Diagnosis of Methionine/Valine Variant Creutzfeldt-Jakob Disease by Protein Misfolding Cyclic Amplification
Volume 24, Number 7—July 2018
Dispatch
Diagnosis of Methionine/Valine Variant Creutzfeldt-Jakob Disease by Protein Misfolding Cyclic Amplification
Daisy Bougard
, Maxime Bélondrade, Charly Mayran, Lilian Bruyère-Ostells, Sylvain Lehmann, Chantal Fournier-Wirth, Richard S. Knight, Robert G. Will, and Alison J.E. Green
, Maxime Bélondrade, Charly Mayran, Lilian Bruyère-Ostells, Sylvain Lehmann, Chantal Fournier-Wirth, Richard S. Knight, Robert G. Will, and Alison J.E. GreenAbstract
A patient with a heterozygous variant of Creutzfeldt-Jakob disease (CJD) with a methionine/valine genotype at codon 129 of the prion protein gene was recently reported. Using an ultrasensitive and specific protein misfolding cyclic amplification–based assay for detecting variant CJD prions in cerebrospinal fluid, we discriminated this heterozygous case of variant CJD from cases of sporadic CJD.
Until recently, all clinical cases of vCJD for which the prion protein gene has been analyzed have been shown to be methionine homozygous at codon 129, a genotype present in almost 40% of Caucasian populations. The report of the first definite heterozygous methionine/valine vCJD patient who died in 2016 (3) underlined previous concern about a possible second wave of vCJD cases (4). The clinical features of this patient were more similar to those of patients with sporadic CJD (sCJD) than to those with vCJD. This patient had met the agreed surveillance diagnostic criteria for probable sCJD (5). However, vCJD was diagnosed during an autopsy; florid plaques were observed by histologic examination of the brain and peripheral detection of PrPTSE in lymphoid tissues. Western blot analysis of brain tissue confirmed a type 2B molecular profile of PrPTSE, which is characteristic for vCJD.
A diagnostic test to identify methionine/valine heterozygous vCJD cases is urgently needed to enable discrimination between heterozygous vCJD and sCJD and in view of the potential reservoir of methionine/valine heterozygous asymptomatic vCJD carriers in the blood donor population. We developed a highly sensitive and specific assay that accurately detects vCJD prions in blood even before the occurrence of clinical signs (6). We adapted this assay, which was based on protein misfolding cyclic amplification (PMCA) (7), for specific detection of vCJD in cerebrospinal fluid (CSF) and confirmed the ability of this assay to differentiate patients with atypical heterozygous vCJD from patients with sCJD.
We blindly analyzed 98 CSF samples provided by the National CJD Research and Surveillance Unit (Edinburgh, Scotland, UK) and the Centre Hospitalier Universitaire de Montpellier (Montpellier, France) after obtaining appropriate consent. Clinicians distributed CSF samples into blinded panels from the United Kingdom and France; 41 from patients with vCJD; 23 from patients with sCJD; 1 from a patient with genetic CJD; and 33 from patients with non-CJD, including samples from patients with Alzheimer’s disease and patients with nonneurodegenerative diseases.
CSF samples were thawed at room temperature and used directly in PMCA. We performed PMCA amplification by using brains from humanized transgenic mice as substrate for normal prion protein. After successive rounds of 160 cycles of PMCA for 15 min and sonication for 20 s, we detected PrPTSE by using Western blot after digestion with proteinase K (6).
Of the 98 CSF samples analyzed, our assay identified 40 of 41 cases of clinical vCJD, including the methionine/valine heterozygous patient, thus showing a diagnostic sensitivity of 97.6% (95% CI 87.1%–99.9%) (Table). One CSF sample from a probable case of vCJD showed a negative result. After decoding by clinicians, we retested this sample in duplicate; it showed a positive result.
Our assay also showed high analytical specificity; 0 of 57 potentially cross-reacting CSF specimens from patients with sCJD, gCJD, Alzheimer's disease, and other nonneurodegenerative diseases showed a positive result (specificity 100% [95% CI 93.7%–100%]) (Table). The case-patient with methionine/valine heterozygous vCJD was specifically discriminated from the 12 methionine/valine heterozygous neuropathologically confirmed sCJD case-patients tested.
We then compared by using Western blot the PrPTSE molecular signature obtained for the clinical vCJD amplified samples from classical methionine homozygous cases and the new methionine/valine heterozygous vCJD case with that of the reference brain sample from a patient with vCJD (Figure). As expected, the profile obtained after PMCA amplification of the CSF from the methionine/valine heterozygous vCJD patient was similar to those obtained for methionine homozygous vCJD patients. The characteristic type 2 mobility and clear predominance of the diglycosylated isoform was obtained for all vCJD patients before or after amplification.
We report a specific detection method that enables clinical diagnosis of a heterozygous methionine/valine heterozygous vCJD patient. This patient was the first definite heterozygous patient described since the start of the vCJD epidemic in the United Kingdom in 1996 (3). Clinical diagnosis was difficult because clinical signs and symptoms, particularly cerebral appearance by magnetic resonance imaging, were suggestive of sCJD (3). The vCJD blood test (direct detection assay) developed by the Medical Research Council Prion Unit (London, UK) (8) showed a negative result for this case-patient. We found characteristic vCJD prion protein amplification in the CSF, which led to a specific diagnosis of vCJD because sCJD samples did not show positive results by PMCA. This result also demonstrates the possibility of amplifying methionine/valine heterozygous vCJD prion protein by PMCA with a substrate from humanized transgenic mice that overexpress homozygous methionine prion protein (9). However, PMCA analysis should be performed in a Biosafety Level 3 laboratory and requires highly experienced personnel.
Iatrogenic transmission of vCJD by blood transfusion has been documented in 3 recipients of nonleukodepleted erythrocyte concentrates from blood donors during development of disease (10). One additional probable case of vCJD transmission by blood transfusion was identified during an autopsy of a methionine/valine heterozygous patient who died from a nonneurologic disorder and in whom vCJD prion protein was detected in the spleen (11). The presence of infectivity in blood of the definite methionine/valine heterozygous vCJD patient involved in our study is uncertain and requires further investigation.
From a clinical point of view, prion amplification technologies, such as PMCA and real-time quaking-induced conversion (RT-QuIC), have already shown their sensitive detection of disease-related prion protein in biologic fluids (6,12–14). Independent studies have shown that detection of prion protein seeding activity in CSF by RT-QuIC might have a specificity of 99%–100% for diagnosis of sCJD (13,15). Application of RT-QuIC and PMCA for CSF samples might represent a suitable strategy for premortem discrimination between sCJD and vCJD including methionine/valine heterozygous case-patients, particularly for cases with a heterozygous codon 129 genotype in which clinical distinction between sCJD and vCJD is problematic.
Dr. Bougard is a research scientist in charge of the Prion Group at Etablissement Français du Sang of Montpellier, France. Her primary research interests include development of innovative tools for the prevention of transfusion risk associated with nonconventional agents.
Acknowledgments
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Volume 10 Number 26 published on: 12 August 2016
CJD/Emerging infections
Common Creutzfeldt-Jakob disease (CJD) biannual update (August 2016)
This six-monthly report provides an update on the enhanced surveillance of potential iatrogenic (healthcare-acquired) exposures to Creutzfeldt-Jakob Disease (CJD). The data is correct as of 30 June 2016. For numbers of CJD case reports, readers should consult data provided by the National CJD Research and Surveillance Unit (NCJDRSU, http://www.cjd.ed.ac.uk/data.html).
Monitoring of patients 'at increased risk' of CJD
Individuals who have been identified as ‘at increased risk’ of CJD as a consequence of their medical care are informed of their exposure and asked to follow public health precautions to avoid potentially transmitting the infection to others. They are also followed up to help determine the risks of CJD transmission to patients through different routes and to ascertain whether any people who may have been exposed to increased CJD risks go on to develop CJD.
Public Health follow up activities include clinical monitoring, General Practitioner (GP) updates, and post mortem investigations to determine whether asymptomatic individuals in these groups have been infected with the CJD agent. Some individuals also provide blood or tissue specimens for research purposes. A number of different organisations are involved in these activities: Public Health England (PHE), Health Protection Scotland (HPS), UCL Institute of Child Health/Great Ormond Street Hospital (ICH), NHS Blood and Transplant (NHSBT), National CJD Research and Surveillance Unit (NCJDRSU), National Prion Clinic (NPC), and the UK Haemophilia Centre Doctors' Organisation (UKHCDO). The PHE CJD Section coordinates the collation of data on individuals identified as ‘at increased risk’ of CJD, and who have been informed of this. These individuals are followed up through public health monitoring and research activities by different organisations.
The PHE CJD Section currently holds data on the following groups of patients who have been identified as ‘at increased risk’ of CJD:
recipients of blood components from donors who subsequently developed vCJD
blood donors to individuals who later developed vCJD
other recipients of blood components from these blood donors
recipients of certain plasma products between 1990 and 2001 (non-bleeding disorder patients)
certain surgical contacts of patients diagnosed with CJD
highly transfused recipients.
Data on the following risk groups are not held by PHE, but are held by other organisations:
bleeding disorder patients who received plasma products between 1990 and 2001 (UKHCDO)
recipients of human derived growth hormone before 1985 (ICH)
patients who could have received a dura mater graft before August 1992 (data not currently collected)
individuals treated with gonadotrophin sourced from humans before 1973 (data not currently collected)
family risk of genetic prion disease (NPC).
The data from the UKHCDO are likely to be a slight underestimate of the true number of patients with bleeding disorders who received UK-sourced clotting factors (1990 to 2001), as there was incomplete reporting of identified patients by haemophilia centres to the UKHCDO database. Notified patients are given the option of removing their details from the UKHCDO database, and are then removed from the ‘at increased risk' totals.
Health Protection Report Vol. 10 No. 26 – 12 August 2016
The data on patients who received human-derived human growth hormone held by the ICH is also a slight underestimate of the total as a small number of these patients are not included in the ICH followup.
Summary of all ‘at increased risk’ groups on which data are collected.
(Data correct as of 30 June 2015)
'At increased risk' Group Identified as 'at increased risk' Number notified Cases Asymptomatic infections a All Alive
Recipients of blood from donors who later developed vCJD 67 27 14 3 1
Blood donors to individuals who later developed vCJD 112 108 103 0 0
Other recipients of blood components from these donors 34 32 16 0 0
Plasma product recipients (non-bleeding disorders) who received UK sourced plasma products 1990- 2001 2 2 2 0 0
Certain surgical contacts of patients diagnosed with CJD 231 188 158 0 0
Highly transfused recipients 3 3 3 0 0
Total for ‘at increased risk’ groups where PHE holds data 449 360 296 3 1
Patients with bleeding disorders who received UK sourced plasma products 1990-2001b 4,025 3,554c 3,089c 0 1
Recipients of human derived growth hormoneb 1,883 1,883 1,500 78 0
Total for all ‘at increased risk’ groups 6,357 5,797 4,885 81 2
a. An asymptomatic infection is when an individual does not exhibit any of the signs and symptoms of CJD in life but abnormal prion protein indicative of CJD infection has been found in tissue obtained at post mortem.
b. These are minimum figures. Central reporting for bleeding disorder patients is incomplete, and a small number of patients have opted out of the central UKHCDO database. A small number of ‘at increased risk’ growth hormone recipients are not included in the Institute of Child Health study. Not all of the ‘at increased risk’ growth hormone recipients have been notified. There is no central record of who has been informed.
c. These are the minimum number of people notified based on those patients who were seen for care after the notification exercise. It is likely that many more of the ‘at increased risk’ patients received their notification letter but as they were not subsequently recorded as being seen for care this cannot be confirmed.
