Thursday, May 17, 2012

Iatrogenic Creutzfeldt-Jakob Disease, Final Assessment

Volume 18, Number 6—June 2012


CME ACTIVITY


Iatrogenic Creutzfeldt-Jakob Disease, Final Assessment




Paul Brown , Jean-Philippe Brandel, Takeshi Sato, Yosikazu Nakamura, Jan MacKenzie, Robert G. Will, Anna Ladogana, Maurizio Pocchiari, Ellen W. Leschek, and Lawrence B. Schonberger Author affiliations: Centre à l’Energie Atomique, Fontenay-aux-Roses, France (P. Brown); Institut National de la Santé et de la Recherche Médicale, Paris, France (J.-P. Brandel); Nanohana Clinic, Tokyo, Japan (T. Sato); Jichi Medical University, Yakushiji, Japan (Y. Nakamura); Western General Hospital, Edinburgh, Scotland, UK (J. MacKenzie, R.G. Will); Istituto Superiore de Sanità, Rome, Italy (A. Ladogana, M. Pocchiari); National Institutes of Health, Bethesda, Maryland, USA (E.W. Leschek); Centers for Disease Control and Prevention, Atlanta, Georgia, USA (L.B. Schonberger)




Abstract


The era of iatrogenic Creutzfeldt-Jakob disease (CJD) has nearly closed; only occasional cases with exceptionally long incubation periods are still appearing. The principal sources of these outbreaks are contaminated growth hormone (226 cases) and dura mater grafts (228 cases) derived from human cadavers with undiagnosed CJD infections; a small number of additional cases are caused by neurosurgical instrument contamination, corneal grafts, gonadotrophic hormone, and secondary infection with variant CJD transmitted by transfusion of blood products. No new sources of disease have been identified, and current practices, which combine improved recognition of potentially infected persons with new disinfection methods for fragile surgical instruments and biological products, should continue to minimize the risk for iatrogenic disease until a blood screening test for the detection of preclinical infection is validated for human use.


The first case of what would eventually become a major outbreak of iatrogenic Creutzfeldt-Jakob disease (CJD) was reported in 1974; the patient had received a corneal transplant from an infected cadaver (1). In the years that followed, other sources of infection were identified: stereotactic electroencephalogram electrodes, neurosurgical instruments, cadaveric dura mater and pituitary glands, and, most recently, secondary variant CJD (vCJD) blood products. The ensemble of iatrogenic cases, including a bibliography of primary references, was last reviewed in 2006 (2). Today, after nearly 40 years of surveillance, the chronology and essential characteristics of iatrogenic CJD have been finalized, and the purpose of this article is to present these data along with a few brief comments about factors that determined the risk for infection and how future risks might be foreseen and avoided.




By far the most common sources of iatrogenic disease were human cadavers from which pituitary hormones and dura mater grafts were obtained (Table 1; Figure); the other major variety of environmentally acquired disease is vCJD. The incidence curves of human growth hormone–associated and dura mater–associated CJD are almost superimposable; a broad peak occurred in the mid-to-late 1990s, just ahead of the sharper peak incidence of vCJD in the United Kingdom at the turn of the century. The incidence in other countries peaked a few years later, in 2004, as a result of the delayed appearance of bovine spongiform encephalopathy in those countries.


The long incubation periods—years to decades—of these low-dose infections pose a particularly difficult problem for public health officials, whose recommendations may diminish the number of new cases but are impotent when it comes to preventing cases in already-infected persons in the preclinical phase of disease. It is worth remembering that the early recognition of iatrogenic sources of CJD was entirely because of a few remarkably astute neurologists, neurosurgeons, and, astonishingly, a pediatric endocrinologist who pursued the unlikely (and unpopular) diagnosis of CJD in a growth hormone recipient (3). It is true that some of these connections had the benefit of comparatively short intervals between the infecting events and the onset of CJD. It is especially fortunate from the standpoint of early recognition of the dura mater association that the interval of 19 months between the operation and onset of symptoms in the first case-patient was among the shortest on record for this form of iatrogenic CJD (Table 2).






Human Growth Hormone


The current worldwide total of growth hormone–associated cases of CJD is 226. Most cases occurred in France (119 cases/1,880 recipients; attack rate 6.3%), the United Kingdom (65 cases/1,800 recipients; attack rate 3.6%), and the United States (29 cases/7,700 recipients; attack rate 0.4%).






In France, further epidemiologic observations have revealed that all 119 cases occurred within a 1,170-patient cohort receiving treatment during a 20-month period, from December 1983 through July 1985, when there seems to have been substantial contamination resulting from sourcing and processing deficiencies. According to these numbers, the attack rate for the at-risk cohort in France increases to 10.2%. No new case has been identified since 2008. In the United Kingdom, no cohort pattern is evident, and cases continue to occur at an average rate of about 2 per year (only 1 in 2011). In the United States, CJD has not occurred in any patient who started treatment after 1977, when a highly selective column chromatography step was introduced into the purification protocol. Since 2003, only 2 new cases have been identified (1 in 2007 and 1 in 2009). An estimated ≈2,700 patients received treatment before 1977, so the attack rate in the United States for this at-risk cohort increases to 1.1% (4). The revised attack rates therefore become 10.2% in France, 3.6% in the United Kingdom, and 1.1% in the United States.


The methionine (M)/valine polymorphism at codon 129 of the PRNP gene has been examined in populations with and without CJD in many countries; results have varied (Table 3). Overall, it is clear that the M allele bestows substantial susceptibility to the sporadic and the iatrogenic forms of CJD; in consequence, the proportion of persons with MM homozygous genotype is overrepresented in both categories of disease (the sole exception occurred in UK growth hormone recipients, which led to speculation that a different strain of the pathogenic agent might have been disseminated) (10). It is also clear that, as a group, persons with heterozygous genotype had longer incubation periods than did those with homozygous genotype, particularly in France. Notwithstanding this statistical conclusion, it is noteworthy that several persons with MM homozygous genotype had incubation periods >30 years, including a patient with recently diagnosed CJD, whose incubation period was 42 years, the current world record for any type of iatrogenic disease.






Incubation periods for the total case population (not just those examined for the codon 129 genotype) ranged from 5 to 42 years (mean 17 years), based on the interval between the midpoint date of what was almost always a multiyear period of treatment and the onset of CJD symptoms; the actual date of infection is impossible to determine. Mean incubation periods for cases in the United States and New Zealand (patients received hormone made in the United States) were 22 and 26 years; United Kingdom, 20 years; and France, 13 years. The shorter incubation periods in France could have resulted partly from the narrower limit for the date of infection in France and are in accord with the mean incubation period of 13.5 years in the 4 gonadotropin recipients from Australia, for whom there is an even more precise date of infection. However, a greater contribution probably came from different infectious doses received by patients in the different countries. Among all patients, the clinical features were distinctive in that, unlike sporadic CJD, signs and symptoms almost never included dementia, which, if it occurred at all, was typically a late component of the clinical course.






Dura Mater




The worldwide tally of dura mater–associated cases is 228, and new cases still continue to occur here and there, the most recent being individual cases in Austria, South Korea, and the Netherlands in 2011. If the pharmaceutical industry (in contrast to government-sponsored laboratories) comes away from the growth hormone story with an almost untainted record—only 1 case has been attributed to industrially prepared hormone (11)—the same cannot be said about the private sector producing dura mater grafts. The source of almost all infections was a manufacturer in Germany, B. Braun Melsungen AG, which has a worldwide distribution network, and the incidence of CJD appears to have more or less paralleled the frequency with which this source of dura mater was used. In Japan, it is estimated that as many as 20,000 patches may have been used each year, and the 142 cases in that country constitute two thirds of the global total. Nevertheless, the overall attack rate in the at-risk patient population in Japan is <0.03%. For the entire (worldwide) group of dura mater–recipient patients, incubation periods ranged from 1.3 to 30 years (mean 12 years), and, except in Japan, the clinical and neuropathologic features were similar to those of sporadic CJD. In Japan, approximately one third of the cases had atypical features (slow progression, noncharacteristic electroencephalogram tracings, plaque deposition, and an atypical prion protein molecular signature on Western blots), which suggested the possibility of 2 different strains of infecting agent (12,13). One patient had florid plaques and a pulvinar sign on magnetic resonance imaging, mimicking vCJD (5).


Evaluation of the influence of the codon 129 genotype is complicated by the fact that the population in Japan, among whom most cases occurred, has a high frequency of the M allele (>90%), which dominated sporadic and dura mater–associated forms of CJD (Table 3) (6–9,14,15). Among the cases in persons not from Japan, the distribution of genotypes approximated that found among patients with sporadic CJD, and, as with growth hormone–associated cases, incubation periods were somewhat longer for persons with heterozygous than with homozygous genotypes.






Current Prevention Strategies




The best way to abolish secondary iatrogenic infections is, obviously, to prevent primary infections, but without a test to identify infected but asymptomatic persons, we cannot entirely eliminate the risk inherent in human-to-human tissue transfer. We are therefore obliged to rely on the default strategies of 1) identification and donor deferral of persons at higher than normal risk for CJD development and 2) inclusion of prion-reduction steps in the sterilization of penetrating instruments and the processing of therapeutic tissues and fluids.






Delineation of high-risk categories initially focused on precisely those groups of persons who were exposed to the known sources of iatrogenic disease: recipients of cadaveric dura mater grafts or pituitary-derived hormones. When vCJD started to occur, restrictions were also placed on donor time of residence in the most heavily infected regions—the United Kingdom and, to a lesser extent, continental Europe—and embargoes were placed on the importation of biological products from these regions. These deferral and import restrictions remain in place today and need some thoughtful reevaluation in view of the near extinction of all such sources of iatrogenic CJD. In the United States, there have been only 4 cases of dura mater–associated disease (the most recent in 2005) and no case of growth hormone–associated CJD for anyone who began treatment after 1977.






On the other hand, the possibility of iatrogenic infection resulting from transfer of tissues or fluids from persons who have contracted a prion disease from animals has not disappeared with the abating epidemics of bovine spongiform encephalopathy and vCJD. A few persons who may be experiencing a long incubation phase of vCJD still pose an obvious danger in the United Kingdom, but an underappreciated potential danger lies in 2 other animal diseases: scrapie and chronic wasting disease (CWD). Although scrapie-infected sheep tissues have been consumed for long enough (hundreds of years) to be considered harmless for humans, the same cannot be said about the atypical strains of scrapie that are beginning to displace the typical strains and with which we do not yet have enough experience to evaluate human pathogenicity. Similarly, we cannot declare with certainty that CWD poses no threat to humans, and CWD is continuing its unchecked spread across the United States and Canada with no guarantee that it will not become globally distributed in the years to come. One hunter has already put a group of unwitting persons at risk for infection by donating a deer, later found to have CWD, for consumption at a rural banquet in New York State (16); more such exposures are likely to occur as CWD continues its geographic expansion.








Future Prevention Strategies




The issue of reducing risk by taking steps to inactivate prions is always a work in progress as new therapeutic products come into production and new methods to inactivate prions are discovered. The tried-and-true laboratory method of prion sterilization (1-hour exposures to either undiluted bleach or 1 N sodium hydroxide followed by steam autoclaving at 3 atmospheres pressure for 20 minutes) is applicable only to nonfragile instruments and not at all to living tissues. The surprising resistance of dura mater to 0.1 N sodium hydroxide (17) and of growth hormone to 6 M urea (18) led to their incorporation into processing protocols before being replaced by nondural tissue or synthetic patches and recombinant hormone. To reduce infectivity, blood, blood products, and other fluids can be subjected to nanofiltration and prion-affinity ligands (19–22), which should also be applicable to other biological products, for example, vaccine and stem cell cultures, should they be susceptible to infection (23). Fragile instruments such as endoscopes and electrodes remain a challenge, but new and gentler methods— alkaline cleaning solutions, phenolics, and gaseous hydrogen peroxide—have proven harmless to instruments and give a high, if not always complete, degree of prion inactivation (24–26).






The ongoing refinement of a quaking-induced conversion detection of the misfolded prion protein holds the best prospect of evolving into a sensitive and practical tool, but it has yet to be validated in blind testing of plasma from symptomatic patients or in presymptomatic persons, even more rigorous but necessary (27,28). It may be necessary to use scrapie-infected animals for presymptomatic validation because only 1 group of humans could furnish appropriate samples—asymptomatic carriers of CJD-inducing mutations—and putting together and testing a reasonable number of such samples will take years to accomplish.






The total numbers of cases for the 2 major causes of iatrogenic CJD during the past 40 years (226 growth hormone cases and 228 dura mater cases) are amazingly close and are likely to remain so after the few additional long-incubating cases finally surface in the next few years. The combination of appropriate blood donor deferrals and the incorporation of tissue, fluid, and instrument infectivity–reduction steps should continue to hold the sources of potential iatrogenic disease to a minimum until such time as a practical screening test for inapparent infection is validated for human use.






Dr Brown spent his career at the National Institutes of Health in the Laboratory of Central Nervous System Studies conducting research on the transmissible spongiform encephalopathies, especially with respect to epidemiology, iatrogenic CJD, disinfection, and blood infectivity. He currently chairs a scientific advisory committee for the Laboratoire Français du Fractionnement et des Biotechnologies in Les Ulis, France, and advises the Centre à l’Energie Atomique in Fontenay-aux-Roses, France.






Acknowledgment




Our profound thanks go to the physicians responsible for the earliest identification of iatrogenic CJD infections and to the multitude of unsung persons in many countries around the world who have worked diligently and continuously to keep track of its global incidence.






