KS-73 (Apr 23, 2008)
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Although the United States and Korea have reached general agreement on the resumption of export of U.S. beef and beef products to Korea, FSIS officials should not issue export certificates for beef and beef products intended for export to Korea at this time. Additional information about the specific conditions, including eligible product, plant approval, and certification requirements are being developed and will be made available as soon as possible. Establishment management may contact AMS at 540-361-7640 for information about AMS program requirements.*
please see the 'meat of the issue' on import health requirements for the U.S. beef and beef products to Korea, or the lack of, as follows ;
IMPORT HEALTH REQUIREMENTS FOR U.S. BEEF AND BEEF PRODUCTS
Import Health Requirements for U.S. Beef and Beef Products
The following import health requirements shall be applied to beef and beef products exported from the United States of America ("United States") to the Republic of Korea ("Korea").
1. Definitions for the purpose of these health requirements are as follows:
(1) "Beef or beef products" includes all edible parts of cattle less than 30 months of age at the time of slaughter and products derived from all edible parts of cattle less than 30 months of age at the time of slaughter as described in the U.S. Federal Meat Inspection Act. However, "beef or beef products" excludes specified risk materials (SRMs); all mechanically recovered meat (MRM)/mechanically separated meat (MSM); and advanced meat recovery product (AMR) from the skull and vertebral column of cattle 30 months of age and over at the time of slaughter. AMR that is free of SRMs and central nervous system tissues (CNS) is allowed. Ground meat, processed products and beef extracts may contain AMR but excludes specified risk materials (SRMs) and all MRM/MSM.
(2) "BSE" means Bovine Spongiform Encephalopathy.
(3) "Cattle" means domesticated bovine animals (Bos taurus and Bos indicus) born and raised in the United States, legally imported into the United States from a country deemed eligible by the Korean government to export beef or beef products to Korea, or raised in the United States for at least 100 days prior to slaughter.
(4) "Food-safety hazard" means any biological, chemical, or physical property that may cause food to be unsafe for human consumption.
(5) "Lot" means a quantity of beef or beef products identified on a single export certificate from one meat establishment, and consists of the same process category and product standard of identity (sub-category).
(6) "Meat establishment" includes any slaughterhouse, processing plant, and storage facility for beef or beef products that operates under U.S. Department of Agriculture (USDA) inspection.
(7) "Non-compliance" means an inconsistency with this protocol that does not constitute a food-safety hazard.
(8) "Serious non-compliance" means a food-safety hazard in a shipped product or a food-safety hazard found during a system audit.
(9) "Specified risk materials" (SRMs) means:
(a) tonsils and distal ileum from cattle of all ages; and
(b) brain, eyes, spinal cord, skull, dorsal root ganglia (DRG) and vertebral column (excluding vertebrae of the tail, transverse processes and spinous processes of the cervical, thoracic and lumbar vertebrae, median crest and wings of the sacrum) from cattle 30 months of age and over at the time of slaughter.(10) "United States" (U.S.) means the fifty states and the District of Columbia.
2. Prior to the loading of the beef or beef products:
(a) the United States has been free of foot-and-mouth disease for the past 12 months and has been free of rinderpest, contagious bovine pleuropneumonia, lumpy skin disease and Rift Valley fever for the past 24 months; and
(b) Vaccination has not been carried out against the aforementioned diseases.
Notwithstanding the above, in the event the Korean government recognizes that effective stamping-out policies ar e in place for the specific disease in the United States, including emergency vaccination if carried out, the required period for recognizing the United States as being free of that disease may be shortened in accordance with World Organization for Animal Health (OlE) guidelines after Korea conducts a risk analysis.
3. In the event a disease set out in item 2 occurs in the United States, the U.S. government shall immediately suspend the issuance of export certificates for all beef and beef products to Korea that do not meet the requirements of item 2.
4. The U.S. government, in accordance with U.S. regulations, continuously maintains measures that meet or exceed OlE guidelines for controlled-risk status to effectively detect and prevent the introduction and spread of BSE. The U.S. government will provide notice to the World Trade Organization (WTO) -according to its WTO commitments -and inform Korea regarding the repeal or amendment of any BSE-related measure.
5. In the event (an) additional case(s) of BSE occur(s) in the United States, the U.S. government shall immediately conduct a thorough epidemiological investigation and inform the Korean government of the results of the investigation. The U.S. government will consult with the Korean government about the findings of the investigation. The Korean government will suspend the importation of beef and beef products if the additional case(s) results in the OlE recognizing an adverse change in the classification of the U.S. BSE status.
