronic wasting disease (CWD)the relative of mad cow disease seen among deer and elk in the USA. Although his feverish search did not lead him to the smoking gun linking CWD to a similar disease in North American people, it did uncover a largely disappointing situation. occurrence of CJD and CWD in the USA. Only a few states have made CJD reportable. Human and animal TSEs should be reportable nationwide and internationally, he complained in a letter to the Journal of the American Medical Association (JAMA 2003; 285: 733). I hope that the CDC does not continue to expect us to still believe that the 85% plus of all CJD cases which are sporadic are all spontaneous, without route or source. region in Colorado. But since early 2002, it has been reported in other areas, including Wisconsin, South Dakota, and the Canadian province of Saskatchewan. Indeed, the occurrence of CWD in states that were not endemic previously increased concern about a widespread outbreak and possible transmission to people and cattle. transmitted to cattle by intracerebral inoculation and that it can cross the mucous membranes of the digestive tract to initiate infection in lymphoid tissue before invasion of the central nervous system. Yet the plausibility of CWD spreading to people has remained elusive. is only reported in those areas known to be endemic foci of CWD. Moreover, US authorities have been criticised for not having performed enough prionic tests in farm deer and elk. issued a directive to state public-health and agriculture officials prohibiting material from CWD-positive animals from being used as an ingredient in feed for any animal species, epidemiological control and research in the USA has been quite different from the situation in the UK and Europe regarding BSE. teeth, Singeltary argues. You get it when they want you to have it, and only what they want you to have. University of Michigan (Ann Arbor, MI, USA), says that current surveillance of prion disease in people in the USA is inadequate to detect whether CWD is occurring in human beings; adding that, the cases that we know about are reassuring, because they do not suggest the appearance of a new variant of CJD in the USA or atypical features in patients that might be exposed to CWD. However, until we establish a system that identifies and analyses a high proportion of suspected prion disease cases we will not know for sure. The USA should develop a system modelled on that established in the UK, he points out. Ali Samii, a neurologist at Seattle VA Medical Center who recently reported the cases of three hunterstwo of whom were friendswho died from pathologically confirmed CJD, says that at present there are insufficient data to claim transmission of CWD into humans; adding that [only] by asking [the questions of venison consumption and deer/elk hunting] in every case can we collect suspect cases and look into the plausibility of transmission further. Samii argues that by making both doctors and hunters more aware of the possibility of prions spreading through eating venison, doctors treating hunters with dementia can consider a possible prion disease, and doctors treating CJD patients will know to ask whether they ate venison. the [Samii] cases because there is no evidence that the men ate CWD-infected meat. He notes that although the likelihood of CWD jumping the species barrier to infect humans cannot be ruled out 100% and that [we] cannot be 100% sure that CWD does not exist in humans& the data seeking evidence of CWD transmission to humans have been very limited. Association (JAMA 2003; 285: 733). I hope that the CDC does not continue to expect us to still believe that the 85% plus of all CJD cases which are sporadic are all spontaneous, without route or source.<<< actually, that quote was from a more recent article in the Journal of Neurology (see below), not the JAMA article... Full Text Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734. http://jama.ama-assn.org/cgi/content/full/285/6/733?maxtoshow=&HITS=10&hits= BRITISH MEDICAL JOURNAL SOMETHING TO CHEW ON BMJ http://www.bmj.com/cgi/eletters/319/7220/1312/b#EL2 BMJ http://www.bmj.com/cgi/eletters/320/7226/8/b#EL1 THE PATHOLOGICAL PROTEIN BY Philip Yam Yam Philip Yam News Editor Scientific American www.sciam.com IN light of Asante/Collinge et al findings that BSE transmission to the -------- Original Message -------- Subject: re-BSE prions propagate as either variant CJD-like or sporadic CJD Date: Thu, 28 Nov 2002 10:23:43 -0000 From: "Asante, Emmanuel A" To: Dear Terry, I have been asked by Professor Collinge to respond to your request. I am a Senior Scientist in the MRC Prion Unit and the lead author on the paper. I have attached a pdf copy of the paper for your attention. Thank you for your interest in the paper. In respect of your first question, the simple answer is, yes. As you will find in the paper, we have managed to associate the alternate phenotype to type 2 PrPSc, the commonest sporadic CJD. It is too early to be able to claim any further sub-classification in respect of Heidenhain variant CJD or Vicky Rimmer's version. It will take further studies, which are on-going, to establish if there are sub-types to our initial finding which we are now reporting. The main point of the paper is that, as well as leading to the expected new variant CJD phenotype, BSE transmission to the 129-methionine genotype can lead to an alternate phenotype which is indistinguishable from type 2 PrPSc. I hope reading the paper will enlighten you more on the subject. If I can be of any further assistance please to not hesitate to ask. Best wishes. Emmanuel Asante <> ____________________________________ Dr. Emmanuel A Asante MRC Prion Unit & Neurogenetics Dept. Imperial College School of Medicine (St. Mary's) Norfolk Place, LONDON W2 1PG Tel: +44 (0)20 7594 3794 Fax: +44 (0)20 7706 3272 email: e.asante@ic.ac.uk (until 9/12/02) New e-mail: e.asante@prion.ucl.ac.uk (active from now) ____________________________________ snip... full text ; http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.htm Creutzfeldt-Jakob disease THE findings from Corinne Ida Lasmézas*, [dagger] , Jean-Guy Fournier*, Hermann Boe*, Domíníque Marcé*, François Lamoury*, Nicolas Kopp [Dagger ] , Jean-Jacques Hauw§, James Ironside¶, Moira Bruce [||] , Dominique Dormont*, and Jean-Philippe Deslys* et al, that The agent responsible http://www.pnas.org/cgi/content/full/041490898v1 Characterization of two distinct prion strains http://vir.sgmjournals.org/cgi/content/abstract/85/8/2471 ALL human TSEs must be made reportable Nationally and Internationally, OF #################### https://lists.aegee.org/bse-l.html ####################
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