Infection report
Volume 10 Number 26 published on: 12 August 2016
CJD/Emerging infections
Summary results of the third national survey of abnormal prion prevalence in archived appendix specimens
In July 2012, the Transmissible Spongiform Encephalopathies (TSE) Risk Assessment SubGroup of the Advisory Committee on Dangerous Pathogens (the successor national advisory committee to the Spongiform Encephalopathy Advisory Committee (SEAC)), considered the results of the second unlinked anonymous national survey of the prevalence of abnormal prion protein in human appendix samples (Appendix-II [1]), and concluded that a further similar survey should be conducted on tissues from population groups considered unexposed to BSE [2]. This third national survey (Appendix-III) of appendix specimens removed at operations prior to the BSE epizootic and appendix specimens from those born in 1996 or later, by which time measures had been put in place to protect the food chain, has now been concluded. This report provides a summary of the results of the Appendix-III survey prior to publication in due course of the complete data.
The Appendix-III survey examined by immunohistochemistry (IHC) appendices removed at operation and collected from 44 hospitals throughout England. Abnormal prion accumulation was detected within the follicular dendritic cells of seven appendices out of 29,516 suitable samples examined. Indirect comparison of available data showed that none of the positive appendices could have come from the 178 known vCJD cases in the UK. Two of the seven positive samples were from the 14,692 appendices removed at operations conducted in 1962 through 1979: both these positive samples were from the 5,865 appendices removed in 1977 through 1979. The other five positive samples were found in the 14,824 appendices from subjects born in 1996 or later and removed at operation in 2000 through 2014: all five were in the sub-group of 10,074 born in 1996 through 2000. Therefore, none of the seven positive appendices were in specimens removed before 1977 or in patients born in 2001 or later.
The planned statistical analysis found no difference between the prevalence observed in the Appendix-II survey of 493 per million (95% Confidence Interval (CI): 282 to 801 per million) and the Appendix-III prevalence in appendices removed between 1962 through 1979 of 136 per million (95%CI: 16 to 492 per million; exact p=0.08), nor with the Appendix-III prevalence in
Health Protection Report Vol. 10 No. 26 – 12 August 2016
appendices from those born in 1996 through 2000 of 337 per million (95%CI: 110 to 787 per million; exact p=0.64). Test accuracy calculations using the Appendix-III data suggest the IHC technique specificity is in the range of 99.975% to over 99.99%. Although specificity of this magnitude (99.99%) implies few false positives, if the true prevalence is very low, then the positive predictive value of the IHC technique will diminish. At the one in 7,000 prevalence observed in the Appendix-III survey of specimens removed in 1979 or earlier, the positive predictive value (PPV) will be 56%, for a specificity of 99.99% and a sensitivity of 90%, compared to a PPV of 82% at the one in 2,000 prevalence observed in the Appendix-II survey.
The Appendix-II and -III surveys were conducted by a collaboration of PHE, the Department of Neurodegenerative Diseases at the UCL Institute of Neurology, the Animal and Plant Health Agency, the National Creutzfeldt-Jakob Disease Research and Surveillance Unit, the Histopathology Department of Derriford Hospital in Plymouth, and the MRC Prion Unit.
In summary, the Appendix-III survey data have not produced a clear answer to the question of whether abnormal prions detected by IHC in the British population is limited to those exposed to the BSE epizootic, and various interpretations are possible. The survey results have been considered by the ACDP TSE Sub-Group and a position paper detailing the conclusions of the committee has been published online, simultaneously with this summary report [3].
References
1. Gill ON, Spencer Y, Richard-Loendt, A, Kelly C, Dabaghian R, Boyes L, et al (2013). Prevalent abnormal prion protein in human appendixes after bovine spongiform encephalopathy epizootic: large scale survey. BMJ 347: f5675, http://www.bmj.com/content/347/bmj.f5675. 2. Advisory Committee on Dangerous Pathogens TSE Risk Assessment Subgroup (July 2012).
Position Statement on occurrence of vCJD and prevalence of infection in the UK population. Available from: ACDP TSE subgroup minutes, agendas and papers, https://app.box.com/s/hhhhg857fjpu2bnxhv6e.
3. Advisory Committee on Dangerous Pathogens TSE Risk Assessment Subgroup (August 2016). “Appendix-III” position statement. Available from: ACDP TSE subgroup minutes, agendas and papers, https://app.box.com/s/hhhhg857fjpu2bnxhv6e.
some history;
Public Release: 21-Dec-2016 UTHealth research could lead to blood test to detect Creutzfeldt-Jakob disease
University of Texas Health Science Center at Houston
HOUSTON - (Dec. 21, 2016) - The detection of prions in the blood of patients with variant Creutzfeldt-Jakob disease could lead to a noninvasive diagnosis prior to symptoms and a way to identify prion contamination of the donated blood supply, according to researchers at McGovern Medical School at The University of Texas Health Science Center at Houston (UTHealth).
The results of the research, led by senior author Claudio Soto, M.D., professor in the Department of Neurology and the director of the George and Cynthia Mitchell Center for Alzheimer's disease and Related Brain Disorders at UTHealth, were published today in Science Translational Medicine, a journal of the American Association for the Advancement of Science. First author of the paper is Luis Concha-Marambio, senior research assistant in the Department of Neurology at McGovern Medical School.
" Our findings, which need to be confirmed in further studies, suggest that our method of detection could be useful for the noninvasive diagnosis of this disease in pre-symptomatic individuals," Soto said. "Early diagnosis would allow any potential therapy to be given before substantial brain damage has occurred. In the case of the blood supply, availability of a procedure to efficiently detect small quantities of the infectious agent would allow removal of blood units contaminated with prions, so that new cases can be minimized substantially."
Human prion diseases are infectious and invariably fatal neurodegenerative diseases. They include sporadic Creutzfeldt-Jakob disease (sCJD), the most common form, and variant Creutzfeldt-Jakob disease (vCJD), which is caused by the transmission of bovine spongiform encephalopathy - commonly known as mad cow disease - from infected cattle to humans.
Since 1990, 178 people in the United Kingdom have died from vCJD, according to the National CJD Research & Surveillance Unit at the University of Edinburgh. The disease has claimed an additional 49 people worldwide, including four United States residents, according to the European Centre for Disease Prevention and Control. In a handful of cases, the disease was spread through the donated blood supply.
The disease can lay silent in the body for decades as damage slowly builds in the brain from the misfolded infectious proteins called prions. On average, people infected with vCJD die two years after the development of the first symptoms, which can include psychiatric alterations such as depression, anxiety and hallucinations that progress to more severe dementia, muscle contractions and loss of coordination.
Soto's team analyzed blood samples from 14 cases of vCJD and 153 controls, which included patients affected by sCJD and other neurodegenerative or neurological disorders as well as healthy subjects. To detect the prions, the team used a protein misfolding cyclic amplification assay, invented in Soto's lab, which mimics the prion replication process in vitro that occurs in prion disease. The results showed that prions could be detected with 100 percent sensitivity and specificity in blood samples from vCJD patients.
The new study builds on years of research by Soto's team, whose detection of prions in urine was published in the New England Journal of Medicine in August 2014. In June of this year, Soto received $11 million from the National Institute of Allergy and Infectious Diseases, part of the National Institutes of Health, to study the pathogenesis, transmission and detection of prion diseases including chronic wasting disease in deer.
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Soto is also on the faculty and Concha-Marambio is a student at The University of Texas Graduate School of Biomedical Sciences at Houston.
Co-authors are Sandra Pritzkow, Ph.D., from McGovern Medical School; Paul Schulz, M.D., professor of neurology from McGovern Medical School and Memorial Hermann Mischer Neuroscience Institute at the Texas Medical Center; Fabio Moda, Ph.D., and Fabrizio Tagliavini, M.D., from Istituto Neurologico Carlo Besta in Milan; and James W. Ironside, FMedSci, FRSE, professor of clinical neuropathology at the National CJD Research and Surveillance Unit at the University of Edinburgh.
The research was supported by grants from the National Institutes of Health (P01AI106705, R42NS079060, R01NS049173).
Disclaimer: AAAS and EurekAlert! are not responsible for the accuracy of news releases posted to EurekAlert! by contributing institutions or for the use of any information through the EurekAlert system.
Detection of prions in blood from patients with variant Creutzfeldt-Jakob disease
Luis Concha-Marambio1,2, Sandra Pritzkow1, Fabio Moda1,3, Fabrizio Tagliavini3, James W. Ironside4, Paul E. Schulz1 and Claudio Soto1,2,*
+ Author Affiliations
↵*Corresponding author. Email: claudio.soto@uth.tmc.edu
Science Translational Medicine 21 Dec 2016: Vol. 8, Issue 370, pp. 370ra183 DOI: 10.1126/scitranslmed.aaf6188
A new blood test for prion diseases Prions are the proteinaceous infectious agents responsible for various animal and human diseases. The transmission of bovine spongiform encephalopathy into humans has led to a new illness, termed variant Creutzfeldt-Jakob disease (vCJD). Currently, the number of people infected by this new disease is unknown, which is a major concern because it has been shown that preclinical carriers of vCJD prions can transmit the disease by blood transfusion. Now, Concha-Marambio et al. report the development of a biochemical test to detect vCJD prions in blood with 100% sensitivity and specificity. Availability of a highly efficient blood test for vCJD is important to minimize further transmission of the disease, to increase blood safety, and to allow early diagnosis of this disease.
Abstract Human prion diseases are infectious and invariably fatal neurodegenerative diseases. They include sporadic Creutzfeldt-Jakob disease (sCJD), the most common form, and variant CJD (vCJD), which is caused by interspecies transmission of prions from cattle infected by bovine spongiform encephalopathy. Development of a biochemical assay for the sensitive, specific, early, and noninvasive detection of prions (PrPSc) in the blood of patients affected by prion disease is a top medical priority to increase the safety of the blood supply. vCJD has already been transmitted from human to human by blood transfusion, and the number of asymptomatic carriers of vCJD in the U.K. alone is estimated to be 1 in 2000 people. We used the protein misfolding cyclic amplification (PMCA) technique to analyze blood samples from 14 cases of vCJD and 153 controls, including patients affected by sCJD and other neurodegenerative or neurological disorders as well as healthy subjects. Our results showed that PrPSc could be detected with 100% sensitivity and specificity in blood samples from vCJD patients. Detection was possible in any of the blood fractions analyzed and could be done with as little as a few microliters of sample volume. The PrPSc concentration in blood was estimated to be ~0.5 pg/ml. Our findings suggest that PMCA may be useful for premortem noninvasive diagnosis of vCJD and to identify prion contamination of the blood supply. Further studies are needed to fully validate the technology.
Copyright © 2016, American Association for the Advancement of Science
Subject: SEAC epidemiology subgroup position statement on the vCJD epidemic (sporadic CJDs ???) Date: January 26, 2006 at 9:01 am PST
SEAC Position Statement
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SEAC response to the SEAC epidemiology subgroup position statement on the vCJD epidemic SEAC welcomed and endorsed the SEAC Epidemiology Subgroup’s position statement on the vCJD epidemic1.