Requirements for Meat Establishments
6. Any meat establishment in the United States that operates under USDA inspection is eligible to produce beef or beef products for Korea. The establishment should be notified to the Korean government in advance.
7. The U.S. government will maintain a regular monitoring and aUditing program for meat establishments that produce beef or beef products for export to Korea to ensure they comply with the relevant provisions of these health requirements and U.S. regulations. In the event of a serious non-compliance, the Food Safely and Inspection Service (FSIS) personnel would issue a Noncompliance Record and would immediately control the non-compliant product If the process that resulted in the non-compliant product is on-going, FSIS would immediately stop the process until it determines tha t appropriate corrective and preventative measures have been taken. Only when FSIS determines that corrective actions are adequate will production be allowed to resume. The U.S. government will inform the Korean government if an establishment is suspended and when corrective action has been taken.
8. The Korean government may conduct on-site aUdits of a representative sample of the meat establishments that export beef or beef products to Korea. When a serious non-compliance with these health requirements has been found as a result of the on-site audit, the Korean government will inform the results to the U.S. government, and the U.S. government shall take appropriate measures and inform the Korean government of the measures taken.
9. The U.S. government shall verify that a suspended meat establishment has determined and implemented appropriate corrective and preventative measures before lifting the suspension described in item 7, item B or item 24. The U.S. government shall inform the Korean government of the corrective action the meat establishment has taken and of the date the meat establishmenfs suspension is lifted.
Requirements for Beef and Beef Products
10. The beef or beef products were derived from cattle born and raised in the United States, from cattle legally imported into the United States from a country deemed eligible by the Korean government to export beef or beef products to Korea, or from cattle raised in the United States for at least 100 days prior to slaughter.
11. Cattle for producing beef or beef products for export were not suspect or confirmed BSE cases; confirmed progenies of BSE cases; or confirmed cohorts of BSE cases, as defined in the Terrestrial Animal Health Code adopted by the OlE.
12. Meat establishments that produce beef or beef products shall maintain a program for the hygienic removal of SRMs.
13. For the purpose of SRM removal, the age of cattle at the time of slaughter was verified by documentation which identifies the age or by dentition.
14. The meat establishments maintain purchase records indicating the facility from which the animals were purchased for slaughter. Records may be disposed of two years after the date of purchase.
15. The beef or beef products were derived from cattle that were slaughtered in meat establishments (slaughterhouses) certified by th e U.S. government as eligible to export beef and beef products to Korea and that passed ante-mortem and post-mortem inspection conducted by USDA inspection personnel under the supervision of the resident USDA veterinarian.
16. The beef or beef products were derived from cattle that were not subjected to a stunning process, prior to slaughter, with a device injecting compressed air or gas into the cranial cavity, or to a pithing process.
17. The beef or beef products were produced and handled in a manner as to prevent contamination from SRMs or from MSM from the skull and vertebral column of cattle 30 months of age and over, in accordance with FSIS regulations.
18. Residues (radioactivity, synthetic antibiotic sUbstances, antibiotic substances, heavy metals, pesticides, hormones, etc.) posing a public health hazard and pathogenic microorganisms in the beef and beef products shall not exceed the tolerance levels established by the Korean government. The beef and beef products may be treated with ionizing radiation, ultraviolet rays, and tenderizers in accordance with Korean regUlations.
19. Sanitary packaging material was used to package the beef or beef products.
20. The processing, storage and transportation of the beef an d beef products were handled in such a manner as to prevent contamination by communicable animal disease pathogens.
21. Refrigerated or cold storage rooms on a ship (aircraft) or container that transports the beef and beef products were sealed by using the seal of the U.S. government or a U.S. government-recognized seal and then certified by a U.S. government veterinarian.
22. Beef and beef products qualify for import quarantine inspection if accompanied by the Export Certificate of Wholesomeness and the Certificate for Export of Beef and Beef Products to the Republic of Korea (ROK) issued by the veterinary authority of the U.S. government, which include the following information to be submitted to the quarantine authority of the Korean government:
(1) Information responsive to items 2,10 and 15-20 above;
(2) Name of the product (including species), number of packages and weight (net weight) listed by each final processing plant;
(3) Names, addresses and establishment numbers of the slaughterhouse, meat processing plant and storage facility;
(4) Slaughtering period and/or processing period (dd/mmJyy-dd/mm/yy);
(5) Names and addresses of the consignor and the consignee;
(6) Date the export certificate was issued and the name and signature of the issuer; and
(7) Container number and seal number.