References




Duffy P, Wolf J, Collins G, DeVoe AB, Streeten B, Cowen D. Letter: possible person-to-person transmission of Creutzfeldt-Jakob disease. N Engl J Med. 1974;290:692–3. DOIPubMed Brown P, Brandel J-P, Preece M, Sato T. Iatrogenic Creutzfeldt-Jakob disease: the waning of an era. Neurology. 2006;67:389–93. DOIPubMed Brown P. Human growth hormone therapy and Creutzfeldt-Jakob disease: a drama in three acts. Pediatrics. 1988;81:85–92.PubMed Abrams JY, Schonberger LB, Belay ED, Maddox RA, Leschek EW, Mills JL, Lower risk of Creutzfeldt-Jakob disease in pituitary growth hormone recipients initiating treatment after 1977. J Clin Endocrinol Metab. 2011;96:E1666–9. DOIPubMed Wakisaka Y, Santa N, Doh-ura K, Kitamoto T, Ibayashi S, Iida M, Increased asymmetric pulvinar magnetic resonance imaging signals in Creutzfeldt-Jakob disease with florid plaques following a cadaveric dura mater graft. Neuropathology. 2006;26:82–8. DOIPubMed Soldevila M, Calafell F, Andrès AM, Yagüe J, Helgason A, Stefánsson K, Prion susceptibility and protective alleles exhibit marked geographic differences. Hum Mutat. 2003;22:104–5. DOIPubMed Nurmi MH, Bishop M, Strain L, Brett F, McGuigan C, Hutchison M, The normal population distribution of PRNP codon 129 polymorphism. Acta Neurol Scand. 2003;108:374–8. DOIPubMed Mercier G, Diéterlen F, Lucotte G. Population distribution of the methionine allele at the PRNP codon 129 polymorphism in Europe and the Middle East. Hum Biol. 2008;80:181–90. DOIPubMed Doh-ura K, Kitamoto T, Sakaki Y, Taateishi J. CJD discrepancy. Nature. 1991;353:801–2. DOIPubMed Brandel J-P, Preece M, Brown P, Croes E, Laplanche J-L, Agid Y, Distribution of codon 129 genotype in human growth hormone–treated CJD patients in France and the UK. Lancet. 2003;362:128–30. DOIPubMed Furtner M, Gelpi E, Kiechl S, Knoflach M, Zangerl A, Gotwald T, Iatrogenic Creutzfeldt-Jakob disease 22 years after human growth hormone therapy: clinical and radiological features. J Neurol Neurosurg Psychiatry. 2008;79:229–31. DOIPubMed Noguchi-Shinohara M, Hamaguchi T, Kitamoto T, Sato T, Nakamura Y, Mizusawa H, Clinical features and diagnosis of dura mater graft–associated Creutzfeldt-Jakob disease. Neurology. 2007;69:360–7. DOIPubMed Yamada M, Noguchi-Shinohara M, Hamaguchi T, Nozaki I, Kitamoto T, Sato T, Dura mater graft–associated Creutzfeldt-Jakob disease in Japan: clinicopathological and molecular characterization of the two distinct subtypes. Neuropathology. 2009;29:609–18. DOIPubMed Nozaki I, Hamaguchi T, Sanjo N, Noguchi-Shinohara M, Sakai K, Nakamura Y, Prospective 10-year surveillance of human prion diseases in Japan. Brain. 2010;133:3043–57. DOIPubMed Ladogana A, Puopolo M, Croes EA, Budka H, Jarius C, Collins S, Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada. Neurology. 2005;64:1586–91. DOIPubMed Garruto RM, Reiber C, Alfonso MP, Gastrich H, Needham K, Sunderman S, Risk behaviors in a rural community with a known point-source exposure to chronic wasting disease. Environ Health. 2008;7:31. DOIPubMed Diringer H, Braig HR. Infectivity of unconventional viruses in dura mater. Lancet. 1989;1:439–40. DOIPubMed Pocchiari M, Peano S, Conz A, Eshkol A, Maillard F, Brown P, Combination ultrafiltration and 6 M urea treatment of human growth hormone effectively minimizes risk from potential Creutzfeldt-Jakob disease virus contamination. Horm Res. 1991;35:161–6. DOIPubMed Yunoki M, Tanaka H, Urayama T, Hattori S, Ohtani M, Ohkubo Y, Prion removal by nanofiltraion under different experimental conditions. Biologicals. 2008;36:27–36. DOIPubMed Cardone F, Simoneau S, Arzel A, Puopolo M, Berardi VA, Abdel-Haq H, Comparison of nanofiltration efficacy in reducing infectivity of centrifuged versus ultracentrifuged 263K scrapie-infected brain homogenates in “spiked” albumin solutions. Transfusion. 2011. Epub ahead of print. DOIPubMed Gregori L, Gurgel PV, Lathrop JT, Edwardson P, Lambert BC, Carbonell RG, Reduction in infectivity of endogenous transmissible spongiform encephalopathies present in blood by adsorption to selective affinity resins. Lancet. 2006;368:2226–30. DOIPubMed Heger A, Bailey A, Neisser-Svae A, Ertl M, Römisch J, Svae TE. Removal of prion infectivity by affinity ligand chromatography during OctaplasLG manufacturing—results from animal bioassay studies. Vox Sang. 2011. Epub ahead of print. DOIPubMed Piccardo P, Cervenakova L, Vasilyeva I, Yakovleva O, Bacik I, Cervenak J, Candidate cell substrates, vaccine production, and transmissible spongiform encephalopathies. Emerg Infect Dis. 2011;17:2262–9. DOIPubMed Fichet G, Comoy E, Duval C, Antioga K, Dehen C, Charbonnier A, Novel methods for disinfection of prion-contaminated medical devices. Lancet. 2004;364:521–6. DOIPubMed Fichet G, Antioga K, Comoy E, Deslys JP, McDonnell G. Prion inactivation using a new gaseous hydrogen peroxide sterilization process. J Hosp Infect. 2007;67:278–86. DOIPubMed Fichet G, Harrison J, McDonnell G. Reduction of risk of prion transmission on surgical devices with effective cleaning processes. Zentr Steril. 2007;15:418–37. Orrú CD, Wilham JM, Raymond LD, Kuhn F, Schroeder B, Raeber AJ, Prion disease blood test using immunoprecipitation and improved quaking-induced conversion. MBiol. 2011;3:e00078-11 [cited 2012 Mar 31]. http://mbio.asm.org/content/2/3/e00078-11.full Orrú CD, Wilham JM, Vascellari S, Hughson AG, Caughey B. New generation QuIC assays for prion seeding activity. Prion. 2012;6. Epub ahead of print.


Figure


Figure. . . . Annual incidence of variant Creutzfeldt-Jakob disease (vCJD) caused by ingestion of meat products contaminated with bovine spongiform encephalopathy agent (A) and iatrogenic CJD caused by contaminated dura...


Tables


Table 1. Global distribution of cases of iatrogenic Creutzfeldt-Jakob disease


Table 2. Incubation periods and clinical presentations of iatrogenic Creutzfeldt-Jakob disease, according to source of infection


Table 3. Comparison of PRNP codon 129 genotype frequencies and incubation periods in growth hormone– and dura mater–associated cases of iatrogenic CJD


Suggested citation for this article: Brown P, Brandel J-P, Sato T, Nakamura Y, MacKenzie J, Will RG, et al. Iatrogenic Creutzfeldt-Jakob disease, final assessment. Emerg Infect Dis [serial on the Internet]. 2012 Jun [date cited]. http://dx.doi.org/10.3201/eid1806.120116


DOI: 10.3201/eid1806.120116






Volume 18, Number 6—June 2012 CME ACTIVITY Iatrogenic Creutzfeldt-Jakob Disease, Final Assessment MEDSCAPE CME Medscape, LLC is pleased to provide online continuing medical education (CME) for this journal article, allowing clinicians the opportunity to earn CME credit.


This activity has been planned and implemented in accordance with the Essential Areas and policies of the Accreditation Council for Continuing Medical Education through the joint sponsorship of Medscape, LLC and Emerging Infectious Diseases. Medscape, LLC is accredited by the ACCME to provide continuing medical education for physicians.


Medscape, LLC designates this Journal-based CME activity for a maximum of 1 AMA PRA Category 1 Credit(s)TM. Physicians should claim only the credit commensurate with the extent of their participation in the activity.


All other clinicians completing this activity will be issued a certificate of participation. To participate in this journal CME activity: (1) review the learning objectives and author disclosures; (2) study the education content; (3) take the post-test with a 70% minimum passing score and complete the evaluation at www.medscape.org/journal/eid; (4) view/print certificate.






Release date: May 16, 2012; Expiration date: May 16, 2013






Learning Objectives


Upon completion of this activity, participants will be able to:




• Distinguish the principal sources of iatrogenic CJD




• Identify countries with the highest rates of documented CJD




• Analyze the clinical presentation of iatrogenic CJD




• Assess new threats which might promote higher rates of CJD.




CME Editor


P. Lynne Stockton, Technical Writer/Editor, Emerging Infectious Diseases. Disclosure: P. Lynne Stockton has disclosed no relevant financial relationships.




CME AUTHOR


Charles P. Vega, MD, Health Sciences Clinical Professor; Residency Director, Department of Family Medicine, University of California, Irvine. Disclosure: Charles P. Vega, MD, has disclosed no relevant financial relationships.




AUTHORS


Disclosures: Paul Brown; Jean-Philippe Brandel; Takeshi Sato, MD; Yosikazu Nakamura, MD, MPH, FFPH; Jan MacKenzie; Anna Ladogana; Ellen W. Leschek, MD; and Lawrence B. Schonberger, MD, MPH, have disclosed no relevant financial relationships. Robert G. Will, FRCP, has disclosed the following relevant financial relationships: served as an advisor or consultant for LFB, Farring. Maurizio Pocchiari, MD, has disclosed the following relevant financial relationships: served as an advisor or consultant for LFB, Farring.










I hope and pray that Paul Brown et al rosey outlook is correct, and the end of iatrogenic Creutzfeldt Jakob Disease is truly over, bbut, I have my doubts. ...TSS






April 12, 2012


Health professions and risk of sporadic Creutzfeldt–Jakob disease, 1965 to 2010


Eurosurveillance,


Volume 17, Issue 15, 12


April 2012


Research articles








Sunday, May 6, 2012


Bovine Spongiform Encephalopathy Mad Cow Disease, BSE May 2, 2012 IOWA State University OIE








Friday, May 11, 2012


Experimental H-type bovine spongiform encephalopathy characterized by plaques and glial- and stellate-type prion protein deposits


In addition, the present data will support risk assessments in some peripheral tissues derived from cattle affected with H-type BSE.








SPONTANEOUS ??? NOT...




How the California cow got the disease remains unknown. Government officials expressed confidence that contaminated food was not the source, saying the animal had atypical L-type BSE, a rare variant not generally associated with an animal consuming infected feed.




However, a BSE expert said that consumption of infected material is the only known way that cattle get the disease under natural conditons. “In view of what we know about BSE after almost 20 years experience, contaminated feed has been the source of the epidemic,” said Paul Brown, a scientist retired from the National Institute of Neurological Diseases and Stroke.




BSE is not caused by a microbe. It is caused by the misfolding of the so-called “prion protein” that is a normal constituent of brain and other tissues. If a diseased version of the protein enters the brain somehow, it can slowly cause all the normal versions to become misfolded. It is possible the disease could arise spontaneously, though such an event has never been recorded, Brown said.










Proposal ID: 29403




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




Background




Alzheimer’s disease and Transmissible Spongiform Encephalopathy disease have both been around a long time, and was discovered in or around the same time frame, early 1900’s. Both disease, and it’s variants, in many cases are merely names of the people that first discovered them. Both diseases are incurable and debilitating brain disease, that are in the end, 100% fatal, with the incubation/clinical period of the Alzheimer’s disease being longer than the TSE prion disease. Symptoms are very similar, and pathology is very similar. I propose that Alzheimer’s is a TSE disease of low dose, slow, and long incubation disease, and that Alzheimer’s is Transmissible, and is a threat to the public via the many Iatrogenic routes and sources. It was said long ago that the only thing that disputes this, is Alzheimer’s disease transmissibility, or the lack of. today, there is enough documented science (some confidential), that shows that indeed Alzheimer’s is transmissible. The risk factor for friendly fire, and or the pass-it-forward mode i.e. Iatrogenic transmission is a real threat, and one that needs to be addressed immediately.




Methods




Through years of research, as a layperson, of peer review journals, transmission studies, and observations of loved ones and friends that have died from both Alzheimer’s and the TSE prion disease i.e. Heidenhain Variant Creutzfelt Jakob Disease CJD.




Results




The likelihood of many victims of Alzheimer’s disease from the many different Iatrogenic routes and modes of transmission as with the TSE prion disease. TSE prion disease survives ashing to 600 degrees celsius, that’s around 1112 degrees farenheit. you cannot cook the TSE prion disease out of meat. you can take the ash and mix it with saline and inject that ash into a mouse, and the mouse will go down with TSE. Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel Production as well. the TSE prion agent also survives Simulated Wastewater Treatment Processes. IN fact, you should also know that the TSE Prion agent will survive in the environment for years, if not decades. you can bury it and it will not go away. TSE prion agent is capable of infected your water table i.e. Detection of protease-resistant cervid prion protein in water from a CWD-endemic area. it’s not your ordinary pathogen you can just cook it out and be done with. that’s what’s so worrisome about Iatrogenic mode of transmission, a simple autoclave will not kill this TSE prion agent.




Conclusions




There should be a Global Congressional Science round table event (one of scientist and doctors et al only, NO CORPORATE, POLITICIANS ALLOWED) set up immediately to address these concerns from the many potential routes and sources of the TSE prion disease, including Alzheimer’s disease, and a emergency global doctrine put into effect to help combat the spread of Alzheimer’s disease via the medical, surgical, dental, tissue, and blood arena’s. All human and animal TSE prion disease, including Alzheimer’s should be made reportable in every state, and Internationally, WITH NO age restrictions. Until a proven method of decontamination and autoclaving is proven, and put forth in use universally, in all hospitals and medical, surgical arena’s, or the TSE prion agent will continue to spread. IF we wait until science and corporate politicians wait until politics let science _prove_ this once and for all, and set forth regulations there from, we will all be exposed to the TSE Prion agents, if that has not happened already. what’s the use of science progressing human life to the century mark, if your brain does not work?








Wednesday, May 16, 2012


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


Proposal ID: 29403












Friday, February 10, 2012


Creutzfeldt-Jakob disease (CJD) biannual update (2012/1) potential iatrogenic (healthcare-acquired) exposure to CJD, and on the National Anonymous Tonsil Archive






Thursday, December 29, 2011


Aerosols An underestimated vehicle for transmission of prion diseases?