A substantial number of subclinical carriers of vCJD infection may exist in the UK population who cannot, at present, be identified. As a result a secondary, possibly self-sustaining, vCJD epidemic could arise due to human-to-human transmission via medical interventions such as blood transfusion and surgery. In order to implement the most appropriate precautionary measures, there is an urgent need to ascertain better the prevalence, age and genotype distribution of subclinical vCJD infection in the UK population. These data will inform assessment and subsequent management of the risks of secondary transmission.
New information on the prevalence, age and genotype distribution of vCJD infection will be provided by the planned testing of samples under collection for the National Anonymous Tonsil Archive (NATA). This testing should be progressed with urgency. However a large proportion of tonsils will be collected from young individuals with relatively low dietary exposures to BSE, and there is uncertainty about the sensitivity of tonsil tests to detect subclinical vCJD infection. It is, therefore, very important that additional programmes to test a range of tissues collected from other age groups in the population are considered.
Testing of tissues collected from autopsies could, potentially, provide substantial data on the prevalence, age and genotype distribution of infection which would complement data from NATA. However, there are practical, ethical and legal issues around post mortem testing. The committee recommends that an expert group is convened with some urgency by the Department of Health to consider and advise how such post mortem testing, or other testing designed to ascertain the prevalence, age and genotype distribution of vCJD in the UK population, might best be undertaken.
SEAC January 2006
1SEAC Epidemiology Subgroup. Position statement on the vCJD epidemic. November 2005.
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Page updated: 26 January 2006
SEAC epidemiology subgroup position statement on the vCJD epidemic Issue
1. SEAC asked the SEAC Epidemiology Subgroup to reassess the nature and future profile of the vCJD epidemic, taking into account new research and the possibility of human to human infection. SEAC identified a number of issues for the Subgroup to consider (see Annex 1).
Background
2. Incidence of vCJD has declined over the past 4 years in the UK1,a. Projections based on the case data suggest the number of additional cases of vCJD arising from the consumption of BSE-infected material might be relatively small (less than 100)2. However, findings from a retrospective survey of appendix and tonsil tissue from operations carried out between 1995 and 2000 on individuals predominantly (83%) in the age range 10-30 years suggest that the number of infected individuals may be greater than projections based on backcalculation from vCJD cases suggest3. Furthermore, research in animal models suggests it is possible that a proportion of infections may not develop into clinical disease, or do so over a longer time scale, and remain at a subclinical level4-7. Although all vCJD cases tested to date (about 85% of cases) have been of the M/M genotype at the polymorphic codon 129 of the human prion protein gene, the effect of other genotypes on the susceptibility to, infectiousness of, and phenotype of, vCJD is uncertain. The finding of an asymptomatic case of probable blood transfusion associated transmission of vCJD of the M/V genotype8 suggests individuals of this genotype are also susceptible to secondary transmission by the intravenous route. It is possible that secondary infection via medical procedures may give rise to additional infections and potentially enable a self-sustaining secondary epidemic.
3. To address the SEAC request, the Subgroup met twice in 2005 to consider data on the epidemiology of the disease, predictions about the epidemic based on infectious disease models and relevant published and unpublished research. This initial position statement has been produced on the basis of these discussions. It is intended that the Subgroup will keep the statement under review in light of emerging scientific or medical information that improves understanding of the epidemiology of vCJD.
General observations
4. It is currently not possible to answer in full the questions set by SEAC due to continued uncertainty about key characteristics of the vCJD epidemic, in particular: the prevalence and distribution of primary and secondary infection in the UK population and incubation periods of the primary and secondary disease. Although infectious disease models provide a very useful means to test hypotheses, firm conclusions about the epidemic can only be drawn by the acquisition of better estimates of the prevalence, age and genotype distribution of infection based on population studies. Continued surveillance to identify new cases of vCJD will also provide valuable information to understand better the epidemic, in particular: trends in the incidence of vCJD, the age and genotype distribution of those who develop vCJD and identification of possible routes of secondary transmission (e.g. maternal or related to invasive medical procedures).
Influence of age on infection
5. Direct evidence to inform assessment of the influence of age on the prevalence of infection is presently limited to data from vCJD cases and the retrospective survey of appendix and tonsil tissue3. The age distribution of vCJD cases has remained relatively stable since the start of the epidemic. This is likely to be due to a combination of age-related differentials in dietary exposure to BSE and in susceptibility to the disease. Modelling studies based on the assumption that the vCJD epidemic is best defined by age-related differentials in susceptibility to vCJD together with a constant incubation period and a time window of dietary BSE exposure related directly to the observed BSE epidemic, which appears to be the most likely scenario, have produced broadly similar results9-11. These studies suggest that age-related susceptibility / exposure was greatest in the 10-20 year old age range, lower in early childhood and much lower later in adult life. However, although this profile might be expected to be reflected in the prevalence of primary infections leading to disease, it is derived from data on vCJD cases. Thus, it is possible that it may not apply to infections in the non-M/M population or to infections that remain at a subclinical level (see later).
6. Given these uncertainties, it is not possible to predict with confidence the relative risk that age groups are infected for all age groups of the population. However, it is almost surely the case that, due to the BSE control measures introduced in the UK, dietary exposure of the post-1996 birth cohorts would be very much lower or even negligible compared with older birth cohorts. Children 9 years old and younger would, therefore, very clearly be at the lowest risk of primary infection. Furthermore, despite relatively large dietary exposure to BSE9, only a single case has been observed in the pre-1940 birth cohort. This strongly suggests that adults over 66 years of age are at relatively low risk of developing primary vCJD, assuming there is no major under ascertainment of the disease in the elderly.
7. If it is assumed that susceptibility to primary vCJD infection (in terms of infections that lead to disease and infections that remain at a subclinical level) was greatest within the 10-20 year age range, it would be predicted that individuals in the 1965-1985 birth cohorts, given the peak in dietary BSE exposure around 1990, are at the greatest risk of being infected. However, as discussed above this hypothetical profile of age-related susceptibility to primary vCJD infection may not apply to the non-M/M population or to infections that remain at a subclinical level. Given the decline in the BSE epidemic, the 1990-1995 birth cohorts are at a much smaller risk, and this risk lowers progressively with year of birth. A continuing lack of vCJD cases in the post-1990 birth cohort will provide reassurance about the validity of this hypothesis.
Influence of genotype on infection
8. There is an indication that non-M/M genotypes are susceptible to vCJD infection from the case of probable blood transfusion associated transmission of vCJD of the M/V genotype8. In addition, atypical immunohistochemical results from two of the three positive appendix samples in the appendix and tonsil survey may also be an indicator of infection in non-M/M genotypes3.
9. Polymorphisms at codon 129 of the human prion protein gene influence susceptibility to, and the incubation of period of, human prion diseases12-14. Observations of kuru suggest that individuals of non-M/M genotypes are generally less susceptible to this disease and have longer incubation periods than individuals of the M/M genotype14. On the basis that these general characteristics are a valid model for vCJD infection, it seems reasonable to assume that primary and secondary vCJD cases in individuals of the M/V and V/V genotypes might arise, although they can be expected to be proportionately fewer in number and possibly appear over a long time scale. Recent projections from an infectious disease model suggest that, in the unlikely situation that other genotypes are equally susceptible to clinical disease, the number of future primary cases may increase up to five-fold compared with current estimates for future cases of the M/M genotype2.
10. It is not possible to predict the clinical phenotype of vCJD cases in non-M/M genotypes, should they arise. However, evidence from fatal familial insomnia and sporadic CJD indicates that codon 129 genotype affects clinical phenotype15,16. Experiments in transgenic mice expressing human forms of the prion protein gene suggest that the neuropathological phenotype of vCJD is influenced by genotype7.
Subclinical carriers of infection
11. Experimental studies in mice suggest primary prion infections may remain at a subclinical level but on secondary transmission result in clinical disease4,5. Thus, asymptomatic animals can be subclinical carriers of infection. The reason that infection in some animals remains at a subclinical level while clinical disease develops in others is not fully understood. The potential existence of subclinical carriers of vCJD may explain the apparent discrepancy between prevalence estimates of primary vCJD infection based on the appendix and tonsil survey and the vCJD case data. Recent projections to explore this possibility suggest the number of subclinical carriers could be of the order of several thousand2. There are currently no data to allow the possible age and genotype distribution of subclinical carriers of infection to be determined.
Additional data to understand the epidemic better
12. As discussed above, knowledge of the prevalence of infection cannot be determined accurately from quantitative models because of the uncertainties regarding the effect of genotype and age at infection and the possibility of secondary transmission from subclinical carriers of infection. Further data are required to understand better the prevalence, age and genotype distribution of both primary and secondary vCJD infection.
13. The PrPSc screening programme, due to begin in 2006, of the very large number of samples under collection for the National Anonymous Tonsil Archive (NATA) will allow more accurate assessments of the prevalence and age and genotype distribution of infection (see table). It is strongly recommended that testing of samples collected by NATA is progressed with all possible urgency.
14. However, although PrPSc tests have always proved positive in tonsils of clinical vCJD cases, there are uncertainties about the sensitivity of tonsil tests to detect asymptomatic and subclinical infection. In addition, although tonsils will be collected from a wide age range of individuals, tonsillectomy is more commonly conducted at relatively young ages. Thus, many of the samples will be collected from individuals with relatively low dietary exposures to BSE. Additional programmes to test other tissues collected from a different age distribution of individuals would provide further data as well as assurance about the findings from NATA (see table).
15. Principally as an infection control measure, a pilot study is underway to investigate the feasibility of testing the spleen, tonsil and / or appendix from cadaveric tissue / organ donors. Pilot studies to screen corneal and multi-organ donors are also under consideration. Although, testing of tissue from cadaveric, corneal and multi-organ donors might provide a limited amount of data, pilot studies to assess the feasibility of such testing are welcomed and encouraged.
16. PrPSc testing of a range of tissues collected from autopsies would, in principle, provide substantial data on the prevalence and age and genotype distribution of the section of the population presumed most likely to carry the majority of primary vCJD infections. However, such a programme relies heavily on consent for testing, which is a legal requirement, from the deceased (in life) or a close relative / carer. Practical and cost considerations around informed consent could have a substantial detrimental impact on the feasibility of such a programme. Nevertheless, it is recommended that serious consideration be given to testing samples collected from autopsies. By comparison, retrospective survey of residual appendectomy or splenectomy tissues, although informative, would be less useful (see table).
17. It is also recommended that enhanced clinical surveillance in the elderly be considered (see table). Although vCJD cases arising from primary transmission of BSE are observed mostly in young adults, there may be potential under-ascertainment of cases in the elderly, possibly due to misdiagnosis. Enhanced surveillance of this section of the population would allow this possibility to be tested. Furthermore, such enhanced surveillance would provide additional assurance that clinical cases of secondary transmission of vCJD may be detected since this section of the population is the group most likely to have undergone invasive medical procedures and / or to have received blood transfusions. Should cases be detected in older age groups, it would be important to undertake statistical analyses to estimate the proportion of cases that might have arisen from the dietary route versus medical interventions, i.e. the proportion of disease that could have arisen from primary or secondary transmission.
18. Clinical monitoring and, with patient consent, post mortem vCJD tests on individuals considered to be ‘at risk of vCJD for public health purposes’ would help to inform assessment of secondary transmission risks. Proposals are currently being developed for blood component recipients and should be considered for all at risk groups.