Import Quarantine Inspection and Regulatory Action
23. If the Korean government detects a food-safety hazard in a lot during the quarantine inspection process, it may reject the lot. The Korean government shall notify and consult with the U.S. government regarding the matter and may request corrective action if appropriate. If an SRM is found, FSIS will conduct an investigation to determine the cause of the problem. Product pro duced by the pertinent me at establishment shall continue to be eligible for import quarantine inspection. However, the Korean government will increase the rate of inspection of subsequent beef and beef products from the meat establishment. After the Korean government inspects five lots of equal or greater quantity of the same product wnhout finding a food-safety hazard, the Korean government shall apply its standard inspection procedures and rates.
24. If the Korean government observes at least two incidents of food-safety hazards involving separate lots from the same meat establishment, the meat establishment may be suspended until corrective action has been taken. Beef and beef products of the meat establishment that were certified prior to the date of suspension shall continue to be eligible for import quarantine inspection. An establishment shall remain suspended until the U.S. government verifies to the Korean government that corrective actions have been completed. The U.S. government shall inform the Korean government of the meat establishment's corrective action and of the date the meat establishmenfs suspension is lifted. The Korean government may include an on-site audit of the establishment during its next system audit in the United States.
25. The Korean government or the U.S. government may request consultations with the other concerning any matter regarding the interpretation or application of these import health requirements. Unless otherwise agreed, the consultations shall be held within seven days of the request in the territory of the government that receives the request.
1. This notice will go into effect on the date of its notification.
2. When the United States publicly announces its enhanced feed ban, Article 1(1) shall be modified to read as follows: "beef or beef products" includes all edible parts of cattle and products derived from all edible parts of cattle as described in the U.S. Federal Meat Inspection Act. However, "beef or beef products" excludes specified risk materials (SRMs); all mechanically recovered meat (MRM) /mechanically separated meat (MSM); and advanced meat recovery product (AMR) from the skull and vertebral column of cattle 30 months of age and over at the time of slaughter. AMR that is free of SRMs and central nervous system tissues (CNS) is allowed. Ground meat, processed products and beef extracts may contain AMR but excludes specified risk materials (SRMs) and all MRM/MSM.
3. During the first 90 days following the effective date of these import health requirements, Korea may audit and/or reject U.S. decisions regarding the listing of new plants or re-Iisting of previously de-listed plants.
4. During the first 180 days following the effective date of these import health requirements, exports of T-bone and Porterhouse steaks will be accompanied by some notation on the box that confirms for Korean officials that these cuts of beef come from cattle under 30 months of age. The Korean government and the U.S. government agree to have consultations upon the completion of the 180 day period with a view to addressing concerns after reviewing the notation's effect on beef trade and its inspection.
On behalf of Korea
A. Ellen Terpstra
Deputy Under Secretary
On behalf of the United States
Why Americans, As Well as Koreans, Should Be Worried About Mad Cow Tainted USA Beef
By Terry S. Singeltary Sr. May 15, 2008
Straight to the Source
Web Note: This is an important commentary by Terry S. Singeltary Sr., on a recent Business Week story on the controversy in South Korea over their government's lifting on the ban on conventional (non-organic) beef, despite the fact that the USDA is still allowing slaughterhouse waste and blood and manure to be fed to cows, and refusing to test all cows at slaughter. See the Mad Cow section of the OCA website for in-depth information. Terry is a regular blogger on the OCA website on Mad Cow issues.
One Korean official says the probability of a human being catching a mad cow disease by eating U.S. beef is like the one of a golf player scoring a hole-in-one and then being killed by lightning.
this is typical BSe. you here industry groups comment 'your more likely to get hit by a car than die from CJD'. well, maybe so, but my mother and many more did not die from getting hit by a car, they died from CJD, my mothers being the hvCJD (confirmed), and my neighbors mother died from CJD (confirmed). the UKBSEnvCJD _only_ theory is incorrect. there are more strains of mad cow than the UK BSE in beef to nvCJD in humans in the UK. The deception by the USDA, FDA, and the Bush administration about mad cow disease, CJD, and all Transmissible Spongiform Encephalopathy over the past 8 years have been outrageous, to a point of being criminal. I am vested in nothing, but the truth.