PRION www.landesbioscience.com


please see more on Aerosols and TSE prion disease here ;






Saturday, February 12, 2011


Another Pathologists dies from CJD, another potential occupational death ?


another happenstance of bad luck, a spontaneous event from nothing, or friendly fire ???






Tuesday, December 14, 2010


Infection control of CJD, vCJD and other human prion diseases in healthcare and community settings part 4, Annex A1, Annex J,


UPDATE DECEMBER 2010






Tuesday, September 14, 2010


Transmissible Spongiform Encephalopathies Advisory Committee; Notice of Meeting October 28 and 29, 2010 (COMMENT SUBMISSION)








Thursday, September 02, 2010


NEUROSURGERY AND CREUTZFELDT-JAKOB DISEASE Health Law, Ethics, and Human Rights The Disclosure Dilemma








Thursday, August 12, 2010


USA Blood products, collected from a donor who was at risk for vCJD, were distributed July-August 2010








Sunday, August 01, 2010




Blood product, collected from a donors possibly at increased risk for vCJD only, was distributed USA JULY 2010
















Thursday, July 08, 2010


Nosocomial transmission of sporadic Creutzfeldt–Jakob disease: results from a risk-based assessment of surgical interventions Public release date: 8-Jul-2010






Thursday, July 08, 2010


GLOBAL CLUSTERS OF CREUTZFELDT JAKOB DISEASE - A REVIEW 2010






Wednesday, June 02, 2010


CJD Annex H UPDATE AFTER DEATH PRECAUTIONS Published: 2 June 2003 Updated: May 2010








Tuesday, May 11, 2010


Current risk of iatrogenic Creutzfeld–Jakob disease in the UK: efficacy of available cleaning chemistries and reusability of neurosurgical instruments








Tuesday, May 04, 2010


Review of the Human Pituitary Trust Account and CJD Issue 20 January 2010








Tuesday, March 16, 2010


Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection: Part 4 REVISED FEB. 2010








Monday, August 17, 2009


Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection: Annex J,K, AND D Published: 2009








Monday, July 20, 2009


Pre-surgical risk assessment for variant Creutzfeldt-Jakob disease (vCJD) risk in neurosurgery and eye surgery units








Friday, July 17, 2009


Revision to pre-surgical assessment of risk for vCJD in neurosurgery and eye surgery units Volume 3 No 28; 17 July 2009








Sunday, May 10, 2009


Meeting of the Transmissible Spongiform Encephalopathies Committee On June 12, 2009 (Singeltary submission)








Thursday, January 29, 2009


Medical Procedures and Risk for Sporadic Creutzfeldt-Jakob Disease, Japan, 1999-2008 (WARNING TO Neurosurgeons and Ophthalmologists) Volume 15, Number 2-February 2009 Research








Wednesday, August 20, 2008


Tonometer disinfection practice in the United Kingdom: A national survey








Tuesday, August 12, 2008


Biosafety in Microbiological and Biomedical Laboratories Fifth Edition 2007 (occupational exposure to prion diseases)








Monday, December 31, 2007


Risk Assessment of Transmission of Sporadic Creutzfeldt-Jakob Disease in Endodontic Practice in Absence of Adequate Prion Inactivation










Subject: CJD: update for dental staff


Date: November 12, 2006 at 3:25 pm PST


1: Dent Update. 2006 Oct;33(8):454-6, 458-60.


CJD: update for dental staff.








Saturday, January 16, 2010


Evidence For CJD TSE Transmission Via Endoscopes 1-24-3 re-Singeltary to Bramble et al


Evidence For CJD/TSE Transmission Via Endoscopes


From Terry S. Singletary, Sr flounder@wt.net 1-24-3








2011 TO 2012 UPDATE




Saturday, December 3, 2011


Candidate Cell Substrates, Vaccine Production, and Transmissible Spongiform Encephalopathies


Volume 17, Number 12—December 2011








Sunday, June 26, 2011


Risk Analysis of Low-Dose Prion Exposures in Cynomolgus Macaque








Monday, February 7, 2011


FDA’s Currently-Recommended Policies to Reduce the Possible Risk of Transmission of CJD and vCJD by Blood and Blood Products 2011 ???






Saturday, March 5, 2011


MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE RISE IN NORTH AMERICA








Sunday, February 12, 2012


National Prion Disease Pathology Surveillance Center Cases Examined1 (August 19, 2011) including Texas








Terry S. Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health Crisis


















full text with source references ;










Subject: Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products APHIS-2008-0010-0008 RIN:0579-AC68


Comment from Terry Singeltary Document ID: APHIS-2008-0010-0008 Document Type: Public Submission This is comment on Proposed Rule: Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products Docket ID: APHIS-2008-0010 RIN:0579-AC68


Topics: No Topics associated with this document View Document: More


Document Subtype: Public Comment Status: Posted Received Date: March 22 2012, at 12:00 AM Eastern Daylight Time Date Posted: March 22 2012, at 12:00 AM Eastern Daylight Time Comment Start Date: March 16 2012, at 12:00 AM Eastern Daylight Time Comment Due Date: May 15 2012, at 11:59 PM Eastern Daylight Time Tracking Number: 80fdd617 First Name: Terry Middle Name: S. Last Name: Singeltary City: Bacliff Country: United States State or Province: TX Organization Name: CJD TSE PRION Submitter's Representative: CONSUMERS


Comment: comment submission Document ID APHIS-2008-0010-0001 Greetings USDA, OIE et al, what a difference it makes with science, from one day to the next. i.e. that mad cow gold card the USA once held. up until that fateful day in December of 2003, the science of BSE was NO IMPORTS TO USA FROM BSE COUNTRY. what a difference a day makes$ now that the shoe is on the other foot, the USDA via the OIE, wants to change science again, just for trade $ I implore the OIE decision and policy makers, for the sake of the world, to refuse any status quo of the USA BSE risk assessment. if at al, the USA BSE GBR should be raise to BSE GBR IV, for the following reasons. North America is awash with many different TSE Prion strains, in many different species, and they are mutating and spreading. IF the OIE, and whatever policy makers, do anything but raise the risk factor for BSE in North America, they I would regard that to be highly suspicious. IN fact, it would be criminal in my opinion, because the OIE knows this, and to knowingly expose the rest of the world to this dangerous pathogen, would be ‘knowingly’ and ‘willfully’, just for the almighty dollar, once again. I warned the OIE about all this, including the risk factors for CWD, and the fact that the zoonosis potential was great, way back in 2002. THE OIE in collaboration with the USDA, made the legal trading of the atypical Nor-98 Scrapie a legal global commodity. yes, thanks to the OIE and the USDA et al, it’s now legal to trade the atypical Nor-98 Scrapie strain all around the globe. IF you let them, they will do the same thing with atypical BSE and CWD (both strains to date). This with science showing that indeed these TSE prion strains are transmissible. I strenuously urge the OIE et al to refuse any weakening to the USA trade protocols for the BSE TSE prion disease (all strains), and urge them to reclassify the USA with BSE GBR IV risk factor. SEE REFERENCE SOURCES IN ATTACHMENTS


SEE Terry S. Singeltary Sr. Attachment WORD FILE ;














Sunday, March 11, 2012


APHIS Proposes New Bovine Spongiform Encephalopathy Import Regulations in Line with International Animal Health Standards Proposal Aims to Ensure Health of the U.S. Beef Herd, Assist in Negotiations






MAD COW USDA ATYPICAL L-TYPE BASE BSE, the rest of the story...


***Oral Transmission of L-type Bovine Spongiform Encephalopathy in Primate Model




***Infectivity in skeletal muscle of BASE-infected cattle




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




***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans.




Sunday, May 6, 2012


Bovine Spongiform Encephalopathy Mad Cow Disease, BSE May 2, 2012 IOWA State University OIE






Friday, May 11, 2012


Experimental H-type bovine spongiform encephalopathy characterized by plaques and glial- and stellate-type prion protein deposits







TSS

Wednesday, May 16, 2012

OIE UPDATE BOVINE SPONGIFORM ENCEPHALOPATHY UNITED STATES OF AMERICA MAY 15, 2012

OIE UPDATE BOVINE SPONGIFORM ENCEPHALOPATHY UNITED STATES OF AMERICA MAY 15, 2012




Information received on 15/05/2012 from Dr John Clifford, Deputy Administrator, Animal and Plant Health Inspection Service, United States Department of Agriculture, Washington, United States of America


Summary


Report type Follow-up report No. 1 Start date 19/04/2012 Date of first confirmation of the event 23/04/2012 Report date 15/05/2012 Date submitted to OIE 15/05/2012 Reason for notification Reoccurrence of a listed disease Date of previous occurrence 2006 Manifestation of disease Sub-clinical infection Causal agent Prion (atypical BSE) Nature of diagnosis Laboratory (advanced) This event pertains to the whole country Related reports Immediate notification (26/04/2012) Follow-up report No. 1 (15/05/2012) Outbreaks There are no new outbreaks in this report


Epidemiology Source of the outbreak(s) or origin of infection Unknown or inconclusive Random mutation Epidemiological comments As part of the United States targeted bovine spongiform encephalopathy (BSE) surveillance system a case of BSE classified as atypical was identified in a dead dairy cow that was to be rendered. The dead animal’s carcass was placed in a secure hazardous waste disposal site. • The cow was culled due to lameness. • The identified animal was never presented for slaughter for human consumption, did not enter food supply channels, and at no time presented any risk to human health. • A comprehensive epidemiological investigation into the incident continues to be conducted. Further epidemiological investigation of the incident has shown that: - The cow was born on the index premises. - Two progeny have been identified – one born in the last 2 years, was stillborn, and another, still living, was humanely euthanized, and tested and found to be negative for BSE. - Investigation of the feed records at the index dairy premises has found no anomalies, and audits of all the feed suppliers to the index premises have shown them to be in compliance with the regulations.


Control measures Measures applied Quarantine Screening No vaccination No treatment of affected animals Measures to be applied No other measures


Future Reporting The event is continuing. Weekly follow-up reports will be submitted.








MAD COW FEED RECALL USA EQUALS 10,878.06 TONS NATIONWIDE Sun Jul 16, 2006 09:22 71.248.128.67




RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINE -- CLASS II


______________________________


PRODUCT


a) PRO-LAK, bulk weight, Protein Concentrate for Lactating Dairy Animals, Recall # V-079-6;


b) ProAmino II, FOR PREFRESH AND LACTATING COWS, net weight 50lb (22.6 kg), Recall # V-080-6;


c) PRO-PAK, MARINE & ANIMAL PROTEIN CONCENTRATE FOR USE IN ANIMAL FEED, Recall # V-081-6;


d) Feather Meal, Recall # V-082-6 CODE


a) Bulk


b) None


c) Bulk


d) Bulk


RECALLING FIRM/MANUFACTURER H. J. Baker & Bro., Inc., Albertville, AL, by telephone on June 15, 2006 and by press release on June 16, 2006. Firm initiated recall is ongoing.


REASON


Possible contamination of animal feeds with ruminent derived meat and bone meal.


VOLUME OF PRODUCT IN COMMERCE 10,878.06 tons




DISTRIBUTION Nationwide




END OF ENFORCEMENT REPORT FOR July 12, 2006


###







-------- Original Message --------


Subject: MAD COW FEED BAN WARNING LETTERS JULY 20, 2004 USA Date: Tue, 20 Jul 2004 09:14:11 -0500 From: "Terry S. Singeltary Sr." Reply-To: Bovine Spongiform Encephalopathy To: BSE-L@uni-karlsruhe.de


######## Bovine Spongiform Encephalopathy #########




USA BSE/TSE TRIPLE FIREWALLS SEEPING IN 2004...TSS




Public Health Service Food and Drug Administration


San Francisco District 1431 Harbor Bay Parkway Alameda, CA 94502-7070 Telephone: 510/337-6700


VIA HAND DELIVERY


Our Reference No. 1000123954




June 23, 2004





Ronald M. Foster, Manager Randall C. Boyce, Manager Trevor O. Foster, Manager George P. Foster, Manager Fresno Farming LLC P.O. Box 457 1000 Davis Street Livingston, California





WARNING LETTER




Dear Mssrs. Foster, Boyce, Foster, and Foster:




The U.S. Food and Drug Administration (FDA) conducted an inspection of your medicated animal feed mill operation, Fresco Farming LLC, located in Traver, California from April 14, 2004 through May 6, 2004, and found significant deviations from the requirements set forth in Title 21, Code of Federal Regulations, Section 589.2000 (21 C.F.R. 589.2000) - Animal Proteins Prohibited in Ruminant Feed. The regulation is intended to prevent the establishment and amplification of Bovine Spongiform Encephalopathy (BSE). Because you failed to follow this rule, products you manufactured and distributed are adulterated within the meaning of Section 402(a)(4) of the Federal Food, Drug, and Cosmetic Act (the Act) because they were prepared, packed, or held under insanitary conditions whereby they may have been rendered injurious to health.




Our inspection found the following violations of 21 C.F.R. 589.2000:




1. Failure to provide for measures to avoid commingling or cross-contamination of products that contain or may contain protein derived from mammalian tissues into animal protein or feeds that may be used for ruminants to comply with 21 C.F.R. 589.2000(e)(1)(iii).




* Your firm uses a vacuum system to clean up spilled product in the tunnel area. This tunnel area houses the two receiving conveyor systems and the elevators for the two conveyor systems. When product, including ruminant meat and bone meal, is spilled onto the floor of this area, the spilled product is vacuumed up by the vacuum system and, via a discharge hose, was placed into a conveyor system that your firm had designated as free of ruminant meat and bone meal. Your firm admitted that it was unaware of the vacuum system discharging into the conveyor systems designated as free of ruminant meat and bone meal and that this had been in place since April 2003. Your firm remedied this problem during FDA s April/May 2004 inspection by removing the discharge hose connection to the conveyer system that your firm had designated as free of ruminant meat and bone meal .


* Your firm uses a dust collection system that pulls dust from systems that receive both ruminant meat and bone meal and feed ingredients intended for ruminants. This dust system then discharged collected product back into the two conveyor systems via a cross connection, thereby making it likely that ruminant meat and bone meal became commingled with ruminant feed ingredients. Your firm admitted that it was unaware of the cross connection and that it had been in place since April 2003. Your firm removed the cross connection during FDA s April/May 2004 inspection.