Self-sustaining epidemic
19. Current risk assessments of secondary transmission through surgery, blood transfusion, dentistry and bone / tissue / organ transplantation17 suggest that, on the basis of what is presently known, transmission via the surgical and blood routes are the most important in terms of the possible contribution to a secondary epidemic. This is on the basis of the relatively high number of surgical procedures and blood transfusions and estimated transmission efficiencies via these routes. By comparison, the risk of transmission via dentistry per procedure is thought to be relatively low, although the number of procedures is relatively large. In contrast, the risk of transmission via transplantation, depending on what tissues / organs are transplanted, is thought to be relatively high but the number of procedures is relatively low. However, uncertainties in key parameters in the risk assessments remain, such as the profile of the primary epidemic, infectivity levels in tissues, transmission efficiencies via routes and the effectiveness of decontamination / infectivity reduction methods.
20. The information currently available from follow up of children born to vCJD cases cannot exclude the possibility of maternal transmission of vCJD. However, on the basis of the information available on prion diseases of humans and animals, maternal transmission of vCJD, if it occurs, is unlikely on its own to support a self-sustaining secondary epidemic.
21. On the basis of current understanding, the transmission risk from the surgical route on its own could create a self-sustaining epidemic under worst case conditions of long incubation period for iatrogenic vCJD and long patient survival times. The National Institute for Health and Clinical Excellence is currently conducting an assessment of precautionary measures to reduce potential transmission risks via surgery. Blood borne transmission on its own is thought unlikely to result in a self-sustaining epidemic, especially given the precautionary measures already enacted (e.g. (deferral of donations from blood recipients, import of plasma for fractionation and leucodepletion). The large number of dental procedures (coupled with good patient survival) implies that any significant risk via that route could have a major impact on the dynamics of secondary infection. In addition, interactions between routes of transmission will also make a self-sustaining epidemic more likely.
22. The likelihood of a self-sustaining epidemic cannot be quantified at present. The complexity and number of interactions between potential routes of transmission make development of a workable model to quantify interactions within and between routes and the effect interactions might have on transmission risks very difficult. Work is underway to develop population level models for the surgical and blood routes with a view to developing a combined model to explore the effect of interactions between these routes. This work should continue to be supported.
23. On the basis of current risk assessments of transmission routes considered in isolation, factors such as the number of potential transmissions, infectivity of tissues, the efficiencies of transmission and the effectiveness of decontamination / infectivity reduction methods are all, to varying extents, key influences on the likelihood of a self-sustaining epidemic arising and the rate that it might develop.
24. On the basis of current understanding, a secondary epidemic is more likely if many individuals are exposed to potentially infectious material from a single individual (e.g. through dispersion of surgical instruments between sets) or vice versa (e.g. if blood products are pooled, exposing each recipient to multiple donors). A secondary epidemic is also generally more likely if patients who have undergone one potentially infectious procedure are at increased risk of undergoing further procedures.
SEAC Epidemiology Subgroup November 2005
a Number of deaths from vCJD in the UK in the years 1999 to 2005: 15 (1999), 28 (2000), 20 (2001), 17 (2002), 18 (2003), 9 (2004) and 3 (end September 2005).
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Table of potential investigative programmes to improve understanding of the vCJD epidemic (27 KB)
References 1. http://www.cjd.ed.ac.uk/vcjdqsep05.htm
2. Clarke & Ghani (2005) Projections of future course of the primary vCJD epidemic in the UK: inclusion of subclinical infection and the possibility of wider genetic susceptibility R. J. Soc. Interface. 2, 19-31.
3. Hilton et al. (2004) Prevalence of lymphoreticular prion protein accumulation in UK tissue samples. J Pathol. 203, 733-739.
4. Hill et al. (2000) Species-barrier-independent prion replication in apparently resistant species. Proc. Natl. Acad. Sci. USA. 97, 10248-10253.
5. Race et al. (2001) Long-term subclinical carrier state precedes scrapie replication and adaptation in a resistant species: analogies to bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease. J Virol. 75, 10106-10112.
6. Asante et al. (2002) BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein. EMBO J. 21, 6358-6366.
7. Wadsworth et al. (2004) Human prion protein V129 prevents expression of variant CJD phenotype. Science. 306, 1793-1796.
8. Peden et al. (2004) Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet. 364, 477-479.
9. Cooper & Bird (2003) Predicting incidence of variant Creutzfeldt-Jakob disease from UK dietary exposure to bovine spongiform encephalopathy for the 1940 to 1969 and post-1969 birth cohorts. Int. J. Epidemiol. 32, 784-791.
10. Ghani et al. (2003) Updated projections of future vCJD deaths in the UK. BMC Infect. Dis. 3, 4-11.
11. Boelle et al. (2004) Epidemiological evidence of higher susceptibility to vCJD in the young. BMC Infect Dis. 4, 26-32.
12. Alperovitch et al. (1999) Codon 129 prion protein genotype and sporadic Creutzfeldt-Jakob disease. Lancet 353, 1673-1674.
13. Huillard d’Aignaux et al. (1999) Incubation period of Creutzfeldt-Jakob disease in human growth hormone recipients in France. Neurology 53, 1197-1201.
14. Lee et al. (2001) Increased susceptibility to kuru of carriers of the PRNP 129 methionine/methionine genotype. J. Infect. Disease. 183, 192-196.
15. Gambetti et al. (1995) Fatal familial insomnia and familial Creutzfeldt-Jakob disease: clinical, pathological and molecular features. Brain Pathol. 5, 43-51.
16. Parchi et al. (1999) Classification of sporadic Creutzfeld-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann. Neurol. 46, 224-233.
17. Economics and Operational Research Division risk assessments, Department of Health.
Annex 1 Issues SEAC asked the SEAC Epidemiology Subgroup to consider:
1. What are the implications of recent research for current models of the vCJD epidemic?
(a) Do recent data on age- and genotype-related effects alter the predicted profile of the vCJD epidemic and the potential number of infective carriers?
(b) Are there likely to be 'carriers' of infectivity who do not develop clinical vCJD within their lifetime, or who present with clinical features not currently recognised as vCJD, and if so, what are the limits on the possible prevalence / age distribution / genotype of such individuals?
2. What new evidence would lead SEAC to believe that the size of the vCJD epidemic is likely to be larger or smaller than current estimates (i.e. what new data would lead SEAC to believe that current estimates may be incorrect)?
(a) Are the current and expected data from population level studies (i.e. tonsil and appendix and other tissue surveys) sufficient to enable estimation of the age / genotype distribution of infection, and what further information would help inform predictions of the profile of the vCJD epidemic?
(b) What are the information barriers to determining the potential risks to public health from carriers of vCJD infectivity?
3. Is there a significant risk of a self-sustaining human vCJD epidemic through secondary transmission of BSE between humans?
(a) What are the relative risks of secondary transmission through medical procedures (transfusion, transplantation, surgery)?
(b) Taking all these potential routes of transmission and their interactions into account, how likely is a self-sustaining epidemic?
(c) If a self-sustaining epidemic is possible, what factors determine its scale?
4. What are the key points at which modification of practice could significantly reduce the risk of a self-sustaining epidemic?
Page updated: 26 January 2006
ZOONOSIS BSE, SCRAPIE, CWD, 2015, 2016, 2017 PRION CONFERENCE ET AL
O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations
Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni, Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys Atomic Energy Commission; Fontenay-aux-Roses, France
Prion diseases (PD) are the unique neurodegenerative proteinopathies reputed to be transmissible under field conditions since decades. The transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that an animal PD might be zoonotic under appropriate conditions. Contrarily, in the absence of obvious (epidemiological or experimental) elements supporting a transmission or genetic predispositions, PD, like the other proteinopathies, are reputed to occur spontaneously (atpical animal prion strains, sporadic CJD summing 80% of human prion cases). Non-human primate models provided the first evidences supporting the transmissibiity of human prion strains and the zoonotic potential of BSE. Among them, cynomolgus macaques brought major information for BSE risk assessment for human health (Chen, 2014), according to their phylogenetic proximity to humans and extended lifetime. We used this model to assess the zoonotic potential of other animal PD from bovine, ovine and cervid origins even after very long silent incubation periods.
*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period,
***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014),
***is the third potentially zoonotic PD (with BSE and L-type BSE),
***thus questioning the origin of human sporadic cases.
We will present an updated panorama of our different transmission studies and discuss the implications of such extended incubation periods on risk assessment of animal PD for human health.
===============
***thus questioning the origin of human sporadic cases***
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***our findings suggest that possible transmission risk of H-type BSE to sheep and human. Bioassay will be required to determine whether the PMCA products are infectious to these animals.
==============
***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice.
***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.
***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.
PRION 2016 TOKYO
Saturday, April 23, 2016
SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016
Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online
Taylor & Francis
Prion 2016 Animal Prion Disease Workshop Abstracts
WS-01: Prion diseases in animals and zoonotic potential
Juan Maria Torres a, Olivier Andreoletti b, J uan-Carlos Espinosa a. Vincent Beringue c. Patricia Aguilar a,
Natalia Fernandez-Borges a. and Alba Marin-Moreno a
"Centro de Investigacion en Sanidad Animal ( CISA-INIA ). Valdeolmos, Madrid. Spain; b UMR INRA -ENVT 1225 Interactions Holes Agents Pathogenes. ENVT. Toulouse. France: "UR892. Virologie lmmunologie MolécuIaires, Jouy-en-Josas. France
Dietary exposure to bovine spongiform encephalopathy (BSE) contaminated bovine tissues is considered as the origin of variant Creutzfeldt Jakob (vCJD) disease in human. To date, BSE agent is the only recognized zoonotic prion. Despite the variety of Transmissible Spongiform Encephalopathy (TSE) agents that have been circulating for centuries in farmed ruminants there is no apparent epidemiological link between exposure to ruminant products and the occurrence of other form of TSE in human like sporadic Creutzfeldt Jakob Disease (sCJD). However, the zoonotic potential of the diversity of circulating TSE agents has never been systematically assessed. The major issue in experimental assessment of TSEs zoonotic potential lies in the modeling of the ‘species barrier‘, the biological phenomenon that limits TSE agents’ propagation from a species to another. In the last decade, mice genetically engineered to express normal forms of the human prion protein has proved essential in studying human prions pathogenesis and modeling the capacity of TSEs to cross the human species barrier.
To assess the zoonotic potential of prions circulating in farmed ruminants, we study their transmission ability in transgenic mice expressing human PrPC (HuPrP-Tg). Two lines of mice expressing different forms of the human PrPC (129Met or 129Val) are used to determine the role of the Met129Val dimorphism in susceptibility/resistance to the different agents.
These transmission experiments confirm the ability of BSE prions to propagate in 129M- HuPrP-Tg mice and demonstrate that Met129 homozygotes may be susceptible to BSE in sheep or goat to a greater degree than the BSE agent in cattle and that these agents can convey molecular properties and neuropathological indistinguishable from vCJD. However homozygous 129V mice are resistant to all tested BSE derived prions independently of the originating species suggesting a higher transmission barrier for 129V-PrP variant.
Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice.
Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.
These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.
why do we not want to do TSE transmission studies on chimpanzees $
5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.
snip...
R. BRADLEY
Title: Transmission of scrapie prions to primate after an extended silent incubation period)
*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS.
*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated.
*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains.