snip...see full text ;
Tuesday, May 13, 2008
Concerned Americans against Mad Cow Disease STATEMENT OF SOLIDARITY with Koreans May 13, 2008
BSE YOUNGEST AGE STATISTICS UNDER 30 MONTHS
Tuesday, May 27, 2008
FDA BSE/Ruminant Feed Inspections Firms Inventory Report Texas Legend Ranch OAI 05/10/2008
"South Korea may demand revision of US beef import pact"
"The agreement, struck last month, has been widely criticized as making too"
"many concessions to the United States"
THE PEOPLE of Korea _should_ be mad about the importing of USA beef into their Country. can you believe these regulations? even IF a BSE case(s) are documented in the USA, the people of Korea still cannot suspend the importing of U.S. beef, NO matter how many more mad cows the USA finds, until a thorough epidemiological investigation is finished. please remember, it took over a year and literally an act of congress to confirm the atypical mad cow in Texas before they finally finish that epidemiological investigation, and even after all that, the Koreans still cannot ban USA beef, until the OIE recognizes an adverse change in the classification of the U.S. BSE status. Considering the USDA and the OIE collaborated to seal the deal of the BSE MRR policy (the legal trading of all strains of TSE globally, just for commodities and futures sake, human health was not even considered), I doubt the OIE would ever change the BSE status for the USA, no matter how many more mad cows are found. It's all about money folks.
WE are talking years now, before the Koreans could ever suspend USA beef due to a BSE case(s) ever being documented in the USA, due to these stupid regulations. This is nothing more than FORCE FEEDING KOREA USDA MAD COW BEEF, i.e. all for a dollar, to hell with human health on a disease with an incubation period of years if not a decade or more.
Please remember, the last two mad cows documented in the USA i.e. Alabama and Texas, both were of the 'atypical' BSE strain, and immediately after that, the USDA shut down the testing from 470,000 to 40,000 in the U.S. in 2007 out of about 35 million cattle slaughtered. also, science is showing that some of these atypical cases are more virulent to humans than the typical UK BSE strain ;
***Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.***
Progress Report from the National Prion Disease Pathology Surveillance Center
An Update from Stephen M. Sergay, MB, BCh & Pierluigi Gambetti, MD
April 3, 2008
IF BSE is not in the USA (just not documented for many different reasons), and only atypical BSE is in the USA (plus CWD, plus, many strains of Scrapie, and Now the Nor-98 documented in 5 different states, plus TME, then why would human mad cow in the USA look like the UK nvCJD from UK BSE cows ? it was shown long ago in studies at Mission Texas that experimental transmission of USA Scrapie to USA Bovine, DID NOT LOOK LIKE UK BSE. so again, in short, why would human mad cow in the USA look like human mad cow in the UK i.e. the (nvCJD). however, I believe that BSE has been in the USA untested and undocumented for years. why on earth then does the USDA refuse to allow creekstone or anyone else test their product? simple, if you don't look/test, you don't find.
ONE only has to read how the USDA et al have legally blocked, blundered, botched, mismanaged, bungled, floundered, and flat out manipulated, the testing in the infamous June 2004 enhanced cover-up program for mad cow surveillance and testing. I mean, I am not really to hip on THE INDUSTRY, testing for mad cow disease, and what that program might consist of, but anything is better than nothing at all. ...
BSE BASE MAD COW TESTING TEXAS, USA, AND CANADA
MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE
In 2007, in one weekly enforcement report, the fda recalled 10,000,000+ pounds of BANNED MAD COW FEED, 'in commerce', and i can tell you that most of it was fed out ;
10,000,000+ LBS. of PROHIBITED BANNED MAD COW FEED I.E. MBM IN COMMERCE USA 2007
Date: March 21, 2007 at 2:27 pm PST REASON Blood meal used to make cattle feed was recalled because it was cross-contaminated with prohibited bovine meat and bone meal that had been manufactured on common equipment and labeling did not bear cautionary BSE statement. VOLUME OF PRODUCT IN COMMERCE 42,090 lbs. DISTRIBUTION WI
REASON Products manufactured from bulk feed containing blood meal that was cross contaminated with prohibited meat and bone meal and the labeling did not bear cautionary BSE statement. VOLUME OF PRODUCT IN COMMERCE 9,997,976 lbs. DISTRIBUTION ID and NV
END OF ENFORCEMENT REPORT FOR MARCH 21, 2007
Subject: MAD COW FEED RECALL USA SEPT 6, 2006 1961.72 TONS IN COMMERCE AL, TN, AND WV Date: September 6, 2006 at 7:58 am PST
snip... see listings and references of enormous amounts of banned mad cow protein 'in commerce' in 2006 and 2005 ;
see full text ;
Friday, April 25, 2008
Substances Prohibited From Use in Animal Food or Feed [Docket No. 2002N-0273] (Formerly Docket No. 02N-0273) RIN 0910-AF46
SPECIFIED RISK MATERIALS
SRM MAD COW RECALL 406 THOUSAND POUNDS CATTLE HEADS WITH TONSILS KANSAS
Sunday, April 20, 2008 Progress Report from the National Prion Disease Pathology Surveillance Center April 3, 2008
Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.