2. Failure to maintain written procedures specifying the clean-out procedure or other means, and specifying the procedures for separating products that contain or may contain protein derived from mammalian tissue from all other protein products from the time of receipt until the time of shipment, to comply with 21 C.F.R. 589.2000(e)(1)(iv). This observation was also noted during FDA s July/August 2003 inspection of your firm.




* There are no written procedures for separating products that contain prohibited material from ingredients used in ruminant feeds from the time of receipt until the time of shipment.




* The written procedure for cleaning out or flushing equipment after mixing feeds containing prohibited material was not adequate to prevent contamination of ruminant feed with prohibited material.






3. Failure to maintain records sufficient to track materials that contain protein derived from mammalian tissues throughout their receipt, processing, and distribution to comply with 21 C.F.R. 589.2000(e)(1)(i). This observation was also noted during FDA s July/August 2003 inspection of your firm.






* Specifically, your firm has failed to develop and implement complete written procedures to separate ruminant meat and bone meal from feed ingredients intended for ruminants from the time of receipt until the time of distribution. The written procedures that do exist fail to address the use of equipment common to ruminant meat and bone meal and ruminant feed ingredients.






The above is not intended to be an all-inclusive list of deficiencies at your facility. As a manufacturer of materials intended for use as animal feed, you are responsible for assuring that your overall operation and the products you manufacture and distribute are in compliance with the law. You should take prompt action to correct these violations, and you should establish a system whereby such violations do not recur. Failure to promptly correct these violations may result in regulatory action without further notice, such as seizure and/or injunction.






You should notify this office in writing within fifteen (15) working days of receiving this letter of the steps you have taken to bring your firm into compliance with the law. Your response should include an explanation of each step being taken to correct the violations and prevent their recurrence. If corrective actions cannot be completed in fifteen (15) working days, state the reason for the delay and the date by which the corrections will be completed. Include copies of any available documentation demonstrating that corrections have been made.






Please send your reply to the U.S. Food and Drug Administration, Attention: Ms. Harumi Kishida, Compliance Officer, 1431 Harbor Bay Parkway, Alameda, California 94502-7070. If you have questions regarding this letter, please contact Ms. Kishida at (510) 337-6824.






Sincerely,




/s/




CD Moss, Acting DD for Barbara J. Cassens District Director San Francisco District




cc:




VIA CERTIFIED MAIL RETURN RECEIPT REQUESTED C. Michael Blasco, Feed Mill Manager Fresno Farming LLC P.O. Box 430 Traver, California 93673












Surveillance for BSE in California


Surveillance for BSE began in 1990. California collected 560 samples in 2001 and approximately 2,000 in 2002 and in 2003. The US sampled 20,543 cattle in 2003 - a sample size designed to detect BSE if it occurred in 1 animal per million adult cattle with a 95% confidence rate. This sample size is more that 47 times the international standard for countries with a “low risk” of BSE.


California is working with the USDA and the cattle industry to determine the best way to enhance BSE surveillance and test as many “high-risk” cattle as possible for 12-18 months. Brain samples will be collected from cattle over 30 months of age that are:


Non-ambulatory (cannot rise or cannot walk)


Showing neurological signs


Condemned, euthanized or died following signs that may be associated with BSE


Dead from unknown cause.


In addition, a random sample of apparently healthy aged cattle will be sampled at California slaughter facilities.


Sample Tests


There are no tests that detect BSE in live animals. Current tests look for the abnormal prion protein in the brain. Two rapid screening tests have recently been licensed for use in the US; Bio-Rad Laboratories rapid TeSeE® test and Idexx HerdChek(R) BSE Antigen Test Kit.


Sensitive screening tests may give false positive results - samples positive to these BSE screening tests will be sent for further confirmatory testing at the national reference laboratory.







P.4.23


Transmission of atypical BSE in humanized mouse models


Liuting Qing1, Wenquan Zou1, Cristina Casalone2, Martin Groschup3, Miroslaw Polak4, Maria Caramelli2, Pierluigi Gambetti1, Juergen Richt5, Qingzhong Kong1 1Case Western Reserve University, USA; 2Instituto Zooprofilattico Sperimentale, Italy; 3Friedrich-Loeffler-Institut, Germany; 4National Veterinary Research Institute, Poland; 5Kansas State University (Previously at USDA National Animal Disease Center), USA


Background: Classical BSE is a world-wide prion disease in cattle, and the classical BSE strain (BSE-C) has led to over 200 cases of clinical human infection (variant CJD). Atypical BSE cases have been discovered in three continents since 2004; they include the L-type (also named BASE), the H-type, and the first reported case of naturally occurring BSE with mutated bovine PRNP (termed BSE-M). The public health risks posed by atypical BSE were largely undefined.


Objectives: To investigate these atypical BSE types in terms of their transmissibility and phenotypes in humanized mice. Methods: Transgenic mice expressing human PrP were inoculated with several classical (C-type) and atypical (L-, H-, or Mtype) BSE isolates, and the transmission rate, incubation time, characteristics and distribution of PrPSc, symptoms, and histopathology were or will be examined and compared.


Results: Sixty percent of BASE-inoculated humanized mice became infected with minimal spongiosis and an average incubation time of 20-22 months, whereas only one of the C-type BSE-inoculated mice developed prion disease after more than 2 years. Protease-resistant PrPSc in BASE-infected humanized Tg mouse brains was biochemically different from bovine BASE or sCJD. PrPSc was also detected in the spleen of 22% of BASE-infected humanized mice, but not in those infected with sCJD. Secondary transmission of BASE in the humanized mice led to a small reduction in incubation time.*** The atypical BSE-H strain is also transmissible with distinct phenotypes in the humanized mice, but no BSE-M transmission has been observed so far.


Discussion: Our results demonstrate that BASE is more virulent than classical BSE, has a lymphotropic phenotype, and displays a modest transmission barrier in our humanized mice. BSE-H is also transmissible in our humanized Tg mice. The possibility of more than two atypical BSE strains will be discussed.


Supported by NINDS NS052319, NIA AG14359, and NIH AI 77774.









MAD COW USDA ATYPICAL L-TYPE BASE BSE, the rest of the story...


***Oral Transmission of L-type Bovine Spongiform Encephalopathy in Primate Model






***Infectivity in skeletal muscle of BASE-infected cattle






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






***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans.






full text ;


atypical L-type BASE BSE






Tuesday, May 1, 2012


BSE MAD COW LETTERS TO USDA (Tom Vilsack, Secretary of Agriculture) and FDA (Magaret Hamburg, Commissioner of FDA) May 1, 2012






Wednesday, May 2, 2012


ARS FLIP FLOPS ON SRM REMOVAL FOR ATYPICAL L-TYPE BASE BSE RISK HUMAN AND ANIMAL HEALTH






Friday, May 4, 2012


May 2, 2012: Update from APHIS Regarding a Detection of Bovine Spongiform Encephalopathy (BSE) in the United States
















Sunday, March 11, 2012


APHIS Proposes New Bovine Spongiform Encephalopathy Import Regulations in Line with International Animal Health Standards Proposal Aims to Ensure Health of the U.S. Beef Herd, Assist in Negotiations






Wednesday, April 4, 2012


Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products APHIS-2008-0010-0008 RIN:0579-AC68






Sunday, May 6, 2012


Bovine Spongiform Encephalopathy Mad Cow Disease, BSE May 2, 2012 IOWA State University OIE








SPONTANEOUS ??? NOT...


How the California cow got the disease remains unknown. Government officials expressed confidence that contaminated food was not the source, saying the animal had atypical L-type BSE, a rare variant not generally associated with an animal consuming infected feed.


However, a BSE expert said that consumption of infected material is the only known way that cattle get the disease under natural conditons.


“In view of what we know about BSE after almost 20 years experience, contaminated feed has been the source of the epidemic,” said Paul Brown, a scientist retired from the National Institute of Neurological Diseases and Stroke.


BSE is not caused by a microbe. It is caused by the misfolding of the so-called “prion protein” that is a normal constituent of brain and other tissues. If a diseased version of the protein enters the brain somehow, it can slowly cause all the normal versions to become misfolded.


It is possible the disease could arise spontaneously, though such an event has never been recorded, Brown said.






Friday, May 11, 2012


Experimental H-type bovine spongiform encephalopathy characterized by plaques and glial- and stellate-type prion protein deposits


In addition, the present data will support risk assessments in some peripheral tissues derived from cattle affected with H-type BSE.














Wednesday, May 16, 2012


Independent experts should be kept from undue suspicion as well as undue influence


IN REPLY TO ;














TSS


Independent experts should be kept from undue suspicion as well as undue influence

IN REPLY TO ;


Needless conflict NatureVolume: 485 Pages: 279–280 Date published: (17 May 2012)


Needless conflict Nature 485,279–280(17 May 2012)doi:10.1038/485279bPublished online 16 May 2012


Independent experts should be kept from undue suspicion as well as undue influence.


Subject terms: Health and medicine Ethics


Article tools Print Email Download PDF View in readcube Download citation Order reprints Rights and permissions


Share/bookmark




We are what we eat. So it should come as no surprise that food-related issues such as bovine spongiform encephalopathy (BSE), bisphenol A contamination, foot-and-mouth disease, Escherichia coli outbreaks and genetic modification resonate with the public. It is unfortunate, then, that discussion of them is often clouded by controversies over the impartiality of scientific advice and whether government regulations are truly unaffected by industry interests.


Questions of food safety, nutrition and agriculture elicit more emotion and public mistrust than almost any other science-based issue. The firestorm over obesity, for example, ignited once again in the United States last week, when the Institute of Medicine issued a report of nearly 500 pages that makes a compelling case that individual choice is not sufficient to prevent obesity in the current environment of inexpensive high-calorie foods and drinks. The report recommends that industry and government take action to get cheap healthy foods into supermarkets and schools, and that the government intervene to ensure that the right dietary messages get through the flood of advertising. The report, of course, was criticized by the industry forces that would have the most to lose if such changes were implemented.


In this highly charged environment, a controversy over alleged conflicts of interest at the top of the European Food Safety Authority (EFSA) has led to media headlines, criticisms from the European Parliament and a feeding frenzy by some non-governmental organizations critical of EFSA (see page 294). Some of those rushing to judge EFSA might do well to remember, however, that whatever the body's shortcomings, it represents a marked improvement on what went before.


EFSA, which is based in Parma, Italy, was created in 2002 in the wake of the BSE scandal and other food crises. Public confidence in experts and governments had evaporated after it emerged that contaminated beef could cause new variant Creutzfeldt–Jakob disease in humans. At fault was a system in which economic imperatives too often blinkered experts and government ministries — not least departments of agriculture — in their assessment of risks and precautions. EFSA was created to change all that, as an independent agency that would provide scientific advice to the European Union and its member states, entirely separate from those responsible for making decisions. Not even the US Food and Drug Administration enjoys that degree of potential freedom from interference: it uses advisory panels of outside experts, but is ultimately part of a government department. This was made clear last year, when President Barack Obama's administration overruled the agency's decision to make the contraceptive Plan B One-Step (levonorgestrel) available to girls under 17 without a prescription (see Nature 480, 413; 2011).


The powerful agrofood industry will always seek to influence policy, whether within EFSA, or in the European Commission, the European Parliament and national ministries that actually make the decisions. As in other technological industries, many experts have industry links, and scientists' own perceptions of risk can be biased by a pro-technology outlook that might, for example, lead them to be too enthusiastic about certain transgenic crops.


“Overseers must take care not to unfairly tar the reputations of scientific experts.”


The safeguards against influence and bias should be the same everywhere: comprehensive and timely declaration of potential competing interests, transparency in decision-making, open airing of dissenting opinions and credible independent oversight. EFSA has taken many steps to implement such safeguards, and there seems to be little evidence that it is more affected than any other food-safety body by undue interest.


The media, non-governmental organizations and elected representatives and their institutions all have important oversight roles. But they also have a responsibility to keep concerns in perspective, and to avoid using them to further personal agendas. Overseers must take care not to unfairly tar the reputations of the many scientific experts who give their time generously and in complete independence to further public-health and science-based decision-making.


The public response to the 2009 swine-flu pandemic points to the risks of unsubstantiated suspicion of scientific advice. There were many wild claims that the medical response to the pandemic was being promoted by industry and industry-influenced experts to sell flu drugs and vaccines. This not only helped to fuel conspiracy theories that the pandemic was a hoax, but also diminished public confidence in health authorities at a time when it was sorely needed.


Advisory bodies must not tolerate shortcomings in procedures to disclose conflicts of interest, but they must defend themselves against any unfair tarnishing of scientific experts. Damage to reputation is extremely dangerous in a society in which the Internet can quickly convert exaggerated claims into supposed facts, and in a political climate in which 'elites' are often suspect. There is more to responsible oversight than just pointing out the problems — real or perceived.


Independent experts should be kept from undue suspicion as well as undue influence.






I kindly wish to submit the following please ;


ONE need not look any further than the USDA et al, when it comes to ‘undue influence’. I have followed the mad cow USDA debacle ever since the first mad cow was covered up in Texas, let alone the second one that finally took and act of congress and the Honorable Phyllis Fong of the OIG. if not for that, that second mad cow in Texas would have never been confirmed either. then you can move on to the Alabama, and Washington mad cow, and not much has changed since. Still the same old USDA et al. just look at the atypical L-type BASE BSE case in California recently, and the false and misleading statements there from by the USDA et al. NOTHING has changed, except their stories, time and time again.


They claim of all those firewalls in place, BSE surviellance, BSE testing, BSE feed ban, all three of those firewalls have failed terribly in the USA, but yet to hear the USDA et al tell it, everything is O.K., no problem, feed ban in place since August 4, 1997, BUT YET, the USDA et al fail to tell you, this mad cow firewall was nothing than ink on paper, it was a PARTIAL AND VOLUNTARY feed ban to begin with, that up until 2006, the amounts of banned suspect mad cow protein that was going into commerce, was measured in TONNAGE, 2007, the measurements were measured in POUNDS, where in 2007, 10 years, one decade, post partial, and voluntary BSE feed ban, there were 10,000,000 MILLION POUNDS, of banned, suspect mad cow protein, mixed with blood, that went out into commerce. AFTER that mad cow warning letter, the warning letters ceased to exist. they never published anymore that I could find.