SPONTANEOUS ATYPICAL BOVINE SPONGIFORM ENCEPHALOPATHY
***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***
CWD TSE Prion Zoonosis to squirrel monkey and macaque
Prion 2017 Conference Abstracts CWD
2017 PRION CONFERENCE
First evidence of intracranial and peroral transmission of Chronic Wasting Disease (CWD) into Cynomolgus macaques: a work in progress
Stefanie Czub1, Walter Schulz-Schaeffer2, Christiane Stahl-Hennig3, Michael Beekes4, Hermann Schaetzl5 and Dirk Motzkus6 1
University of Calgary Faculty of Veterinary Medicine/Canadian Food Inspection Agency; 2Universitatsklinikum des Saarlandes und Medizinische Fakultat der Universitat des Saarlandes; 3 Deutsches Primaten Zentrum/Goettingen; 4 Robert-Koch-Institut Berlin; 5 University of Calgary Faculty of Veterinary Medicine; 6 presently: Boehringer Ingelheim Veterinary Research Center; previously: Deutsches Primaten Zentrum/Goettingen
This is a progress report of a project which started in 2009. 21 cynomolgus macaques were challenged with characterized CWD material from white-tailed deer (WTD) or elk by intracerebral (ic), oral, and skin exposure routes. Additional blood transfusion experiments are supposed to assess the CWD contamination risk of human blood product. Challenge materials originated from symptomatic cervids for ic, skin scarification and partially per oral routes (WTD brain). Challenge material for feeding of muscle derived from preclinical WTD and from preclinical macaques for blood transfusion experiments. We have confirmed that the CWD challenge material contained at least two different CWD agents (brain material) as well as CWD prions in muscle-associated nerves.
Here we present first data on a group of animals either challenged ic with steel wires or per orally and sacrificed with incubation times ranging from 4.5 to 6.9 years at postmortem. Three animals displayed signs of mild clinical disease, including anxiety, apathy, ataxia and/or tremor. In four animals wasting was observed, two of those had confirmed diabetes. All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals. Protein misfolding cyclic amplification (PMCA), real-time quaking-induced conversion (RT-QuiC) and PET-blot assays to further substantiate these findings are on the way, as well as bioassays in bank voles and transgenic mice.
At present, a total of 10 animals are sacrificed and read-outs are ongoing. Preclinical incubation of the remaining macaques covers a range from 6.4 to 7.10 years. Based on the species barrier and an incubation time of > 5 years for BSE in macaques and about 10 years for scrapie in macaques, we expected an onset of clinical disease beyond 6 years post inoculation.
PRION 2017 DECIPHERING NEURODEGENERATIVE DISORDERS
Subject: PRION 2017 CONFERENCE DECIPHERING NEURODEGENERATIVE DISORDERS VIDEO
PRION 2017 CONFERENCE DECIPHERING NEURODEGENERATIVE DISORDERS
*** PRION 2017 CONFERENCE VIDEO
TUESDAY, JUNE 13, 2017
PRION 2017 CONFERENCE ABSTRACT
First evidence of intracranial and peroral transmission of Chronic Wasting Disease (CWD) into Cynomolgus macaques: a work in progress
SATURDAY, JULY 29, 2017
Risk Advisory Opinion: Potential Human Health Risks from Chronic Wasting Disease CFIA, PHAC, HC (HPFB and FNIHB), INAC, Parks Canada, ECCC and AAFC
ZOONOTIC, ZOONOSIS, CHRONIC WASTING DISEASE CWD TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY TSE PRION
10. ZOONOTIC, ZOONOSIS, CHRONIC WASTING DISEASE CWD TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY TSE PRION AKA MAD DEER ELK DISEASE IN HUMANS, has it already happened, that should be the question...
''In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids.'' Scientific opinion on chronic wasting disease (II)
EFSA Panel on Biological Hazards (BIOHAZ) Antonia Ricci Ana Allende Declan Bolton Marianne Chemaly Robert Davies Pablo Salvador Fernández Escámez ... See all authors
First published: 17 January 2018 https://doi.org/10.2903/j.efsa.2018.5132 ;
also, see;
8. Even though human TSE‐exposure risk through consumption of game from European cervids can be assumed to be minor, if at all existing, no final conclusion can be drawn due to the overall lack of scientific data. In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids. It might be prudent considering appropriate measures to reduce such a risk, e.g. excluding tissues such as CNS and lymphoid tissues from the human food chain, which would greatly reduce any potential risk for consumers. However, it is stressed that currently, no data regarding a risk of TSE infections from cervid products are available.
snip...
The tissue distribution of infectivity in CWD‐infected cervids is now known to extend beyond CNS and lymphoid tissues. While the removal of these specific tissues from the food chain would reduce human dietary exposure to infectivity, exclusion from the food chain of the whole carcass of any infected animal would be required to eliminate human dietary exposure.
zoonosis zoonotic cervid tse prion cwd to humans, preparing for the storm
***An alternative to modeling the species barrier is the cell-free conversion assay which points to CWD as the animal prion disease with the greatest zoonotic potential, after (and very much less than) BSE.116***
Volume 2: Science
4. The link between BSE and vCJD
4.29 The evidence discussed above that vCJD is caused by BSE seems overwhelming. Uncertainties exist about the cause of CJD in farmers, their wives and in several abattoir workers. It seems that farmers at least might be at higher risk than others in the general population. 1 Increased ascertainment (ie, increased identification of cases as a result of greater awareness of the condition) seems unlikely, as other groups exposed to risk, such as butchers and veterinarians, do not appear to have been affected. The CJD in farmers seems to be similar to other sporadic CJD in age of onset, in respect to glycosylation patterns, and in strain-typing in experimental mice. Some farmers are heterozygous for the methionine/valine variant at codon 129, and their lymphoreticular system (LRS) does not contain the high levels of PrPSc found in vCJD. It remains a remote possibility that when older people contract CJD from BSE the resulting phenotype is like sporadic CJD and is distinct from the vCJD phenotype in younger people...BSE INQUIRY
Summary and Recommendation:
snip...
Health Portfolio partners were recently made aware of initial findings from a research project led by a CFIA scientist that have demonstrated that cynomolgus macaques can be infected via intracranial exposure and oral gavage with CWD infected muscle.
These findings suggest that CWD, under specific experimental conditions, has the potential to cross the human species barrier, including by enteral feeding of CWD infected muscle.
*** WDA 2016 NEW YORK ***
We found that CWD adapts to a new host more readily than BSE and that human PrP was unexpectedly prone to misfolding by CWD prions.
In addition, we investigated the role of specific regions of the bovine, deer and human PrP protein in resistance to conversion by prions from another species.
***We have concluded that the human protein has a region that confers unusual susceptibility to conversion by CWD prions.
Student Presentations Session 2
The species barriers and public health threat of CWD and BSE prions
Ms. Kristen Davenport1, Dr. Davin Henderson1, Dr. Candace Mathiason1, Dr. Edward Hoover1 1Colorado State University
Chronic wasting disease (CWD) is spreading rapidly through cervid populations in the USA. Bovine spongiform encephalopathy (BSE, mad cow disease) arose in the 1980s because cattle were fed recycled animal protein.
These and other prion diseases are caused by abnormal folding of the normal prion protein (PrP) into a disease causing form (PrPd), which is pathogenic to nervous system cells and can cause subsequent PrP to misfold. CWD spreads among cervids very efficiently, but it has not yet infected humans. On the other hand, BSE was spread only when cattle consumed infected bovine or ovine tissue, but did infect humans and other species.
The objective of this research is to understand the role of PrP structure in cross-species infection by CWD and BSE. To study the propensity of each species’ PrP to be induced to misfold by the presence of PrPd from verious species, we have used an in vitro system that permits detection of PrPd in real-time.
We measured the conversion efficiency of various combinations of PrPd seeds and PrP substrate combinations.
We observed the cross-species behavior of CWD and BSE, in addition to feline-adapted CWD and BSE. We found that CWD adapts to a new host more readily than BSE and that human PrP was unexpectedly prone to misfolding by CWD prions. In addition, we investigated the role of specific regions of the bovine, deer and human PrP protein in resistance to conversion by prions from another species.
***We have concluded that the human protein has a region that confers unusual susceptibility to conversion by CWD prions. CWD is unique among prion diseases in its rapid spread in natural populations. BSE prions are essentially unaltered upon passage to a new species, while CWD adapts to the new species. This adaptation has consequences for surveillance of humans exposed to CWD. Wildlife Disease Risk Communication Research Contributes to Wildlife Trust Administration Exploring perceptions about chronic wasting disease risks among wildlife and agriculture professionals and stakeholders
CDC CWD 2018 TRANSMISSION
*** The potential impact of prion diseases on human health was greatly magnified by the recognition that interspecies transfer of BSE to humans by beef ingestion resulted in vCJD. While changes in animal feed constituents and slaughter practices appear to have curtailed vCJD, there is concern that CWD of free-ranging deer and elk in the U.S. might also cross the species barrier. Thus, consuming venison could be a source of human prion disease. Whether BSE and CWD represent interspecies scrapie transfer or are newly arisen prion diseases is unknown. Therefore, the possibility of transmission of prion disease through other food animals cannot be ruled out. There is evidence that vCJD can be transmitted through blood transfusion. There is likely a pool of unknown size of asymptomatic individuals infected with vCJD, and there may be asymptomatic individuals infected with the CWD equivalent. These circumstances represent a potential threat to blood, blood products, and plasma supplies.
Transmission Studies
Mule deer transmissions of CWD were by intracerebral inoculation and compared with natural cases {the following was written but with a single line marked through it ''first passage (by this route)}...TSS
resulted in a more rapidly progressive clinical disease with repeated episodes of synocopy ending in coma. One control animal became affected, it is believed through contamination of inoculum (?saline). Further CWD transmissions were carried out by Dick Marsh into ferret, mink and squirrel monkey. Transmission occurred in ALL of these species with the shortest incubation period in the ferret.
snip...
Prion Infectivity in Fat of Deer with Chronic Wasting Disease▿
Brent Race#, Kimberly Meade-White#, Richard Race and Bruce Chesebro* + Author Affiliations
In mice, prion infectivity was recently detected in fat. Since ruminant fat is consumed by humans and fed to animals, we determined infectivity titers in fat from two CWD-infected deer. Deer fat devoid of muscle contained low levels of CWD infectivity and might be a risk factor for prion infection of other species.
Prions in Skeletal Muscles of Deer with Chronic Wasting Disease
Here bioassays in transgenic mice expressing cervid prion protein revealed the presence of infectious prions in skeletal muscles of CWD-infected deer, demonstrating that humans consuming or handling meat from CWD-infected deer are at risk to prion exposure.
*** now, let’s see what the authors said about this casual link, personal communications years ago, and then the latest on the zoonotic potential from CWD to humans from the TOKYO PRION 2016 CONFERENCE.
see where it is stated NO STRONG evidence. so, does this mean there IS casual evidence ???? “Our conclusion stating that we found no strong evidence of CWD transmission to humans”
From: TSS (216-119-163-189.ipset45.wt.net)
Subject: CWD aka MAD DEER/ELK TO HUMANS ???
Date: September 30, 2002 at 7:06 am PST
From: "Belay, Ermias"
To: Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias"
Sent: Monday, September 30, 2002 9:22 AM
Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Dear Sir/Madam,
In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD. That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: 404-639-3091). Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated.
Ermias Belay, M.D. Centers for Disease Control and Prevention
-----Original Message-----
From: Sent: Sunday, September 29, 2002 10:15 AM
Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Sunday, November 10, 2002 6:26 PM ......snip........end..............TSS
Thursday, April 03, 2008
A prion disease of cervids: Chronic wasting disease 2008 1: Vet Res. 2008 Apr 3;39(4):41 A prion disease of cervids: Chronic wasting disease Sigurdson CJ.
snip...