see full text ;
CJD TEXAS (cjd clusters)
CJD USA RISING
The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.
Communicated by: Terry S. Singeltary Sr.
[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP]
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.
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:
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  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?
THE PATHOLOGICAL PROTEIN
Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9
BY Philip Yam
CHAPTER 14 LAYING ODDS
Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.
Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.
Terry S. Singeltary, Sr Bacliff, Tex
1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. FREE FULL TEXT
2 January 2000 British Medical Journal U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well
15 November 1999 British Medical Journal vCJD in the USA * BSE in U.S.
Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME. Since previous incidences of TME were associated with common or shared feeding practices, we obtained a careful history of feed ingredients used over the past 12-18 months. The rancher was a "dead stock" feeder using mostly (>95%) downer or dead dairy cattle and a few horses. Sheep had never been fed.
USDA CERTIFIED DEAD STOCK DOWNER COW SCHOOL LUNCH PROGRAM for children
please note, dead stock downer cattle i.e. non-ambulatory, are the most likely to have mad cow disease.
see full text 18 pages ;
Thu Dec 6, 2007 11:38
FDA IN CRISIS MODE, AMERICAN LIVES AT RISK
FDA SCIENCE AND MISSION AT RISK
[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 9/13/2005
3. Prof. A. Robertson gave a brief account of BSE. The US approach was to accord it a _very low profile indeed_. Dr. A Thiermann showed the picture in the ''Independent'' with cattle being incinerated and thought this was a fanatical incident to be _avoided_ in the US _at all costs_ $
Attachment to Singeltary comment
January 28, 2007
I would kindly like to submit the following to ;
BSE; MRR; IMPORTATION OF LIVE BOVINES AND PRODUCTS DERIVED FROM BOVINES [Docket No. APHIS-2006-0041] RIN 0579-AC01
[Federal Register: January 9, 2007 (Volume 72, Number 5)] [Proposed Rules] [Page 1101-1129] From the Federal Register Online via GPO Access [wais.access.gpo.gov] [DOCID:fr09ja07-21]
BSE; MRR; IMPORTATION OF LIVE BOVINES AND PRODUCTS DERIVED FROM BOVINES [Docket No. APHIS-2006-0041] RIN 0579-AC01 Date: January 9, 2007 at 9:08 am PST
[Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)
APHIS-2006-0041-0006 TSE advisory committee for the meeting December 15, 2006
Tuesday, June 3, 2008 SCRAPIE USA UPDATE JUNE 2008 NOR-98 REPORTED PA
NOR-98 ATYPICAL SCRAPIE 5 cases documented in USA in 5 different states USA 007
CHRONIC WASTING DISEASE
8. Human susceptibility to CWD
Millions of North Americans hunt deer and elk (U.S. Department of the Interior, Census Bureau), and there is no doubt that people have been exposed to CWD through venison consumption, particularly in light of recent data showing CWD prions in muscle . Human susceptibility to CWD or to other newly emerging animal TSE [9, 14] is still unclear, although we can be somewhat reassured in that there have been no large scale outbreaks of human TSE cases in Colorado and Wyoming, where CWD has existed for decades . Up until approximately 10 years ago, autopsies were not performed on suspect human TSE cases in many states due to biosafety concerns, therefore the diagnosis of potential new TSE strains has been hampered. This indicates that clinical TSE diagnoses in humans were not confirmed, nor was any strain typing done to look for the appearance of potentially subtle or unusual pathological or biochemical phenotypes of a new TSE strain. Fortunately, the autopsy rate for suspect cases is improving. At the National Prion Disease Pathology Surveillance Center at Case Western Reserve University (Cleveland, Ohio), Creutzfeldt-Jakob disease (CJD) suspect cases are studied and classified by CJD subtype. Thus far,