They claim the BSE testing was doing it’s job, until they found out that not only their testing techniques were wrong, but they were TESTING HEALTHY CATTLE, THEY NEW DID NOT HAVE BSE. all this again proven by the OIG and the GAO.


They claim the BSE surveillance program worked, again, a lie. Just look at the GAO and OIG reports about that ENHANCED BSE SURVEILLANCE PROGRAM to test only healthy cows, OR, the OBEX ONLY DIAGNOSTIC criteria that was used.


They claim NO link to sporadic CJD, and this is false as well.


IN my opinion, until we get corporate industry out of policy decision making for the USDA, APHIS, FSIS, FDA et al, until that is changed, you will never have any sound science policy making for consumer safety.


they call it GREED $$$


SOURCE REFERENCES


MAD COW USDA ATYPICAL L-TYPE BASE BSE, the rest of the story...


***Oral Transmission of L-type Bovine Spongiform Encephalopathy in Primate Model




***Infectivity in skeletal muscle of BASE-infected cattle




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




***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans.




Friday, May 11, 2012


Experimental H-type bovine spongiform encephalopathy characterized by plaques and glial- and stellate-type prion protein deposits ***In addition, the present data will support risk assessments in some peripheral tissues derived from cattle affected with H-type BSE.






Re: [BSE-L] Experimental H-type bovine spongiform encephalopathy characterized by plaques and glial- and stellate-type prion protein deposits “Unfortunately, a detailed and all-encompassing analysis of neuropathology and topographical distribution of immunolabeled PrPSc in H-type BSE-affected cattle could not be performed, since only the obex region is routinely sampled for BSE surveillance testing and the remaining brain as well as the carcasses are not available in most countries [3,10,12,13,24-27]. Recently, clinical signs and biochemical properties of experimental German H-type BSE cases have been reported [20]. The primary objective of this study was to investigate the transmissibility of H-type BSE, using a field isolate detected in the active surveillance program in Canada [12]. The secondary objective was to extend the knowledge of the topographical distribution and deposition patterns of immunolabeled PrPSc in H-type BSE.”


UPDATED...PLEASE SEE ;


Tuesday, November 02, 2010


BSE - ATYPICAL LESION DISTRIBUTION (RBSE 92-21367) statutory (obex only) diagnostic criteria CVL 1992






SEE FULL TEXT ;






Tuesday, April 24, 2012


MAD COW DISEASE USA 4TH CASE DOCUMENTED ATYPICAL BSE CALIFORNIA






Wednesday, April 25, 2012


4th MAD COW DISEASE U.S.A. CALIFORNIA ATYPICAL L-TYPE BSE 2012






Sunday, May 6, 2012


Bovine Spongiform Encephalopathy Mad Cow Disease, BSE May 2, 2012 IOWA State University OIE




SPONTANEOUS ??? NOT...


How the California cow got the disease remains unknown. Government officials expressed confidence that contaminated food was not the source, saying the animal had atypical L-type BSE, a rare variant not generally associated with an animal consuming infected feed.


However, a BSE expert said that consumption of infected material is the only known way that cattle get the disease under natural conditons. “In view of what we know about BSE after almost 20 years experience, contaminated feed has been the source of the epidemic,” said Paul Brown, a scientist retired from the National Institute of Neurological Diseases and Stroke.


BSE is not caused by a microbe. It is caused by the misfolding of the so-called “prion protein” that is a normal constituent of brain and other tissues. If a diseased version of the protein enters the brain somehow, it can slowly cause all the normal versions to become misfolded. It is possible the disease could arise spontaneously, though such an event has never been recorded, Brown said.




Tuesday, January 17, 2012


Annual report of the Scientific Network on BSE-TSE EFSA-Q-2011-01110 Issued: 20 December 2011






Friday, January 6, 2012


OIE 2012 Training Manual on Wildlife Diseases and Surveillance and TSE Prion disease






Harvard Risk Assessment of Bovine Spongiform Encephalopathy Update, October 31, 2005 INTRODUCTION The United States Department of Agriculture’s Food Safety and Inspection Service (FSIS) held a public meeting on July 25, 2006 in Washington, D.C. to present findings from the Harvard Risk Assessment of Bovine Spongiform Encephalopathy Update, October 31, 2005 (report and model located on the FSIS website:




Comments on technical aspects of the risk assessment were then submitted to FSIS.


Comments were received from Food and Water Watch, Food Animal Concerns Trust (FACT), Farm Sanctuary, R-CALF USA, Linda A Detwiler, and Terry S. Singeltary.


This document provides itemized replies to the public comments received on the 2005 updated Harvard BSE risk assessment. Please bear the following points in mind:




Monday, January 2, 2012


EFSA Minutes of the 6th Meeting of the EFSA Scientific Network on BSE-TSE Brussels, 29-30 November 2011




Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products APHIS-2008-0010-0008 RIN:0579-AC68


Comment from Terry Singeltary Document ID: APHIS-2008-0010-0008 Document Type: Public Submission This is comment on Proposed Rule: Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products Docket ID: APHIS-2008-0010 RIN:0579-AC68


Topics: No Topics associated with this document View Document: More


Document Subtype: Public Comment Status: Posted Received Date: March 22 2012, at 12:00 AM Eastern Daylight Time Date Posted: March 22 2012, at 12:00 AM Eastern Daylight Time Comment Start Date: March 16 2012, at 12:00 AM Eastern Daylight Time Comment Due Date: May 15 2012, at 11:59 PM Eastern Daylight Time Tracking Number: 80fdd617 First Name: Terry Middle Name: S. Last Name: Singeltary City: Bacliff Country: United States State or Province: TX Organization Name: CJD TSE PRION Submitter's Representative: CONSUMERS


Comment: comment submission Document ID APHIS-2008-0010-0001 Greetings USDA, OIE et al, what a difference it makes with science, from one day to the next. i.e. that mad cow gold card the USA once held. up until that fateful day in December of 2003, the science of BSE was NO IMPORTS TO USA FROM BSE COUNTRY. what a difference a day makes$ now that the shoe is on the other foot, the USDA via the OIE, wants to change science again, just for trade $ I implore the OIE decision and policy makers, for the sake of the world, to refuse any status quo of the USA BSE risk assessment. if at al, the USA BSE GBR should be raise to BSE GBR IV, for the following reasons. North America is awash with many different TSE Prion strains, in many different species, and they are mutating and spreading. IF the OIE, and whatever policy makers, do anything but raise the risk factor for BSE in North America, they I would regard that to be highly suspicious. IN fact, it would be criminal in my opinion, because the OIE knows this, and to knowingly expose the rest of the world to this dangerous pathogen, would be ‘knowingly’ and ‘willfully’, just for the almighty dollar, once again. I warned the OIE about all this, including the risk factors for CWD, and the fact that the zoonosis potential was great, way back in 2002. THE OIE in collaboration with the USDA, made the legal trading of the atypical Nor-98 Scrapie a legal global commodity. yes, thanks to the OIE and the USDA et al, it’s now legal to trade the atypical Nor-98 Scrapie strain all around the globe. IF you let them, they will do the same thing with atypical BSE and CWD (both strains to date). This with science showing that indeed these TSE prion strains are transmissible. I strenuously urge the OIE et al to refuse any weakening to the USA trade protocols for the BSE TSE prion disease (all strains), and urge them to reclassify the USA with BSE GBR IV risk factor. SEE REFERENCE SOURCES IN ATTACHMENTS


SEE Terry S. Singeltary Sr. Attachment WORD FILE ;








Sunday, March 11, 2012


APHIS Proposes New Bovine Spongiform Encephalopathy Import Regulations in Line with International Animal Health Standards Proposal Aims to Ensure Health of the U.S. Beef Herd, Assist in Negotiations




L-BSE, TME, AND SPORADIC CJD aka mad cow disease in North America


Moreover, transmission experiments to non-human primates suggest that some TSE agents in addition to Classical BSE prions in cattle (namely L-type Atypical BSE, Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic wasting disease (CWD) agents) might have zoonotic potential.


snip...






Thursday, August 12, 2010


Seven main threats for the future linked to prions


First threat


The TSE road map defining the evolution of European policy for protection against prion diseases is based on a certain numbers of hypotheses some of which may turn out to be erroneous. In particular, a form of BSE (called atypical Bovine Spongiform Encephalopathy), recently identified by systematic testing in aged cattle without clinical signs, may be the origin of classical BSE and thus potentially constitute a reservoir, which may be impossible to eradicate if a sporadic origin is confirmed.


***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases.


Second threat


snip...






Saturday, June 25, 2011


Transmissibility of BSE-L and Cattle-Adapted TME Prion Strain to Cynomolgus Macaque


Transmissibility of BSE-L and Cattle-Adapted TME Prion Strain to Cynomolgus Macaque


Emmanuel Comoy,1,† Valérie Durand,1 Evelyne Correia,1 Sophie Freire,1 Jürgen Richt,2 Justin Greenlee,3 Juan-Maria Torres,4 Paul Brown,1 Bob Hills5 and Jean-Philippe Deslys1


1Atomic Energy Commission; Fontenay-aux-Roses, France; 2Kansas State University; Manhattan, KS USA; 3USDA; Ames, IA USA; 4INIA; Madrid, Spain; 5Health Canada; Ottawa, ON Canada†Presenting author; Email: emmanuel.comoy@cea.fr


The epidemiology of Transmissible mink encephalopathy (TME) indicates an alimentary origin. Several inter-species transmission experiments have not succeeded in establishing with certainty any natural reservoir of this prion strain, although both ovine and bovine sources have been suspected. Cattle exposed to TME develop a spongiform encephalopathy that is distinct from classical Bovine Spongiform Encephalopathy (c-BSE).


Inoculation of c-BSE to cynomolgus macaque provided early evidence of a possible risk to humans, and remains an important model to define the risk of both primary (oral transmission from cattle to primate) and secondary (intravenous intra-species transmission) exposures. We have also evaluated the transmissibility of other cattle prion strains to macaques, including L- and H- atypical forms of BSE, namely BSE-L and BSE-H, and cattle-adapted TME.


BSE-L induced a neurological disease distinct from c-BSE. Peripheral exposures demonstrate the transmissibility of BSE-L by oral, intravenous, and intra-cerebral routes, with incubation periods similar to c-BSE. Cattle-adapted TME also induced a rapid disease in cynomolgus macaque. The clinical features, lesion profile, and biochemical signature of the induced disease was similar to the features observed in animals exposed to BSE-L, suggesting a link between the two prion strains. Secondary transmissions to a common host (transgenic mouse overexpressing bovine PrP) of cattle-TME and BSE-L before or after passage in primates induced diseases with similar incubation periods: like the c-BSE strain, these cattle strains maintained their distinctive features regardless of the donor species and passages.


If the link between TME and BSE-L is confirmed, our results would suggest that BSE-L in North America may have existed for decades, and highlight a possible preferential transmission of animal prion strains to primates after passage in cattle.




=====================end...tss====================




link url not available, please see PRION 2011 ;






Volume 13, Number 12–December 2007 Research


Phenotypic Similarity of Transmissible Mink Encephalopathy in Cattle and L-type Bovine Spongiform Encephalopathy in a Mouse Model


Thierry Baron,* Anna Bencsik,* Anne-Gaëlle Biacabe,* Eric Morignat,* andRichard A. Bessen†*Agence Française de Sécurité Sanitaire des Aliments–Lyon, Lyon, France; and†Montana State University, Bozeman, Montana, USA


Abstract


Transmissible mink encepholapathy (TME) is a foodborne transmissible spongiform encephalopathy (TSE) of ranch-raised mink; infection with a ruminant TSE has been proposed as the cause, but the precise origin of TME is unknown. To compare the phenotypes of each TSE, bovine-passaged TME isolate and 3 distinct natural bovine spongiform encephalopathy (BSE) agents (typical BSE, H-type BSE, and L-type BSE) were inoculated into an ovine transgenic mouse line (TgOvPrP4). Transgenic mice were susceptible to infection with bovine-passaged TME, typical BSE, and L-type BSE but not to H-type BSE. Based on survival periods, brain lesions profiles, disease-associated prion protein brain distribution, and biochemical properties of protease-resistant prion protein, typical BSE had a distint phenotype in ovine transgenic mice compared to L-type BSE and bovine TME.The similar phenotypic properties of L-type BSE and bovine TME in TgOvPrP4 mice suggest that L-type BSE is a much more likely candidate for the origin of TME than is typical BSE.


snip...


Conclusion


These studies provide experimental evidence that the Stetsonville TME agent is distinct from typical BSE but has phenotypic similarities to L-type BSE in TgOvPrP4 mice. Our conclusion is that L-type BSE is a more likely candidate for a bovine source of TME infection than typical BSE. In the scenario that a ruminant TSE is the source for TME infection in mink, this would be a second example of transmission of a TSE from ruminants to non-ruminants under natural conditions or farming practices in addition to transmission of typical BSE to humans, domestic cats, and exotic zoo animals(37). The potential importance of this finding is relevant to L-type BSE, which based on experimental transmission into humanized PrP transgenic mice and macaques, suggests that L-type BSE is more pathogenic for humans than typical BSE (24,38).






PLEASE NOTE *


Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.


snip...


The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...






Thursday, March 29, 2012


atypical Nor-98 Scrapie has spread from coast to coast in the USA 2012


NIAA Annual Conference April 11-14, 2011San Antonio, Texas






LET’S look at that mad cow feed firewall the USDA and FDA et al speak so highly of. now remember, .005 grams is lethal. ...




10,000,000+ LBS. of PROHIBITED BANNED MAD COW FEED I.E. BLOOD LACED MBM IN COMMERCE USA 2007


Date: March 21, 2007 at 2:27 pm PST


RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINES -- CLASS II


PRODUCT


Bulk cattle feed made with recalled Darling's 85% Blood Meal, Flash Dried, Recall # V-024-2007


CODE


Cattle feed delivered between 01/12/2007 and 01/26/2007


RECALLING FIRM/MANUFACTURER


Pfeiffer, Arno, Inc, Greenbush, WI. by conversation on February 5, 2007.


Firm initiated recall is ongoing.