*** twenty-seven CJD patients who regularly consumed venison were reported to the Surveillance Center***,
snip... full text ;
> However, to date, no CWD infections have been reported in people.
key word here is 'reported'. science has shown that CWD in humans will look like sporadic CJD. SO, how can one assume that CWD has not already transmitted to humans? they can't, and it's as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it's being misdiagnosed as sporadic CJD. ...terry
*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ ***
*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).***
FRIDAY, JUNE 08, 2018
Chronic wasting disease management in ranched elk using rectal biopsy testing URGENT UPDATE!
http://chronic-wasting-disease.blogspot.com/2018/06/chronic-wasting-disease-management-in.html
Chronic wasting disease management in ranched elk using rectal biopsy testing URGENT UPDATE!
http://chronic-wasting-disease.blogspot.com/2018/06/chronic-wasting-disease-management-in.html
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA Diagnosis and Reporting of Creutzfeldt-Jakob Disease
To the Editor:
In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.
Terry S. Singeltary, Sr Bacliff, Tex
1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323.
TUESDAY, AUGUST 18, 2009
BSE-The Untold Story - joe gibbs and singeltary 1999 - 2009
SUNDAY, AUGUST 09, 2009
CJD...Straight talk with...James Ironside...and...Terry Singeltary... 2009
THURSDAY, DECEMBER 17, 2009
An Unusual Case of Variant CJD 18 December 2009
TUESDAY, SEPTEMBER 28, 2010
Variant CJD: where has it gone, or has it? Pract Neurol 2010; 10: 250–251
Variant CJD: where has it gone, or has it?
FRIDAY, AUGUST 12, 2011
Creutzfeldt-Jakob disease (CJD) biannual update (2011/2), Incidents Panel, National Anonymous Tonsil Archive
Published Date: 2012-09-06 17:44:35
Subject: PRO/AH/EDR> Prion disease update 2012 (09)
Archive Number: 20120906.1284090
Archive Number: 20120906.1284090
PRION DISEASE UPDATE 2012 (09)
Wednesday, December 21, 2016
TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES 2016 ANNUAL REPORT ARS RESEARCH
Wednesday, December 14, 2016
Diagnosis of Human Prion Disease Using Real-Time Quaking-Induced Conversion Testing of Olfactory Mucosa and Cerebrospinal Fluid Samples
TUESDAY, MAY 31, 2016
New insights in the transfusional risk assessment of variant Creutzfeldt-Jakob Disease: Transfusional transmission of vCJD prions in the absence of detectable abnormal prion protein Prion 2016 Tokyo
Prion 2016 Conference Tokyo
Wednesday, December 14, 2016
Diagnosis of Human Prion Disease Using Real-Time Quaking-Induced Conversion Testing of Olfactory Mucosa and Cerebrospinal Fluid Samples
THURSDAY, JANUARY 19, 2017
Variant Creutzfeldt–Jakob Disease in a Patient with Heterozygosity at PRNP Codon 129
TUESDAY, DECEMBER 12, 2017
Creutzfeldt Jakob Disease CJD National Prion Disease Pathology Surveillance Center Cases Examined to December 14, 2017
http://creutzfeldt-jakob-disease.blogspot.com/2017/12/creutzfeldt-jakob-disease-cjd-national.html
Tuesday, December 12, 2017
Neuropathology of iatrogenic Creutzfeldt–Jakob disease and immunoassay of French cadaver-sourced growth hormone batches suggest possible transmission of tauopathy and long incubation periods for the transmission of Abeta pathology
http://tauopathies.blogspot.com/2017/12/neuropathology-of-iatrogenic.html
MONDAY, OCTOBER 02, 2017
Creutzfeldt Jakob Disease United States of America USA and United Kingdom UK Increasing and Zoonotic Pontential From Different Species
http://creutzfeldt-jakob-disease.blogspot.com/2017/10/creutzfeldt-jakob-disease-united-states.html
THURSDAY, AUGUST 17, 2017
*** Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States revisited 2017
Singeltary et al
http://creutzfeldt-jakob-disease.blogspot.com/2017/08/monitoring-occurrence-of-emerging-forms.html
Creutzfeldt Jakob Disease CJD National Prion Disease Pathology Surveillance Center Cases Examined to December 14, 2017
http://creutzfeldt-jakob-disease.blogspot.com/2017/12/creutzfeldt-jakob-disease-cjd-national.html
Tuesday, December 12, 2017
Neuropathology of iatrogenic Creutzfeldt–Jakob disease and immunoassay of French cadaver-sourced growth hormone batches suggest possible transmission of tauopathy and long incubation periods for the transmission of Abeta pathology
http://tauopathies.blogspot.com/2017/12/neuropathology-of-iatrogenic.html
MONDAY, OCTOBER 02, 2017
Creutzfeldt Jakob Disease United States of America USA and United Kingdom UK Increasing and Zoonotic Pontential From Different Species
http://creutzfeldt-jakob-disease.blogspot.com/2017/10/creutzfeldt-jakob-disease-united-states.html
THURSDAY, AUGUST 17, 2017
*** Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States revisited 2017
Singeltary et al
http://creutzfeldt-jakob-disease.blogspot.com/2017/08/monitoring-occurrence-of-emerging-forms.html
TUESDAY, JUNE 05, 2018
sporadic CJD or BSe, Bull Shit Encephalopathy diagnosis of one in a million, the truth of the matter
SUNDAY, APRIL 8, 2018
Transmissible Spongiform Encephalopathy TSE Prion Disease Global Pandemic Urgent Update April 9, 2018
Wednesday, May 30, 2018
Dromedary camels in northern Africa have a neurodegenerative prion disease that may have originated decades ago
***> IMPORTS AND EXPORTS <***
SEE MASSIVE AMOUNTS OF BANNED ANIMAL PROTEIN AKA MAD COW FEED IN COMMERCE USA DECADES AFTER POST BAN
*** PRION 2018 CONGRESSIONAL ABSTRACT AND POSTER BOOKS ??? ***
-----Original Message-----
From: Terry Singeltary
To: xestioneventos
Sent: Fri, Jun 22, 2018 9:14 am
Subject: Re: PRION 2018 CONGRESSIONAL ABSTRACTS AND POSTERS
From: Terry Singeltary
To: xestioneventos
Sent: Fri, Jun 22, 2018 9:14 am
Subject: Re: PRION 2018 CONGRESSIONAL ABSTRACTS AND POSTERS
very sad for those that donated their loved ones brains for this research, and very disturbing that you will not share this information with them. this is the first year of ALL PRION CONFERENCE that this information was/is not shared...very sad...thank you, kind regards, terry
-----Original Message-----
From: Oficina de Congresos USC
To: 'Terry Singeltary' <flounder9@verizon.net>
Sent: Fri, Jun 22, 2018 2:20 am
Subject: RE: PRION 2018 CONGRESSIONAL ABSTRACTS AND POSTERS
From: Oficina de Congresos USC
To: 'Terry Singeltary' <flounder9@verizon.net>
Sent: Fri, Jun 22, 2018 2:20 am
Subject: RE: PRION 2018 CONGRESSIONAL ABSTRACTS AND POSTERS
Dear friend,
we are not allowed to share this information. As long as I know, this information is just available for the conference attendants.
best regards,
xxxxxxxxx
Oficina de Congresos da USC
Complexo CEA-CE
Parque Vista Alegre - Rua Salvadas s/n
15705 Santiago de Compostela
tel 881816329 ext 16329
E-mail:@usc.es
De: Terry Singeltary [mailto:flounder9@verizon.net]
Enviado el: jueves, 21 de junio de 2018 18:19
Para: @usc.es
Asunto: PRION 2018 CONGRESSIONAL ABSTRACTS AND POSTERS
Enviado el: jueves, 21 de junio de 2018 18:19
Para: @usc.es
Asunto: PRION 2018 CONGRESSIONAL ABSTRACTS AND POSTERS
Hello Again,
a kind greetings from Bacliff, Texas.
is there any way now that the conference is over, that i can get a copy of the PRION 2018 CONGRESSIONAL ABSTRACTS AND POSTERS. i have followed the tse prion science with great interest following the demise of my mother to the heidenhain variant of creutzfeldt jakob disease hvCJD. i have no access to the full conference pdf files, but it would be most appreciative i you might send them to me...
with kindest regards, terry
THURSDAY, MAY 24, 2018
Prion 2018 May, 22-25 2018 Santiago de Compostela
http://transmissiblespongiformencephalopathy.blogspot.com/2018/05/prion-2018-may-22-25-2018-santiago-de.html
Prion 2018 May, 22-25 2018 Santiago de Compostela
http://transmissiblespongiformencephalopathy.blogspot.com/2018/05/prion-2018-may-22-25-2018-santiago-de.html
NIH may destroy human brain collection
By Steve Mitchell Medical Correspondent
Washington, DC, Mar. 24 (UPI) -- The National Institutes of Health may discard part or all of a rare collection that includes hundreds of human brain samples from patients that suffered from a disorder similar to mad cow disease -- unless another researcher or institution takes them on, United Press International has learned.
Several scientists said the collection, which is held by the NIH's National Institute for Neurological Disorders and Stroke in Bethesda, Md. -- and includes brains and other tissue samples from people afflicted with the brain-wasting illness Creutzfeldt Jakob disease -- is irreplaceable and could even provide insight into treatments for the fatal disorder. Currently, there is no cure for CJD and patients typically die within a year after symptoms begin.
However, NIH officials in control of the collection's fate told UPI the remaining samples are of little scientific value and may be disposed of if researchers outside the agency do not claim it. That position stands in sharp contrast with CJD experts who thought the collection should be preserved.
"It's invaluable," said Dr. Paul Brown, former medical director of the NIH's Laboratory for Central Nervous System Studies, whose expertise is in CJD and mad cow disease (also known as bovine spongiform encephalopathy, or BSE).
The collection is badly in need of organization and no one is certain how many brains or other tissue samples it contains, said Brown, who worked with the collection since its inception in the 1960's until his retirement last year. There could be brains, blood, spinal fluid and various other tissues from 1,000 people or more, he said. Some of the specimens would be of scientific use today, he said.
"This collection has the unique value of stretching back to the beginning of when these diseases were discovered," Brown told UPI, noting that the first samples were obtained in 1963. "It would be as though you had in your hands the possibility of finding out when AIDS started."
Bruce Johnson, a former technician at the CNSS lab who worked extensively with the collection before he retired in 2003, told UPI he was told "in two years they (NIH officials)are going to destroy it, if nobody wants it."
Eugene Major, acting director of the basic neuroscience program at the NIH, said no specific timeframe had been established.
"We have not set a firm deadline date," Major told UPI. "We are working very hard with investigators that we know in order to be able to make sure that whatever we deem is valuable is potentially kept here." Some samples already have been determined not to have any research value and have been "removed and disposed of," he said.
Others samples have been given out to Dr. David Asher at the Food and Drug Administration and Pierluigi Gambetti at the National Prion Disease Pathology Surveillance Center in Cleveland, Ohio.
Major maintained the remaining collection was not particularly valuable for research. "Whatever had been collected here that has not already been distributed to responsible investigators who could use them really has very little remaining value," he said.
Neither Asher nor Gambetti returned phone calls from UPI, but Brown said he thought Asher had received only a dozen or two samples at most and Gambetti had not received much at all.
Neil Cashman, a brain-disease researcher at the University of Toronto's Center for Research in Neurodegenerative Diseases -- who has tried to obtain the collection from the NIH -- said it was priceless.