*** twenty-seven CJD patients who regularly consumed venison were reported to the Surveillance Center***,
however there have been no unusual or novel prion subtypes that might indicate the appearance of a new prion strain [7, 41]. Other indirect studies of human susceptibility to CWD also suggest that the risk is low. In biochemical conversion studies, Raymond et al.  showed that the efficiency of CWD to convert recombinant human PrP into amyloid fibrils was low, but similar to that of both BSE and scrapie fibrils to do the same. These results suggest that there is a molecular incompatibility in the conversion of human PrPC by CWD, sheep scrapie, or BSE, and that cross species infections in humans may be rare events. To determine whether common PrPSc strain features may link CWD and CJD, histopathology and the PrPSc biochemical characteristics from deer and elk were compared with that of humans with sporadic CJD (sCJD) cases that are methionine homozygous at codon 129 of the Prnp gene by Xie et al. , although strain features including histologic profile, target organs, and glycoform patterns will not necessarily remain the same upon crossing species barriers [6, 5, 8, 57]. The PrPSc form is cleaved by proteinase-K (PK) at different sites depending on the conformation of the protein and may aid determination of whether the PrPSc conformation is similar. By western blot (SDS-PAGE) of elk CWD, the unglycosylated PK-resistant PrPSc migrated at 21 kDa, similar to sCJD (MM1 subtype) and the PK cleavage site was the same, occurring at residues 78 and 82 as assessed by N-terminal sequencing. Conformational stability was evaluated by measuring the PrPSc stability under partially denaturing conditions and also showed no significant difference between elk CWD and sCJD MM1 PrPSc. However, elk CWD and human sCJD MM1 strains exhibited distinct glycoform patterns by two dimensional gel electrophoresis, suggesting that the strains differed. Future studies may utilize luminescent conjugated polymers, which were recently shown to distinguish naturally- and experimentally-derived prion strains . To study elk-human prion species barriers, Kong et al. inoculated elk CWD into transgenic mice expressing either human PrP or elk PrP. Whereas the elk PrP expressing mice developed disease after only 118-142 days post-inoculation, human PrP expressing mice (129M) did not develop any features of TSE after more than 657 or more than 756 days . In accordance with these results, Tamgüney et al. also reported that human PrP overexpressing mice were not susceptible to 9 CWD isolates from mule deer, white-tailed deer, and elk . However, mice have a limited lifespan and further passages may be necessary to detect low levels of prion infectivity that may be present subclinically. Although indirect evidence is accumulating that there may be a robust species barrier for CWD transmission to humans, one report indicates nonhuman primate susceptibility to CWD. Intracerebral inoculation of squirrel monkeys (Saimiri sciureus) demonstrated a positive CWD transmission . Among non-human primates, however, the Prnp sequence of the new world monkeys are the most distant from humans , and therefore may not indicate that human prion conversion would occur by CWD.
11. Disease control challenges posed by CWD
Evidence is building that indicates efficient horizontal transmission occurs in CWD, indeed a complicating aspect in disease control . Potential transmission mechanisms range from spread via direct contact among animals to environmental exposure through grazing in areas contaminated by prion-infected secretions, excretions (saliva, urine, feces), tissues (placenta), or decomposed carcasses. Recently, in a breakthrough finding, saliva from CWD infected deer was shown to transmit prion disease . An additional experiment by Miller and colleagues showed that CWD-infected carcasses allowed to decay naturally in confined pastures can lead to CWD infections in captive deer, demonstrating the potential for environmental contamination to spread infection . Modelling studies have provided further
support that environmental contamination is likely playing a significant role in transmitting CWD [56, 53]. Additionally, infectious prions have been demonstrated to bind soil particles and remain infectious to animals by both intracerebral and oral exposure routes [38, 37]. Prion infectivity has been recovered from soil more than two years after experimental exposure to prions, suggesting the soil may serve as a reservoir for CWD prions . Taken together, these results indicate that there may even be multiple sources for CWD exposure, perhaps through direct contact and environmental routes. Significant challenges to CWD eradication exist in free-ranging cervids. Infected deer and elk range over a broad geographic region, and even previously surmised geographic barriers such as the Continental Divide have proven passable by infected animals. Ridding the environment of CWD-contaminated soil or even CWD-infected carcasses is not possible. Moreover, the available ante-mortem diagnostic tests for surveillance are laborious and impractical for large numbers of free-ranging animals [74, 88, 95]. Therefore for a wildlife manager, this disease is costly to survey and difficult to control.