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


___________________________________


PRODUCT


Custom dairy premix products: MNM ALL PURPOSE Pellet, HILLSIDE/CDL Prot- Buffer Meal, LEE, M.-CLOSE UP PX Pellet, HIGH DESERT/ GHC LACT Meal, TATARKA, M CUST PROT Meal, SUNRIDGE/CDL PROTEIN Blend, LOURENZO, K PVM DAIRY Meal, DOUBLE B DAIRY/GHC LAC Mineral, WEST PIONT/GHC CLOSEUP Mineral, WEST POINT/GHC LACT Meal, JENKS, J/COMPASS PROTEIN Meal, COPPINI - 8# SPECIAL DAIRY Mix, GULICK, L-LACT Meal (Bulk), TRIPLE J - PROTEIN/LACTATION, ROCK CREEK/GHC MILK Mineral, BETTENCOURT/GHC S.SIDE MK-MN, BETTENCOURT #1/GHC MILK MINR, V&C DAIRY/GHC LACT Meal, VEENSTRA, F/GHC LACT Meal, SMUTNY, A- BYPASS ML W/SMARTA, Recall # V-


025-2007


CODE


The firm does not utilize a code - only shipping documentation with commodity and weights identified.


RECALLING FIRM/MANUFACTURER


Rangen, Inc, Buhl, ID, by letters on February 13 and 14, 2007. Firm initiated recall is complete.


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






2006 HOW ABOUT THAT ALABAMA MAD COW FEED THAT WAS FED TO THE ALABAMA MAD COW ???


P.9.21


Molecular characterization of BSE in Canada


Jianmin Yang1, Sandor Dudas2, Catherine Graham2, Markus Czub3, Tim McAllister1, Stefanie Czub1 1Agriculture and Agri-Food Canada Research Centre, Canada; 2National and OIE BSE Reference Laboratory, Canada; 3University of Calgary, Canada


Background: Three BSE types (classical and two atypical) have been identified on the basis of molecular characteristics of the misfolded protein associated with the disease. To date, each of these three types have been detected in Canadian cattle.


Objectives: This study was conducted to further characterize the 16 Canadian BSE cases based on the biochemical properties of there associated PrPres. Methods: Immuno-reactivity, molecular weight, glycoform profiles and relative proteinase K sensitivity of the PrPres from each of the 16 confirmed Canadian BSE cases was determined using modified Western blot analysis.


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


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




what about that ALABAMA MAD COW, AND MAD COW FEED THERE FROM IN THAT STATE ???


2006 was a banner year for banned suspect mad cow protein in commerce. in 2006, it was measured in TONNAGE...


*** (see mad cow feed in COMMERCE IN ALABAMA...TSS)


BANNED MAD COW FEED IN COMMERCE IN ALABAMA


Date: September 6, 2006 at 7:58 am PST PRODUCT


a) EVSRC Custom dairy feed, Recall # V-130-6;


b) Performance Chick Starter, Recall # V-131-6;


c) Performance Quail Grower, Recall # V-132-6;


d) Performance Pheasant Finisher, Recall # V-133-6.


CODE None RECALLING FIRM/MANUFACTURER Donaldson & Hasenbein/dba J&R Feed Service, Inc., Cullman, AL, by telephone on June 23, 2006 and by letter dated July 19, 2006. Firm initiated recall is complete.


REASON


Dairy and poultry feeds were possibly contaminated with ruminant based protein.


VOLUME OF PRODUCT IN COMMERCE 477.72 tons


DISTRIBUTION AL


______________________________




PRODUCT Bulk custom dairy pre-mixes,


Recall # V-120-6 CODE None RECALLING FIRM/MANUFACTURER Ware Milling Inc., Houston, MS, by telephone on June 23, 2006. Firm initiated recall is complete. REASON Possible contamination of dairy animal feeds with ruminant derived meat and bone meal.


VOLUME OF PRODUCT IN COMMERCE 350 tons


DISTRIBUTION AL and MS


______________________________


PRODUCT


a) Tucker Milling, LLC Tm 32% Sinking Fish Grower, #2680-Pellet, 50 lb. bags, Recall # V-121-6;


b) Tucker Milling, LLC #31120, Game Bird Breeder Pellet, 50 lb. bags, Recall # V-122-6;


c) Tucker Milling, LLC #31232 Game Bird Grower, 50 lb. bags, Recall # V-123-6;


d) Tucker Milling, LLC 31227-Crumble, Game Bird Starter, BMD Medicated, 50 lb bags, Recall # V-124-6;


e) Tucker Milling, LLC #31120, Game Bird Breeder, 50 lb bags, Recall # V-125-6;


f) Tucker Milling, LLC #30230, 30 % Turkey Starter, 50 lb bags, Recall # V-126-6;


g) Tucker Milling, LLC #30116, TM Broiler Finisher, 50 lb bags, Recall # V-127-6


CODE All products manufactured from 02/01/2005 until 06/20/2006 RECALLING FIRM/MANUFACTURER Recalling Firm: Tucker Milling LLC, Guntersville, AL, by telephone and visit on June 20, 2006, and by letter on June 23, 2006. Manufacturer: H. J. Baker and Brothers Inc., Stamford, CT. Firm initiated recall is ongoing.


REASON Poultry and fish feeds which were possibly contaminated with ruminant based protein were not labeled as "Do not feed to ruminants".


VOLUME OF PRODUCT IN COMMERCE 7,541-50 lb bags


DISTRIBUTION AL, GA, MS, and TN


END OF ENFORCEMENT REPORT FOR AUGUST 9, 2006


###




Subject: MAD COW FEED RECALL AL AND FL VOLUME OF PRODUCT IN COMMERCE 125 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


###




MAD COW FEED RECALL USA EQUALS 10,878.06 TONS NATIONWIDE Sun Jul 16, 2006 09:22 71.248.128.67


RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINE -- CLASS II


______________________________


PRODUCT


a) PRO-LAK, bulk weight, Protein Concentrate for Lactating Dairy Animals, Recall # V-079-6;


b) ProAmino II, FOR PREFRESH AND LACTATING COWS, net weight 50lb (22.6 kg), Recall # V-080-6;


c) PRO-PAK, MARINE & ANIMAL PROTEIN CONCENTRATE FOR USE IN ANIMAL FEED, Recall # V-081-6;


d) Feather Meal, Recall # V-082-6 CODE


a) Bulk


b) None


c) Bulk


d) Bulk


RECALLING FIRM/MANUFACTURER H. J. Baker & Bro., Inc., Albertville, AL, by telephone on June 15, 2006 and by press release on June 16, 2006. Firm initiated recall is ongoing.


REASON


Possible contamination of animal feeds with ruminent derived meat and bone meal.


VOLUME OF PRODUCT IN COMMERCE 10,878.06 tons


DISTRIBUTION Nationwide


END OF ENFORCEMENT REPORT FOR July 12, 2006


###






Saturday, July 23, 2011


CATTLE HEADS WITH TONSILS, BEEF TONGUES, SPINAL CORD, SPECIFIED RISK MATERIALS (SRM's) AND PRIONS, AKA MAD COW DISEASE




Saturday, November 6, 2010


TAFS1 Position Paper on Position Paper on Relaxation of the Feed Ban in the EU Berne, 2010 TAFS


INTERNATIONAL FORUM FOR TRANSMISSIBLE ANIMAL DISEASES AND FOOD SAFETY a non-profit Swiss Foundation




NOW, what about that mad cow BSE surveillance and testing program ???


PAUL BROWN COMMENT TO ME ON THIS ISSUE


Tuesday, September 12, 2006 11:10 AM


"Actually, Terry, I have been critical of the USDA handling of the mad cow issue for some years, and with Linda Detwiler and others sent lengthy detailed critiques and recommendations to both the USDA and the Canadian Food Agency."


OR, what the Honorable Phyllis Fong of the OIG found ;


Audit Report


Animal and Plant Health Inspection Service


Bovine Spongiform Encephalopathy (BSE) Surveillance Program – Phase II


and


Food Safety and Inspection Service


Controls Over BSE Sampling, Specified Risk Materials, and Advanced Meat Recovery Products - Phase III


Report No. 50601-10-KC January 2006


Finding 2 Inherent Challenges in Identifying and Testing High-Risk Cattle Still Remain




""These 9,200 cases were different because brain tissue samples were preserved with formalin, which makes them suitable for only one type of test--immunohistochemistry, or IHC."


THIS WAS DONE FOR A REASON!


THE IHC test has been proven to be the LEAST LIKELY to detect BSE/TSE in the bovine, and these were probably from the most high risk cattle pool, the ones the USDA et al, SHOULD have been testing. ...TSS


USDA 2003


We have to be careful that we don't get so set in the way we do things that we forget to look for different emerging variations of disease. We've gotten away from collecting the whole brain in our systems. We're using the brain stem and we're looking in only one area. In Norway, they were doing a project and looking at cases of Scrapie, and they found this where they did not find lesions or PRP in the area of the obex. They found it in the cerebellum and the cerebrum. It's a good lesson for us. Ames had to go back and change the procedure for looking at Scrapie samples. In the USDA, we had routinely looked at all the sections of the brain, and then we got away from it. They've recently gone back. Dr. Keller: Tissues are routinely tested, based on which tissue provides an 'official' test result as recognized by APHIS.


Dr. Detwiler: That's on the slaughter. But on the clinical cases, aren't they still asking for the brain? But even on the slaughter, they're looking only at the brainstem. We may be missing certain things if we confine ourselves to one area.


snip.............


Dr. Detwiler: It seems a good idea, but I'm not aware of it. Another important thing to get across to the public is that the negatives do not guarantee absence of infectivity. The animal could be early in the disease and the incubation period. Even sample collection is so important. If you're not collecting the right area of the brain in sheep, or if collecting lymphoreticular tissue, and you don't get a good biopsy, you could miss the area with the PRP in it and come up with a negative test. There's a new, unusual form of Scrapie that's been detected in Norway. We have to be careful that we don't get so set in the way we do things that we forget to look for different emerging variations of disease. We've gotten away from collecting the whole brain in our systems. We're using the brain stem and we're looking in only one area. In Norway, they were doing a project and looking at cases of Scrapie, and they found this where they did not find lesions or PRP in the area of the obex. They found it in the cerebellum and the cerebrum. It's a good lesson for us. Ames had to go back and change the procedure for looking at Scrapie samples. In the USDA, we had routinely looked at all the sections of the brain, and then we got away from it. They've recently gone back.


Dr. Keller: Tissues are routinely tested, based on which tissue provides an 'official' test result as recognized by APHIS .


Dr. Detwiler: That's on the slaughter. But on the clinical cases, aren't they still asking for the brain? But even on the slaughter, they're looking only at the brainstem. We may be missing certain things if we confine ourselves to one area.


snip...


FULL TEXT;


Completely Edited Version PRION ROUNDTABLE


Accomplished this day, Wednesday, December 11, 2003, Denver, Colorado


2005






FINAL REPORT 2ND TEXAS MAD COW




Subject: USDA OIG SEMIANNUAL REPORT TO CONGRESS FY 2007 1st Half (bogus BSE sampling FROM HEALTHY USDA CATTLE) Date: June 21, 2007 at 2:49 pm PST


Owner and Corporation Plead Guilty to Defrauding Bovine Spongiform Encephalopathy (BSE) Surveillance Program


An Arizona meat processing company and its owner pled guilty in February 2007 to charges of theft of Government funds, mail fraud, and wire fraud. The owner and his company defrauded the BSE Surveillance Program when they falsified BSE Surveillance Data Collection Forms and then submitted payment requests to USDA for the services. In addition to the targeted sample population (those cattle that were more than 30 months old or had other risk factors for BSE), the owner submitted to USDA, or caused to be submitted, BSE obex (brain stem) samples from healthy USDA-inspected cattle. As a result, the owner fraudulently received approximately $390,000. Sentencing is scheduled for May 2007.


snip...


Topics that will be covered in ongoing or planned reviews under Goal 1 include:


soundness of BSE maintenance sampling (APHIS),


implementation of Performance-Based Inspection System enhancements for specified risk material (SRM) violations and improved inspection controls over SRMs (FSIS and APHIS),


snip...


The findings and recommendations from these efforts will be covered in future semiannual reports as the relevant audits and investigations are completed.


4 USDA OIG SEMIANNUAL REPORT TO CONGRESS FY 2007 1st Half




-MORE Office of the United States Attorney District of Arizona FOR IMMEDIATE RELEASE For Information Contact Public Affairs February 16, 2007 WYN HORNBUCKLE Telephone: (602) 514-7625 Cell: (602) 525-2681


CORPORATION AND ITS PRESIDENT PLEAD GUILTY TO DEFRAUDING GOVERNMENT’S MAD COW DISEASE SURVEILLANCE PROGRAM


PHOENIX -- Farm Fresh Meats, Inc. and Roland Emerson Farabee, 55, of Maricopa, Arizona, pleaded guilty to stealing $390,000 in government funds, mail fraud and wire fraud, in federal district court in Phoenix. U.S. Attorney Daniel Knauss stated, “The integrity of the system that tests for mad cow disease relies upon the honest cooperation of enterprises like Farm Fresh Meats. Without that honest cooperation, consumers both in the U.S. and internationally are at risk. We want to thank the USDA’s Office of Inspector General for their continuing efforts to safeguard the public health and enforce the law.” Farm Fresh Meats and Farabee were charged by Information with theft of government funds, mail fraud and wire fraud. According to the Information, on June 7, 2004, Farabee, on behalf of Farm Fresh Meats, signed a contract with the U.S. Department of Agriculture (the “USDA Agreement”) to collect obex samples from cattle at high risk of mad cow disease (the “Targeted Cattle Population”). The Targeted Cattle Population consisted of the following cattle: cattle over thirty months of age; nonambulatory cattle; cattle exhibiting signs of central nervous system disorders; cattle exhibiting signs of mad cow disease; and dead cattle. Pursuant to the USDA Agreement, the USDA agreed to pay Farm Fresh Meats $150 per obex sample for collecting obex samples from cattle within the Targeted Cattle Population, and submitting the obex samples to a USDA laboratory for mad cow disease testing. Farm Fresh Meats further agreed to maintain in cold storage the sampled cattle carcasses and heads until the test results were received by Farm Fresh Meats.