"It would be like destroying an art museum," Cashman told UPI. "There's all this information and insight that's locked up in these tissues and if it's destroyed it will be lost forever."
The Memorial Institute for Neurodegenerative Diseases Inc., a non-profit organization consisting of more than 40 university and institute researchers from the United States, Canada, United Kingdom and France, also thinks the brain collection is invaluable.
"It is the opinion of the Board of Directors ... of The MIND Inc., that the ... brain bank should not be broken up nor destroyed," said Harry E. Peery, MIND's executive director, in a letter to UPI. "We believe that this collection is of inestimable research value and should be kept intact."
The institute, at the University of Saskatchewan in Saskatoon, applied for possession of the collection in early 2004, but received a letter from the NINDS indicating the fate of the collection had not yet been determined.
"We have heard nothing further since that time" and continue to be interested in acquiring the complete collection, Peery said.
CJD belongs to a group of rare, brain-wasting disorders that are little understood, incurable and fatal. This includes mad cow disease in cows, chronic wasting disease in deer and elk. The most infamous of these illnesses in humans is variant CJD, which people can contract from eating beef products infected with the mad-cow pathogen.
Although vCJD has infected more than 154 people worldwide, only one case has ever been detected in the United States -- in a Florida woman who is thought to have contracted the disease while living in the United Kingdom. However, the NIH brain samples have never been screened for vCJD -- something Johnson thinks is critically important.
"No one has ever looked to see if any American (in the collection) in the past had variant CJD," Johnson said. "You think it would be required that they do that. You think it would be a Congressional mandate that they test these brains: 'Let's see if we've got this disease in our country.'"
Johnson noted at least one brain in the collection he personally had examined -- from a French woman collected in 1971 -- showed evidence of possible vCJD infection, but the sample needed further study to be sure.
Other samples in the collection include the brains of patients who were only 16 years old when they were diagnosed with CJD. This would be unusual for sporadic CJD, because generally it strikes those over age 60. Variant CJD, on the other hand, typically occurs in patients in their 20s or younger.
"I thought it was absolutely vital (to test these brains)," Johnson said. "Maybe there's a dozen cases in there of variant CJD."
Major disagreed. "There's really no reason to do that," he said. "The effort it would take to screen those samples ... would not give us any new insights into variant CJD beyond what it is we already know."
Johnson said he was frustrated with the NIH administration's lack of interest in preserving the collection or testing for vCJD. "They don't understand," he said, "they honest-to-god don't understand what it's all about."
Patient advocates also objected to the possible destruction of the brains.
Terry Singeltary, whose mother died of a type of CJD called Heidenhain variant in 1997, said he is outraged and families of other CJD victims probably will be, too.
"A lot of these families went through a lot of heartache and a lot of trouble to get these brain samples to the NIH," Singeltary told UPI. "Now they're just going to discard them because they're not of scientific use? That's just asinine. That stuff is valuable information."
Graham Steel, vice-chair of the Human BSE Foundation in the United Kingdom, told UPI, "The potential loss of such important tissue samples would be a massive blow for TSE (the group of diseases that includes CJD and BSE) research in the United States. This should not be allowed to happen."
Singeltary noted there currently is no cure for these diseases. "If you don't have any answers yet, why would you throw these specimens away?" he asked.
He added that more sensitive tests are just becoming available and could help determine the origin of some of the CJD cases. "We've all been sitting around waiting for more sensitive tests to get validated because we want answers," he said.
"You know, it must be an embarrassment," Johnson said. "Some Senator is going to eventually say 'What is NIH doing about mad cow disease?' And people are going to scratch their heads and say 'not much'." He added, "What's going to happen (is) one of these senators or their wife is going to develop spontaneous CJD one day and ... there's going to be hell raised and they're going to ask, 'Why isn't NIH working on this?'"
--
SCIENCE NEWS
Groups seek to save NIH brain collection
Published: April 1, 2005 at 4:48 PM By STEVE MITCHELL, Medical Correspondent
WASHINGTON, April 1 (UPI) -- Scientists, consumer groups and patient-advocates have embarked upon efforts -- including petitioning members of Congress and seeking storage space at a Canadian university -- to prevent the National Institutes of Health from destroying an irreplaceable collection of human brains from patients afflicted with a condition similar to mad cow disease.
As United Press International reported last week, the NIH has begun shopping for a new home for its collection of brains, spinal fluid and other tissues from hundreds of patients around the world who died from Creutzfeldt Jakob disease -- an incurable, fatal, brain-wasting illness. The collection dates back to 1963 and the consensus among scientists in this field is it is invaluable for research and could provide insights that might aid in developing diagnostic tests, treatments or cures for CJD.
NIH officials, however, maintain the remaining samples in the collection -- stored in some 30 freezers by the National Institute for Neurological Disorders and Stroke in Bethesda, Md. -- are of little value and may be disposed of if researchers or institutions do not come forward to claim them.
Families of patients who died of CJD have reacted with outrage, concerned that the effort mounted to collect the brains in the first place has been all for naught. Several have contacted their respective members of Congress and urged them to step in.
"The brains and brain tissue were sent to NIH in good faith for future research and destroying them is an outrage," Terry Singeltary, a patient advocate in Bacliff, Texas, wrote in a letter to Sen. Kay Bailey Hutchinson, R-Texas, and several other members of the state's congressional delegation. Singeltary's mother died of a type of CJD called Heidenhain variant in 1997.
Hutchinson's office did not return a call from UPI.
Eugene Major, who serves as acting director of the NINDS and is responsible for the fate of the brain collection, did not return a call from UPI.
"The patients these brains were taken from suffered greatly before they died of CJD," Heather Larson of Phoenix, whose mother succumbed to CJD last year at the age of 56, wrote in a letter to Arizona Republican Sens. John McCain and Jon Kyl, and Republican Rep. John Shadegg. "Their brains hold answers that can save human lives. Destroying the brains at Bethesda would greatly hinder the research being done to fight this disease and would cost many their lives."
The offices of McCain and Kyl did not return UPI's calls.
"The ravages of this disease, and the toll it takes not only on its victims but on family and loved ones, cannot easily be described to someone who has not witnessed it personally," Patty Cook of Kansas City, Kan., wrote in a letter to Kansas Republican Sens. Sam Brownback and Pat Roberts, and Democratic Rep. Dennis Moore.
"I urge you to do whatever you can to ensure these brains are not destroyed," added Cook, whose mother died of CJD in 1982.
Brownback's office did not return a call from UPI.
CJD belongs to a group of diseases -- called transmissible spongiform encephalopathies or TSEs -- that includes mad cow disease, chronic wasting disease in deer and elk, scrapie in sheep and several types of CJD in humans. There is no cure for CJD and it typically results in death within a year after the onset of symptoms.
Consumer groups also are concerned and are considering taking steps to ensure the brain collection will be preserved.
"This is outrageous," Michael Hansen, a biologist and senior research associate with Consumers Union in Yonkers, N.Y., told UPI. "Those brains are a critical resource for CJD science and they must be at a research facility."
Hansen added that his late friend, Joe Gibbs, the former chief of NINDS's Laboratory of Central Nervous System Studies, told him the brain of famed choreographer George Balanchine, who died of CJD in 1983, resides in the collection.
"How can we claim to be a scientific country if we're going to be throwing away an irreplaceable repository of the first evidence of these diseases?" asked Felicia Nestor, who serves as a consultant to Public Citizen.
There may be hope yet for the collection, however.
Neil Cashman, an expert on TSEs at the University of Toronto's Center for Research in Neurodegenerative Diseases, told UPI he has been attempting to drum up support for acquiring the collection with his colleagues at the University of British Columbia in Vancouver -- where he plans to move this summer.
"I'm trying to organize support for an official letter from UBC to NIH to request the collection," Cashman said.
The letter will probably go out in about a month, he said.
"The goal would be to make it a resource for the world and make the tissues available to scientists who had a reasonable request," he added.
Singeltary said he has heard from at least one other prominent scientist in this field who said they planned to contact the NIH and urge it to reconsider the fate of the collection.
One brain in the collection, that of a French woman who died in 1971, may help provide clues about the origins of variant CJD -- a condition similar to CJD that humans can contract from eating beef products contaminated with the mad-cow pathogen. The first recognized case of vCJD occurred in 1995 in the United Kingdom, but an NIH scientist said he tested the French woman's brain in 2000 and found signs consistent with vCJD -- not CJD.
French researchers currently are re-examining specimens from the case to determine if the woman was indeed infected with vCJD. If she was, it would suggest the disease began infecting people more than 20 years earlier than previously thought.
Cashman said the case underscores the value of the NIH brain collection.
"There is information locked up in these freezers that will be lost forever if this collection is destroyed," he said.
--
E-mail: sciencemail@upi.com
© 2005 United Press International, Inc. All Rights Reserved.
NIH sends mixed signals on CJD brains
By Steve Mitchell Medical Correspondent
Washington, DC, Apr. 7 (UPI) -- A National Institutes of Health official who told United Press International the agency might destroy its collection of brains from human patients afflicted with a condition similar to mad cow disease reportedly has told the head of a patient-advocate group the collection would be preserved.
The official, Eugene Major, acting director of the basic neuroscience program at the NIH, has not responded to e-mail or a phone call from UPI seeking clarification of his remarks, and the official status of the collection remains unknown.
As reported by UPI on March 24, the collection is stored in freezers by the NIH's National Institute for Neurological Disorders and Stroke in Bethesda, Md. It contains brains and other tissue samples from hundreds of people who died from the brain-wasting illness Creutzfeldt Jakob disease, as well as tissues from an untold number of experimental animals.
The consensus of scientists in this field is the collection, which dates back to 1963, is invaluable for research and could even provide insight into treatments for the fatal disorder. Currently, there is no cure for CJD and patients typically die within a year after symptoms begin.
Florence Kranitz, president of the non-profit advocacy group CJD Foundation, told UPI she had "a very long conversation" with Major, in which he told her the remaining tissues in the collection would not be destroyed.
"He reassured me in no uncertain terms," Kranitz said, noting constituents of the foundation and other CJD advocacy groups had been expressing concerns to her the tissues would be destroyed.
Kranitz, who has personal reasons for wanting the collection preserved -- her husband died of CJD in 2000 -- said she plans to meet with Major at the end of April to discuss the issue further.
CJD belongs to a group of diseases collectively known as transmissible spongiform encephalopathies, or TSEs, that includes mad cow disease in cows, chronic wasting disease in deer and elk, and scrapie in sheep. All TSEs are incurable and fatal.
Major previously told UPI some samples already have been destroyed and others have been given to researchers at the Food and Drug Administration and the National Prion Disease Pathology Surveillance Center in Cleveland.
Major said the remaining collection "has very little remaining value" and could be destroyed if another entity does not claim them.
Bruce Johnson, a former NIH scientist who retired at the end of 2003, said he had been told the collection would be destroyed in two years if no one took the samples from the NIH.
In response to hearing that Major had failed to confirm to UPI the brain collection would not be destroyed, Patricia Ewanitz, who lives in Port Jefferson Station, N.Y., and is founder of the advocacy group CJD Voice, said, "The brain tissue might not be indispensable to the National Institutes of Health but it is absolutely necessary to the families who thought enough of science to donate the brains, brain tissue and blood in hopes of someday finding an answer to why their loved one died."
Ewanitz, whose husband died of CJD in 1997, added, "It now seems like such a joke."
Terry Singeltary, whose mother passed away from a type of CJD in 1997, said the NIH should use the samples for scientific research, not just store them in freezers.