CWD in cervids is efficiently transmitted, likely more than any other TSE in animals or humans. Therefore, it is unlikely that this TSE can be eradicated, but perhaps through an improved understanding of transmission routes, biological factors influencing pathogenesis, and the molecular basis of CWD prion conversion, a targeted strategy for interrupting disease spread may be developed.
I thank Drs. Michael Miller, Jason Bartz and Mathias Heikenwalder for critical review of the manuscript.
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SEE FULL TEXT CWD ;
Thursday, April 03, 2008
A prion disease of cervids: Chronic wasting disease 2008
Transmissible Mink Encephalopathy TME
Tuesday, April 29, 2008
Interference at the EPA - Science and Politics at the U.S. Environmental Protection Agency
please see full text ;
Greetings again Honorable President Young-hui, KIWA (Koreatown Immigrant Workers Alliance), and all Korea,
You might want to communicate these factors of iCJD to your fellow Koreans in the USA who are having medical, surgical and or dental work done. With the many different animal TSEs in the USA of typical and atypical phenotype, the consumption thereof of these TSE infected animals, and the following medical, surgical, and or dental work done on any individual consuming such tainted product, the fact that Koreans are more susceptible to contracting a TSE, one must ponder all these factors ;
Polymorphisms of the prion protein gene (PRNP) in a Korean population Journal Journal of Human Genetics Publisher Springer Japan ISSN 1434-5161 (Print) 1435-232X (Online) Issue Volume 49, Number 6 / June, 2004 Category Short Communication DOI 10.1007/s10038-004-0150-7 Pages 319-324 Subject Collection Biomedical and Life Sciences SpringerLink Date Monday, May 17, 2004
Byung-Hoon Jeong1, Jae-Hwan Nam2, Yun-Jung Lee1, Kyung-Hee Lee1, Myoung-Kuk Jang1, Richard I. Carp3, Ho-Dong Lee2, Young-Ran Ju2, Sangmee Ahn Jo2, Keun-Yong Park2 and Yong-Sun Kim1, 4
(1) Ilsong Institute of Life Science, Hallym University, Ilsong Building, 1605-4, Gwanyang-dong, Dongan-gu, Anyang, Kyounggi-do, 431-060, South Korea (2) Department of Virology, Korea National Institute of Health, Eunpyung-gu, Seoul, 122-701, South Korea (3) New York State Institute for Basic Research in Developmental Disabilities, Staten Island, NY 10314, USA (4) Department of Microbiology, College of Medicine, Hallym University, 1605-4, Gwanyang-dong, Dongan-gu, Anyang, Kyounggi-do, 431-060, South Korea
Received: 7 January 2004 Accepted: 5 March 2004 Published online: 18 May 2004
Abstract Human prion protein gene (PRNP) has been considered to be involved in the susceptibility of humans to prion diseases. Polymorphisms of methionine (Met)/valine (Val) at codon 129 and of glutamic acid (Glu)/lysine (Lys) at codon 219 are thought to play an important role in susceptibility to sporadic, iatrogenic and variant Creutzfeldt–Jakob disease (CJD). Although the genotype distribution of polymorphisms in PRNP open reading frame (ORF) has been reported in many European populations, among Asian groups, it has been reported only in the Japanese population. We examined the PRNP polymorphisms in 529 healthy Koreans. We observed that genotype frequencies at codon 129 was 94.33% Met/Met, 5.48% Met/Val, and 0.19% Val/Val with an allele frequency of 0.971:0.029 Met:Val, and that genotype frequencies at codon 219 was 92.06% Glu/Glu, 7.94% Glu/Lys, and 0% Lys/Lys with an allele frequency of 0.96:0.04 Glu:Lys. The frequencies of the Glu/Glu genotype (2=10.075, P=0.0015) and of the Glu allele (2=9.486, P=0.0021) at codon 219 were significantly higher in the Korean population than the Japanese population. In addition, the genotype frequency of heterozygotes (12.7%) at codons 129 or/and 219 was significantly lower in Koreans than in people from Great Britain (2=89.52, P<0.0001).