Evidence uncovered during the government’s investigation established that Farm Fresh Meats and Farabee submitted samples from cattle outside the Targeted Cattle Population. Specifically, Farm Fresh Meats and Farabee submitted, or caused to be submitted, obex samples from healthy, USDA inspected cattle, in order to steal government moneys.


Evidence collected also demonstrated that Farm Fresh Meats and Farabee failed to maintain cattle carcasses and heads pending test results and falsified corporate books and records to conceal their malfeasance. Such actions, to the extent an obex sample tested positive (fortunately, none did), could have jeopardized the USDA’s ability to identify the diseased animal and pinpoint its place of origin. On Wednesday, February 14, 2007, Farm Fresh Meats and Farabee pleaded guilty to stealing government funds and using the mails and wires to effect the scheme. According to their guilty pleas:


(a) Farm Fresh Meats collected, and Farabee directed others to collect, obex samples from cattle outside the Targeted Cattle Population, which were not subject to payment by the USDA;


(b) Farm Fresh Meats 2 and Farabee caused to be submitted payment requests to the USDA knowing that the requests were based on obex samples that were not subject to payment under the USDA Agreement;


(c) Farm Fresh Meats completed and submitted, and Farabee directed others to complete and submit, BSE Surveillance Data Collection Forms to the USDA’s testing laboratory that were false and misleading;


(d) Farm Fresh Meats completed and submitted, and Farabee directed others to complete and submit, BSE Surveillance Submission Forms filed with the USDA that were false and misleading;


(e) Farm Fresh Meats falsified, and Farabee directed others to falsify, internal Farm Fresh Meats documents to conceal the fact that Farm Fresh Meats was seeking and obtaining payment from the USDA for obex samples obtained from cattle outside the Targeted Cattle Population; and


(f) Farm Fresh Meats failed to comply with, and Farabee directed others to fail to comply with, the USDA Agreement by discarding cattle carcasses and heads prior to receiving BSE test results. A conviction for theft of government funds carries a maximum penalty of 10 years imprisonment. Mail fraud and wire fraud convictions carry a maximum penalty of 20 years imprisonment. Convictions for the above referenced violations also carry a maximum fine of $250,000 for individuals and $500,000 for organizations. In determining an actual sentence, Judge Earl H. Carroll will consult the U.S. Sentencing Guidelines, which provide appropriate sentencing ranges. The judge, however, is not bound by those guidelines in determining a sentence.


Sentencing is set before Judge Earl H. Carroll on May 14, 2007. The investigation in this case was conducted by Assistant Special Agent in Charge Alejandro Quintero, United States Department of Agriculture, Office of Inspector General. The prosecution is being handled by Robert Long, Assistant U.S. Attorney, District of Arizona, Phoenix. CASE NUMBER: CR-07-00160-PHX-EHC RELEASE NUMBER: 2007-051(Farabee) # # #




Section 2. Testing Protocols and Quality Assurance Controls


In November 2004, USDA announced that its rapid screening test, Bio-Rad Enzyme Linked Immunosorbent Assay (ELISA), produced an inconclusive BSE test result as part of its enhanced BSE surveillance program. The ELISA rapid screening test performed at a BSE contract laboratory produced three high positive reactive results.40 As required,41 the contract laboratory forwarded the inconclusive sample to the APHIS National Veterinary Services Laboratories (NVSL) for confirmatory testing. NVSL repeated the ELISA testing and again produced three high positive reactive results.42 In accordance with its established protocol, NVSL ran its confirmatory test, an immunohistochemistry (IHC) test, which was interpreted as negative for BSE. In addition, NVSL performed a histological43 examination of the tissue and did not detect lesions44 consistent with BSE.


Faced with conflicting results, NVSL scientists recommended additional testing to resolve the discrepancy but APHIS headquarters officials concluded no further testing was necessary because testing protocols were followed. In our discussions with APHIS officials, they justified their decision not to do additional testing because the IHC is internationally recognized as the "gold standard." Also, they believed that conducting additional tests would undermine confidence in USDA’s established testing protocols.






FDA STATEMENT FOR IMMEDIATE RELEASE May 4, 2004 Media Inquiries: 301-827-6242 Consumer Inquiries: 888-INFO-FDA


Statement on Texas Cow With Central Nervous System Symptoms


On Friday, April 30th, the Food and Drug Administration learned that a cow with central nervous system symptoms had been killed and shipped to a processor for rendering into animal protein for use in animal feed.


FDA, which is responsible for the safety of animal feed, immediately began an investigation. On Friday and throughout the weekend, FDA investigators inspected the slaughterhouse, the rendering facility, the farm where the animal came from, and the processor that initially received the cow from the slaughterhouse.


FDA's investigation showed that the animal in question had already been rendered into "meat and bone meal" (a type of protein animal feed). Over the weekend FDA was able to track down all the implicated material. That material is being held by the firm, which is cooperating fully with FDA.


Cattle with central nervous system symptoms are of particular interest because cattle with bovine spongiform encephalopathy or BSE, also known as "mad cow disease," can exhibit such symptoms. In this case, there is no way now to test for BSE. But even if the cow had BSE, FDA's animal feed rule would prohibit the feeding of its rendered protein to other ruminant animals (e.g., cows, goats, sheep, bison).


FDA is sending a letter to the firm summarizing its findings and informing the firm that FDA will not object to use of this material in swine feed only. If it is not used in swine feed, this material will be destroyed. Pigs have been shown not to be susceptible to BSE. If the firm agrees to use the material for swine feed only, FDA will track the material all the way through the supply chain from the processor to the farm to ensure that the feed is properly monitored and used only as feed for pigs.


To protect the U.S. against BSE, FDA works to keep certain mammalian protein out of animal feed for cattle and other ruminant animals. FDA established its animal feed rule in 1997 after the BSE epidemic in the U.K. showed that the disease spreads by feeding infected ruminant protein to cattle.


Under the current regulation, the material from this Texas cow is not allowed in feed for cattle or other ruminant animals. FDA's action specifying that the material go only into swine feed means also that it will not be fed to poultry.


FDA is committed to protecting the U.S. from BSE and collaborates closely with the U.S. Department of Agriculture on all BSE issues. The animal feed rule provides crucial protection against the spread of BSE, but it is only one of several such firewalls. FDA will soon be improving the animal feed rule, to make this strong system even stronger.


#




SEE FULL TEXT OF ALL THIS HERE ;


2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006




ALABAMA MAD COW CASE






Saturday, August 14, 2010


BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY


(see COPIOUS AMOUNTS OF mad cow feed in COMMERCE IN ALABAMA...TSS)




Texas BSE Investigation Final Epidemiology Report August 2005




State-Federal Team Responds to Texas BSE Case


JUNE 30, 2005


(please note 7+ month delay in final confirmation so the BSE MRR policy could be set in stone first. $$$...tss)








SEE ATTEMPTED COVER-UP BEFORE THE END AROUND BY FONG ET AL OF THE O.I.G


The U.S. Department of Agriculture confirmed June 29 that genetic testing had verified bovine spongiform encephalopathy (mad cow disease) in a 12-year-old cow that was born and raised in a Texas beef cattle herd.


Subsequent epidemiological investigations resulted in the culling and testing of 67 adult animals from the index herd. Bio-Rad tests for BSE were conducted on all 67 animals by the National Veterinary Services Laboratory (NVSL) in Ames, Iowa. All tests were negative.


On July 12, Texas officials lifted the quarantine on the source herd. At press time, USDA's Animal and Plant Health Inspection Service was tracing animals of the same age that had left the ranch.


Timeline


The BSE-positive animal was a Brahman-cross cow born and raised in a single Texas herd. The location of the ranch was not disclosed.


On Nov. 11, 2004, the 12-year-old cow was taken to a Texas auction market. Because of its condition, the cow was sent to Champion Pet Foods in Waco, Texas. The company produces several blends of dog food, primarily for the greyhound industry.


On Nov. 15, the animal arrived dead at Champion. Under procedures established by USDA's intensive surveillance program, a sample was sent to the USDA-approved Texas Veterinary Medical Diagnostic Testing Laboratory (TVMDL) at Texas A&M University.


Between June 1, 2004, and June 1, 2005, TVMDL tested nearly 34,000 samples from Texas, New Mexico, Arkansas and Louisiana. They tested the sample from Champion on Nov. 19 using a Bio-Rad ELISA rapid test for BSE. Initial results were inconclusive.


Because of the inconclusive results, a representative from USDA took the entire carcass to TVMDL where it was incinerated. USDA's Animal and Plant Health Inspection Service (APHIS) began tracing the animal and herd.


The sample was then sent to the National Veterinary Services Laboratory for further testing. Two Immunohistochemistry (IHC) tests were conducted and both were negative for BSE. At that point APHIS stopped their trace.


USDA scientists also ran an additional, experimental IHC "rapid" tissue fixation test for academic purposes. This test has not been approved internationally.


Some abnormalities were noted in the experimental test, but because the two approved tests came back negative, the results were not reported beyond the laboratory.


Monitoring by OIG


USDA's Office of Inspector General (OIG) has been monitoring implementation of the BSE expanded surveillance program and evaluating the following:


* Effectiveness of the surveillance program;


* Performance of BSE laboratories in complying with policies and procedures for conducting tests and reporting results;


* Enforcement of the ban on specified risk materials in meat products;


* Controls to prevent central nervous system tissue in advanced meat recovery products;


* Ante mortem condemnation procedures; and


* Procedures for obtaining brain tissue samples from condemned cattle.


While reviewing voluminous records, OIG auditors noticed conflicting test results on one sample-rapid inconclusive, IHC negative, experimental reactive.


Sample retested


At the recommendation of the Inspector General, the sample was retested during the week of June 5 with a second confirmatory test, the Western Blot. The results were reactive.


USDA scientists then conducted an additional IHC confirmatory test, using different antibodies from the November 2004 test. On Friday, June 10, Secretary of Agriculture Mike Johanns publicly announced the results as a "weak positive."


On June 16 an official with USDA's National Veterinary Services Laboratory hand-carried samples for further testing to the Veterinary Laboratory Agency (VLA) in Weybridge, England. Since 1991, the VLA has been a BSE reference laboratory for the World Organization for Animal Health (OIE).


Experts from the Weybridge lab confirmed the accuracy of the results of USDA's November confirmatory IHC test, concurring that the case could not have been confirmed on the basis of this sample. They also examined the November experimental IHC test and interpreted the results to be positive.


Weybridge also conducted additional tests, including IHC, OIE-prescribed Western Blot, NaTTA Western Blot and Prionics Western Blot tests.


To better understand the conflicting results, USDA also conducted Bio-Rad and IDEXX rapid screening tests, IHC and OIE-prescribed Western Blot. USDA also used DNA sequencing to determine the prion protein gene sequence of the animal.




Texas even had a 'secret' test that showed that mad cow positive; experimental IHC test results, because the test was not a validated procedure, and because the two approved IHC tests came back negative, the results were not considered to be of regulatory significance and therefore were not reported beyond the laboratory. . A Western blot test conducted the week of June 5, 2005, returned positive for BSE.




48 hr BSE confirmation turnaround took 7+ months to confirm this case, so the BSE MRR policy could be put into place. ...TSS


-------- Original Message --------


Subject: re-USDA's surveillance plan for BSE aka mad cow disease


Date: Mon, 02 May 2005 16:59:07 -0500


From: "Terry S. Singeltary Sr."


To: paffairs@oig.hhs.gov, HHSTips@oig.hhs.gov, contactOIG@hhsc.state.tx.us


Greetings Honorable Paul Feeney, Keith Arnold, and William Busbyet al at OIG, ...............


snip...


There will be several more emails of my research to follow. I respectfully request a full inquiry into the cover-up of TSEs in the United States of America over the past 30 years. I would be happy to testify...


Thank you, I am sincerely, Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518 xxx xxx xxxx


Date: June 14, 2005 at 1:46 pm PST


In Reply to:


Re: Transcript Ag. Secretary Mike Johanns and Dr. John Clifford, Regarding further analysis of BSE Inconclusive Test Results


posted by TSS on June 13, 2005 at 7:33 pm:


Secretary of Agriculture Ann M. Veneman resigns Nov 15 2004, three days later inclusive Mad Cow is announced. June 7th 2005 Bill Hawks Under Secretary for Marketing and Regulatory Programs resigns. Three days later same mad cow found in November turns out to be positive. Both resignation are unexpected. just pondering... TSS


MAD COW IN TEXAS NOVEMBER 2004. ...TSS


-------- Original Message --------


Subject: Re: BSE 'INCONCLUSIVE' COW from TEXAS ???


Date: Mon, 22 Nov 2004 17:12:15 -0600


From: "Terry S. Singeltary Sr."


To: Carla EverettReferences: [log in to unmask]; [log in to unmask] ;


Greetings Carla, still hear a rumor;


Texas single beef cow not born in Canada no beef entered the food chain?


and i see the TEXAS department of animal health is ramping up for something, but they forgot a url for update?


I HAVE NO ACTUAL CONFIRMATION YET...


can you confirm??? terry


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


-------- Original Message --------


Subject: Re: BSE 'INCONCLUSIVE' COW from TEXAS ???


Date: Fri, 19 Nov 2004 11:38:21 -0600


From: Carla Everett


To: "Terry S. Singeltary Sr."References;[log in to unmask];


The USDA has made a statement, and we are referring all callers to the USDA web site. We have no information about the animal being in Texas.


Carla


At 09:44 AM 11/19/2004, you wrote:


Greetings Carla,


i am getting unsubstantiated claims of this BSE 'inconclusive' cow is from


TEXAS. can you comment on this either way please?


thank you,


Terry S. Singeltary Sr


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


-------- Original Message --------


Subject: Re: BSE 'INCONCLUSIVE' COW from TEXAS ???


Date: Mon, 22 Nov 2004 18:33:20 -0600


From: Carla Everett


To: "Terry S. Singeltary Sr."References: <[log in to unmask]><[log in to unmask] us><[log in to unmask]> <[log in to unmask]us> <[log in to unmask]>


our computer department was working on a place holder we could post USDA's announcement of any results. There are no results to be announced tonight by NVSL, so we are back in a waiting mode and will post the USDA announcement when we hear something.


At 06:05 PM 11/22/2004,


you wrote:


why was the announcement on your TAHC site removed?