European Journal of Human Genetics (2005) 13, 1094–1097. doi:10.1038/sj.ejhg.5201460; published online 29 June 2005
Polymorphism at 3' UTR +28 of the prion-like protein gene is associated with sporadic Creutzfeldt–Jakob disease Byung-Hoon Jeong1, Nam-Ho Kim1, Eun-Kyoung Choi1, Chaeyoung Lee1, Young-Han Song1, Jae-Il Kim2, Richard I Carp2 and Yong-Sun Kim1,3
1Ilsong Institute of Life Science, Hallym University, 1605-4 Gwanyang-dong
Our results are the first genetic association study of the PRND noncoding region with sporadic CJD. Recently, we reported that the distributions of codons 129 and 219 genotypes of PRNP in a Korean population differ significantly from those reported for other ethnic groups.24 Thus, further investigations in different ethnic groups including Europeans will be necessary to assess association between sporadic CJD and the PRND 3' UTR +28 polymorphism. Furthermore, since it is unknown whether this polymorphism affects mRNA stability or gene expression of PRND, further experiments should be conducted to clarify the role of this polymorphism in PRND function.
iatrogenic Creutzfeldt Jakob Disease
Reports of incidents of potential iatrogenic exposure to CJD via surgery: 01 January 2000 to 31 Dec 2007
There were a total of 329 incidents reported during this period (table 1). Eleven surgical incidents were reported between 1 July and 31 December 2007 (since the previous update report). A surgical incident occurs when a patient undergoes surgery but is only identified as having CJD or being at risk of CJD at a later date. This means that the Advisory Committee on Dangerous Pathogens (ACDP) transmissible spongiform encephalopathy working group infection control guidelines would not have been followed. The surgery carried out on an index patient with, or at risk of, CJD may result in contamination of the instruments with abnormal prion protein. Table 1 shows the number of CJD surgical incidents reported to the CJD Incidents Panel from January 2000 to December 2007 by the diagnosis of the index patient.
Table 1 CJD Surgical Incidents (n=329) reported to the CJD Incidents Panel, by diagnosis of index patient: January 2000 to Dec 2007
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Wednesday, January 02, 2008 Risk factors for sporadic Creutzfeldt-Jakob disease Wednesday, January 02, 2008 Risk factors for sporadic Creutzfeldt-Jakob disease
FURTHER INTO THIS STUDY ;
Monday, December 31, 2007 Risk Assessment of Transmission of Sporadic Creutzfeldt-Jakob Disease in Endodontic Practice in Absence of Adequate Prion Inactivation
Wednesday, June 4, 2008
SEAC 2008 ONE HUNDREDTH MEETING OF THE SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE
In summary, although this young woman clearly died of a prion disease, we could not conclude she had vCJD, but also we could not be sure this was sporadic CJD either. Although we were not able to make definite conclusions on the basis of a single patient, we felt it important to report this tragic death and these findings in the medical literature so as to bring this to the attention of other doctors who might see similar patients in the future. This unusual finding reminds us of the importance of keeping alert to the possibility that BSE prions will cause disease in individuals with different genetic types, who may develop a disease that may resemble sporadic CJD, or vCJD, or have a new pattern of disease. This work emphasises the importance of continuing to study the rogue prion protein type in patients and we thank all patients and families who have kindly consented to use their, or their loved one’s, tissues for this medical research.
Reference: Creutzfeldt-Jakob Disease, Prion Protein Gene Codon 129VV, and a Novel PrPSc Type in a Young British Woman Simon Mead; Susan Joiner; Melanie Desbruslais; Jonathan A. Beck; Michael O’ Donoghue; Peter Lantos; Jonathan D. F. Wadsworth; John Collinge Arch Neurol. 2007;64(12):1780-1784.
www.cjdsupport.net Newsletter issue 17 april 2008
Creutzfeldt-Jakob Disease, Prion Protein Gene Codon 129VV, and a Novel PrPSc ype in a Young British Woman
And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward , as well as between L-type BSE and CJD . These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.
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Thursday, June 05, 2008
Review on the epidemiology and dynamics of BSE epidemics
The statistical incidence of CJD cases in the United States has been *** revised *** to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.
SIGN THE PETITION TODAY, it may be the only recourse for the USA consumer to finally get USA beef tested for BSE. We must stand together and demand a safer BSE free beef product
Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518