Bovine Spongiform Encephalopathy:


November 22: Press Release title here


star image More BSE information


terry


Carla Everett wrote:


no confirmation on the U.S.'inconclusive test...


no confirmation on location of animal. ;


FROM HERE, IT TOOK 7 MONTHS TO CONFIRM THIS MAD COW, while the BSE MRR policy was being bought and sold...(in my opinion...tss)






Saturday, August 16, 2008


Qualitative Analysis of BSE Risk Factors in the United States February 13, 2000 at 3:37 pm PST (BSE red book)




TEXAS OFFICIALS DEAD WRONG ON AMOUNT OF INFECTIVITY TO CAUSE A TSE PRION DISEASE ;


"FDA has determined that each animal could have consumed, at most and in total, five-and-one-half grams – approximately a quarter ounce — of prohibited material. These animals weigh approximately 600 pounds."


5.5 GRAMS OF INFECTIOUS PROHIBITED MAD COW FEED FOR EACH OF THE 1,222 ANIMALS (5.5 GRAMS X 1,222 ANIMALS) IS ENOUGH INFECTIOUS MAD COW FEED TO KILL A SMALL HERD OF COWS...TSS


U.S. Food and Drug Administration FDA News | Today the Food and Drug Administ…U.S. Food and Drug Administration FDA News


Today the Food and Drug Administration announced the results of tests taken on feed used at a Texas feedlot that was suspected of containing meat and bone meal from other domestic cattle — a violation of FDA’s 1997 prohibition on using ruminant material in feed for other ruminants. Results indicate that a very low level of prohibited material was found in the feed fed to cattle.


FDA has determined that each animal could have consumed, at most and in total, five-and-one-half grams – approximately a quarter ounce — of prohibited material. These animals weigh approximately 600 pounds.


It is important to note that the prohibited material was domestic in origin (therefore not likely to contain infected material because there is no evidence of BSE in U.S. cattle), fed at a very low level, and fed only once. The potential risk of BSE to such cattle is therefore exceedingly low, even if the feed were contaminated.


According to Dr. Bernard Schwetz, FDA’s Acting Principal Deputy Commissioner, “The challenge to regulators and industry is to keep this disease out of the United States. One important defense is to prohibit the use of any ruminant animal materials in feed for other ruminant animals. Combined with other steps, like U.S. Department of Agriculture’s (USDA) ban on the importation of live ruminant animals from affected countries, these steps represent a series of protections, to keep American cattle free of BSE.”


Despite this negligible risk, Purina Mills, Inc., is nonetheless announcing that it is voluntarily purchasing all 1,222 of the animals held in Texas and mistakenly fed the animal feed containing the prohibited material. Therefore, meat from those animals will not enter the human food supply. FDA believes any cattle that did not consume feed containing the prohibited material are unaffected by this incident, and should be handled in the beef supply clearance process as usual.


FDA believes that Purina Mills has behaved responsibly by first reporting the human error that resulted in the misformulation of the animal feed supplement and then by working closely with State and Federal authorities.


This episode indicates that the multi-layered safeguard system put into place is essential for protecting the food supply and that continued vigilance needs to be taken, by all concerned, to ensure these rules are followed routinely.


FDA will continue working with USDA as well as State and local officials to ensure that companies and individuals comply with all laws and regulations designed to protect the U.S. food supply.




FOR IMMEDIATE RELEASE P01-05 January 30, 2001 Print Media: 301-827-6242 Consumer Inquiries: 888-INFO-FDA


FDA ANNOUNCES TEST RESULTS FROM TEXAS FEED LOT


Today the Food and Drug Administration announced the results of tests taken on feed used at a Texas feedlot that was suspected of containing meat and bone meal from other domestic cattle -- a violation of FDA's 1997 prohibition on using ruminant material in feed for other ruminants. Results indicate that a very low level of prohibited material was found in the feed fed to cattle.


FDA has determined that each animal could have consumed, at most and in total, five-and-one-half grams - approximately a quarter ounce -- of prohibited material. These animals weigh approximately 600 pounds.


It is important to note that the prohibited material was domestic in origin (therefore not likely to contain infected material because there is no evidence of BSE in U.S. cattle), fed at a very low level, and fed only once. The potential risk of BSE to such cattle is therefore exceedingly low, even if the feed were contaminated.


According to Dr. Bernard Schwetz, FDA's Acting Principal Deputy Commissioner, "The challenge to regulators and industry is to keep this disease out of the United States. One important defense is to prohibit the use of any ruminant animal materials in feed for other ruminant animals. Combined with other steps, like U.S. Department of Agriculture's (USDA) ban on the importation of live ruminant animals from affected countries, these steps represent a series of protections, to keep American cattle free of BSE."


Despite this negligible risk, Purina Mills, Inc., is nonetheless announcing that it is voluntarily purchasing all 1,222 of the animals held in Texas and mistakenly fed the animal feed containing the prohibited material. Therefore, meat from those animals will not enter the human food supply. FDA believes any cattle that did not consume feed containing the prohibited material are unaffected by this incident, and should be handled in the beef supply clearance process as usual.


FDA believes that Purina Mills has behaved responsibly by first reporting the human error that resulted in the misformulation of the animal feed supplement and then by working closely with State and Federal authorities.


This episode indicates that the multi-layered safeguard system put into place is essential for protecting the food supply and that continued vigilance needs to be taken, by all concerned, to ensure these rules are followed routinely.


FDA will continue working with USDA as well as State and local officials to ensure that companies and individuals comply with all laws and regulations designed to protect the U.S. food supply.




PRION 2009 CONGRESS BOOK OF ABSTRACTS


O.4.3


Spread of BSE prions in cynomolgus monkeys (Macaca fascicularis) after oral transmission


Edgar Holznagel1, Walter Schulz-Schaeffer2, Barbara Yutzy1, Gerhard Hunsmann3, Johannes Loewer1 1Paul-Ehrlich-Institut, Federal Institute for Sera and Vaccines, Germany; 2Department of Neuropathology, Georg-August University, Göttingen, Germany, 3Department of Virology and Immunology, German Primate Centre, Göttingen, Germany


Background: BSE-infected cynomolgus monkeys represent a relevant animal model to study the pathogenesis of variant Creutzfeldt-Jacob disease (vCJD).


Objectives: To study the spread of BSE prions during the asymptomatic phase of infection in a simian animal model.


Methods: Orally BSE-dosed macaques (n=10) were sacrificed at defined time points during the incubation period and 7 orally BSE-dosed macaques were sacrificed after the onset of clinical signs. Neuronal and non-neuronal tissues were tested for the presence of proteinase-K-resistant prion protein (PrPres) by western immunoblot and by paraffin-embedded tissue (PET) blot technique.


Results: In clinically diseased macaques (5 years p.i. + 6 mo.), PrPres deposits were widely spread in neuronal tissues (including the peripheral sympathetic and parasympathetic nervous system) and in lymphoid tissues including tonsils. In asymptomatic disease carriers, PrPres deposits could be detected in intestinal lymph nodes as early as 1 year p.i., but CNS tissues were negative until 3 – 4 years p.i. Lumbal/sacral segments of the spinal cord and medulla oblongata were PrPres positive as early as 4.1 years p.i., whereas sympathetic trunk and all thoracic/cervical segments of the spinal cord were still negative for PrPres. However, tonsil samples were negative in all asymptomatic cases.


Discussion: There is evidence for an early spread of BSE to the CNS via autonomic fibres of the splanchnic and vagus nerves indicating that trans-synaptical spread may be a time-limiting factor for neuroinvasion. Tonsils were predominantly negative during the main part of the incubation period indicating that epidemiological vCJD screening results based on the detection of PrPres in tonsil biopsies may mostly tend to underestimate the prevalence of vCJD among humans.




P04.27


Experimental BSE Infection of Non-human Primates: Efficacy of the Oral Route


Holznagel, E1; Yutzy, B1; Deslys, J-P2; Lasmézas, C2; Pocchiari, M3; Ingrosso, L3; Bierke, P4; Schulz-Schaeffer, W5; Motzkus, D6; Hunsmann, G6; Löwer, J1 1Paul-Ehrlich-Institut, Germany; 2Commissariat à l´Energie Atomique, France; 3Instituto Superiore di Sanità, Italy; 4Swedish Institute for Infectious Disease control, Sweden; 5Georg August University, Germany; 6German Primate Center, Germany


Background:


In 2001, a study was initiated in primates to assess the risk for humans to contract BSE through contaminated food. For this purpose, BSE brain was titrated in cynomolgus monkeys.


Aims:


The primary objective is the determination of the minimal infectious dose (MID50) for oral exposure to BSE in a simian model, and, by in doing this, to assess the risk for humans. Secondly, we aimed at examining the course of the disease to identify possible biomarkers.


Methods:


Groups with six monkeys each were orally dosed with lowering amounts of BSE brain: 16g, 5g, 0.5g, 0.05g, and 0.005g. In a second titration study, animals were intracerebrally (i.c.) dosed (50, 5, 0.5, 0.05, and 0.005 mg).


Results:


In an ongoing study, a considerable number of high-dosed macaques already developed simian vCJD upon oral or intracerebral exposure or are at the onset of the clinical phase. However, there are differences in the clinical course between orally and intracerebrally infected animals that may influence the detection of biomarkers.


Conclusions:


Simian vCJD can be easily triggered in cynomolgus monkeys on the oral route using less than 5 g BSE brain homogenate. The difference in the incubation period between 5 g oral and 5 mg i.c. is only 1 year (5 years versus 4 years). However, there are rapid progressors among orally dosed monkeys that develop simian v CJD as fast as intracerebrally inoculated animals.


The work referenced was performed in partial fulfillment of the study “BSE in primates“ supported by the EU (QLK1-2002-01096).




Simian vCJD can be easily triggered in cynomolgus monkeys on the oral route using less than 5 g BSE brain homogenate.




look at the table and you'll see that as little as 1 mg (or 0.001 gm) caused 7% (1 of 14) of the cows to come down with BSE;


Risk of oral infection with bovine spongiform encephalopathy agent in primates


Corinne Ida Lasmézas, Emmanuel Comoy, Stephen Hawkins, Christian Herzog, Franck Mouthon, Timm Konold, Frédéric Auvré, Evelyne Correia, Nathalie Lescoutra-Etchegaray, Nicole Salès, Gerald Wells, Paul Brown, Jean-Philippe Deslys Summary The uncertain extent of human exposure to bovine spongiform encephalopathy (BSE)--which can lead to variant Creutzfeldt-Jakob disease (vCJD)--is compounded by incomplete knowledge about the efficiency of oral infection and the magnitude of any bovine-to-human biological barrier to transmission. We therefore investigated oral transmission of BSE to non-human primates. We gave two macaques a 5 g oral dose of brain homogenate from a BSE-infected cow. One macaque developed vCJD-like neurological disease 60 months after exposure, whereas the other remained free of disease at 76 months. On the basis of these findings and data from other studies, we made a preliminary estimate of the food exposure risk for man, which provides additional assurance that existing public health measures can prevent transmission of BSE to man.


snip...


BSE bovine brain inoculum


100 g 10 g 5 g 1 g 100 mg 10 mg 1 mg 0·1 mg 0·01 mg


Primate (oral route)* 1/2 (50%)


Cattle (oral route)* 10/10 (100%) 7/9 (78%) 7/10 (70%) 3/15 (20%) 1/15 (7%) 1/15 (7%)


RIII mice (ic ip route)* 17/18 (94%) 15/17 (88%) 1/14 (7%)


PrPres biochemical detection


The comparison is made on the basis of calibration of the bovine inoculum used in our study with primates against a bovine brain inoculum with a similar PrPres concentration that was


inoculated into mice and cattle.8 *Data are number of animals positive/number of animals surviving at the time of clinical onset of disease in the first positive animal (%). The accuracy of


bioassays is generally judged to be about plus or minus 1 log. ic ip=intracerebral and intraperitoneal.


Table 1: Comparison of transmission rates in primates and cattle infected orally with similar BSE brain inocula


Published online January 27, 2005




Calves were challenged by mouth with homogenised brain from confirmed cases of BSE. Some received 300g (3 doses of 100g), some 100g, 10g or 1g. They were then left to develop BSE, but were not subjected to the normal stresses that they might have encountered in a dairy herd. Animals in all four groups developed BSE. There has been a considerable spread of incubation period in some of the groups, but it appears as if those in the 1 and 10g challenge groups most closely fit the picture of incubation periods seen in the epidemic. Experiments in progress indicate that oral infection can occur in some animals with doses as low as 0.01g and 0.001g. .........




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 than say 100 grams) was probably given with the benefit of hindsight; particularly if one considers that later in the same answer Mr Bradley expresses his surprise that it could take as little of 1 gram of brain to cause BSE by the oral route within the same species. This information did not become available until the "attack rate" 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 ensure that the actual result was within both a lower and an upper limit within the study and the designing scientists would not have expected all the dose levels to trigger infection. The dose ranges chosen by the most informed scientists at that time ranged from 1 gram to three times one hundred grams. It is clear that the designing scientists must have also shared Mr Bradley's surprise at the results because all the dose levels right down to 1 gram triggered infection.






The beef import forecast for the second quarter was unchanged from last month’s, despite pressure from higher-than-expected domestic cow slaughter that has remained high through most of this period. Beef imports into the United States from Australia, New Zealand, and Uruguay provide additional processing beef that, along with domestic cow and bull beef, is mixed with 50-percent trim from fed cattle to make ground beef. Forecast beef exports were raised slightly, mainly on continued (though gradual) improvements in sales to major Asian markets. In late May the World Animal Health Organization – known by its French acronym, OIE – designated the United States as having “controlled risk status” for bovine spongiform encephalopathy, or BSE. This designation reflects the OIE’s view that beef produced in the United States is safe for export, since BSE control measures such as feed bans and removal of specified risk materials result in negligible risk to consumers. However, the OIE standards are only guidelines. Individual countries may adopt differing standards, and those countries that do accept OIE standards must still undertake the bureaucratic processes to revise their rules and procedures.






Wednesday, April 25, 2012


USA MAD COW DISEASE AND CJD THERE FROM SINGELTARY ET AL 1999 – 2012






TSS