Both Singeltary and Ewanitz said they would feel more reassured if Major verified in writing the collection will not be destroyed.
"I would go further and ask Major what he plans to do with them," Singeltary said. "If the samples are just going to sit up there and go bad, then they should give them out to researchers looking for cause and cure."
The revelation the NIH might destroy part or all of the collection sparked an outcry from patient advocates, consumer groups and scientists.
Advocates have been contacting their members of Congress, urging them to investigate and prevent the NIH from destroying the brains. Consumer groups also have gotten involved and scientists have taken steps to obtain the collection or have urged Major not to destroy the samples.
Felicia Nestor, who serves as a consultant to Public Citizen, told UPI she had contacted certain legislators and at least one was considering looking into the situation. Nestor asked the legislator's name be withheld.
Kranitz said Major also told her he plans "to advertise in professional neurological journals and by whatever means necessary to make it known" to researchers in the field the tissues are available.
Major previously said, however, that efforts to inform researchers of the availability of the collection were already underway and included informing NIH grantees. He added he had personally notified researchers at scientific meetings, but no TSE researcher contacted by UPI was aware of this.
"I was never informed," said Laura Manuelidis, an expert on these diseases and section chief of surgery in the neuropathology department at Yale University. She said the first she had heard of the situation was in UPI's March 24 report.
Manuelidis also said she contacted Major, expressing interest in the specimens, but so far has not received a response.
"I sent a letter to (Major) on (March 25) about our interest in these specimens, but he has not replied," she told UPI in an e-mail.
Neil Cashman, a TSE expert at the University of Toronto, who said he was not aware the samples might be destroyed, has lobbied colleagues at the University of British Columbia -- where Cashman is scheduled to move to this summer -- to help draft a letter requesting the collection.
The Memorial Institute for Neurodegenerative Diseases Inc., a non-profit organization consisting of more than 40 university and institute researchers from the United States, Canada, the United Kingdom and France, requested the collection in January, 2004. So far, the institute has not been informed of a decision by the NIH.
Asked if Major had told him whether the collection would be preserved, MIND Executive Director Harry Peery said, "We have heard nothing further from Eugene Major or anyone else at the NIH regarding the brain collection."
--
http://www.upi.com/Science_News/2005/04/07/NIH-sends-mixed-signals-on-CJD-brains/UPI-25701112902231/
my letter to Government Officials begging for help ;
-------- Original Message --------
Subject: NIH to destroy our loved ones brain tissues, WE NEED YOUR HELP PLEASE Date: Fri, 25 Mar 2005 16:04:57 -0600 From: "Terry S. Singeltary Sr."
To: senator@hutchison.senate.gov
CC: Judith.Zaffirini@senate.state.tx.us, Bob.Deuell@senate.state.tx.us, District98.Truitt@house.state.tx.us, District115.Jackson@house.state.tx.us, Jane.Nelson@senate.state.tx.us, District96.Zedler@house.state.tx.us, Jon.Lindsay@senate.state.tx.us, f-gilstrap@tamu.edu, ka-phillips@tamu.edu
References: <422ca640 .3030108="" wt.net="">
Greetings again Honorable Senator Hutchison and other Honorable Members of Texas Office,
My name is Terry S. Singeltary Sr. I lost my Mother to hvCJD aka mad cow.THE Heidenhain Variant of Creutzfeldt Jakob Disease. (there is more than one strain of mad cow disease and i will reference last)
I am once again writing to you on a matter of extreme importance. I would appreciate your assistance in writing to the National Institutes of Health requesting that the brain tissue collected over the years at NINDS from family members of Creutzfeldt-Jakob Disease victims be preserved and recorded and not discarded.
[See attached articles]
THE WASHINGTON TIMES UNITED PRESS INTERNATIONAL
NIH may destroy human brain collection
By Steve Mitchell Medical Correspondent Washington, DC, Mar. 24 (UPI) -- ...
SNIP...END...TSS
FINALLY ONE, and only one Senator, did reply to my concerns, and help us with preserving the brain tissue bank at NIH. I had sent a hard copy via US postal to the Honorable Senator John Cornyn (see below), but none of the (above) Senators and other officials ever bothered to reply.
See The Honorable John Cornyn Reply below ;
JOHN CORNYN TEXAS UNITED STATES SENATE WASHINGTON, DC 20510-4305 April 26,2005
Mr. Terry Singeltary
P.O. Box 42
Bacliff, Texas 77518
Dear Mr. Singeltary:
In response to your recent request for my assistance, I have contacted the National Institutes of Health. I will write you again as soon as I receive a reply. I appreciate having the opportunity to represent you in the United States Senate and to be of service in this matter.
Sincerely,
JOHN CORNYN United States Senator JC:djl
===============
JOHN CORNYN TEXAS UNITED STATES SENATE WASHINGTON, DC 20510-4305
May 18,2005
Mr. Terry Singeltary
P.O. Box 42
Bacliff, Texas 77518
Dear Mr. Singeltary:
Enclosed is the reply I received from the Department of Health and Human Services in response to my earlier inquiry on your behalf. I hope this will be useful to you. I appreciate having the opportunity to represent you in the United States Senate. Thank you for taking time to contact me. Sincerely,
JOHN CORNYN United States Senate JC:djl Enclosure
DEPARTMENT OF HEALTH & HUMAN SERVICES National Institutes of HealthNational Institute of Neurological Disorders and Stroke NINDS Building 31, Room 8A52 31 Center Dr., MSC 2540 Bethesda, Maryland 20892-2540 Phone: 301-496-9746 Fax: 301-496-0296 Email: [log in to unmask]
May 10, 2005
The Honorable John Cornyn United States Senator Occidental Tower5005 LBJ Freeway, Suite 1150Dallas, Texas 75244-6199
Dear Senator Cornyn:
Your letter to the National Institutes of Health (NIH) forwarding correspondence from Mr. Terry S. Singeltary, Sr., has been forwarded to me for reply. Mr. Singeltary is concerned about the preservation of Creutzfeldt-Jakob disease (CJD) brain samples that have been maintained by the National Institute of Neurological Disorders and Stroke (NINDS) Intramural Research program for many years. I am sorry to learn that Mr. Singeltary's mother died of CJD and can certainly understand his desire that any tissues that could help investigators unravel the puzzle of this deadly disease are preserved. I hope he will be pleased to learn that all the brains and other tissues with potential to help scientists learn about CJD are, and will continue to be, conserved. (The tissues that are discarded are those that have either decayed to an extent that renders them no longer appropriate for research or those for which we do not have sufficient identification.) The purpose of gathering these brains and tissues is to help scientists learn about CJD. To that end, some of the NINDS-held samples are distributed to investigators who can demonstrate that they have a compelling research or public health need for such materials. For example, samples have been transferred to NIH grantee Dr. Pierluigi Gambetti, who heads the National Prion Diseases Pathology Surveillance Center at Case Western Reserve University in Ohio and works with the Centers for Disease Control and Prevention to monitor all cases of CJD in the United States. Dr. Gambetti studies the tissues to learn about the formation, physical and chemical properties, and pathogenic mechanisms of prion proteins, which are believed to be involved in the cause of CJD. Samples have also been transferred to Dr. David Asher, at the U.S. Food and Drug Administration, for use in assessing a potential diagnostic test for CJD.
Page 2 - The Honorable John Cornyn
in closing, we know that donating organs and tissue from loved ones is a very difficult and personal choice that must often be made at the most stressful of times. We at the NINDS are grateful to those stalwart family members who make this choice in the selfless hope that it will help others afflicted with CJD. We also know the invaluable contribution such donations make to the advancement of medical science, and we are dedicated to the preservation of all of the tissue samples that can help in our efforts to overcome CJD.
I hope this information is helpful to you in responding to Mr. Singeltary. Sincerely,
Story C. Landis, Ph.D. Director, National Institute of Neurological Disorders and Stroke
==================================
NIH says it will preserve CJD brains
By STEVE MITCHELL
WASHINGTON, May 31 (UPI) -- The National Institutes of Health apparently has reversed its position on the fate of an invaluable collection of brains from people afflicted with a condition similar to mad cow disease, saying in a letter to a U.S. senator it will not destroy the collection.
An NIH official had told United Press International previously that the brain collection, which consists of samples from hundreds of people who died from the brain-wasting illness called Creutzfeldt Jakob disease, could be discarded if another entity does not claim them.
That sparked an outcry from patient-advocacy groups, consumer watchdogs and scientists, and the agency now appears to have backed away from that course.
"All the brains and other tissues with potential to help scientists learn about CJD are, and will continue to be, conserved," Story Landis, director of the National Institute of Neurological Disorders and Stroke, which oversees the brain collection, wrote in a May 10 letter to Sen. John Cornyn, R-Texas.
Cornyn had inquired about the status of the collection in April.
Last March, Eugene Major, acting director of the basic neuroscience program at the NIH, told UPI the useful portions of the collection had been doled out to scientists and the remaining samples had "very little remaining value" and could be destroyed.
Landis could not be reached for comment Tuesday. NINDS spokesman Paul Girolami told UPI he had been unable to locate her.
Scientists think the collection, which dates back to 1963, is invaluable for research on CJD and similar diseases and could even provide insight into treatments. There is no cure for CJD and patients typically die within a year after symptoms begin.
"Absolutely, the collection is worth keeping," Bruce Johnson, a former NIH scientist who said he had been told the collection would be destroyed in two years if no one took the samples from the agency, told UPI.
The Memorial Institute for Neurodegenerative Diseases Inc., a non-profit organization consisting of more than 40 researchers from several countries, offered to take the collection off of NIH's hands more than a year ago and so far has not heard anything from the agency, Harry Peery, MIND's executive director, told UPI.
CJD belongs to a group of incurable and fatal diseases collectively known as transmissible spongiform encephalopathies, or TSEs, that includes mad cow disease in cows, chronic wasting disease in deer and elk, and scrapie in sheep.
Variant CJD, or vCJD, is a relatively new TSE, which people can contract from consuming beef products infected with the mad cow pathogen.
Despite Landis' assurance the collection will be preserved, some family members of the patients who donated their brains to the NIH are still skeptical. This is because the wording Landis used in the letter leaves open the possibility that some brain samples are being destroyed.
"The tissues that are discarded are those that have either decayed to an extent that renders them no longer appropriate for research or those for which we do not have sufficient identification," Landis wrote.
"Which ones" are being destroyed? asked Terry Singeltary, who is involved with several CJD patient groups.
"With a system like this, they could destroy whatever and whenever they wanted, for whatever reason they wanted," Singeltary, whose mother died of CJD in 1997, told UPI.
"It's a perfect excuse to discard some suspicious tissue resembling vCJD or some atypical TSE related to animal TSEs in the USA," he added.
Although the collection includes samples from CJD patients as young as 16 that could make them candidates for possible vCJD, the brains have never been screened for evidence of the disease. The only confirmed vCJD case in the United States occurred in a Florida woman who is thought to have contracted the disease in England.
Johnson said he along with renowned CJD expert Paul Brown were in the process of sorting through the samples to match them up with patient identification documents until they both retired. Some of the samples may prove impossible to identify, he said, but he and Brown are the only ones familiar enough with the collection to organize it and neither has been asked back by the agency to aid in the identification process.
Steve Mitchell is UPI's Medical Correspondent. E-mail: [log in to unmask]
Copyright 2005 by United Press International. All Rights Reserved.
=====================
US scientist accused of selling tissue samples Deal said to earn $285,000 for vials that cost millions By Diedtra Henderson, Globe Staff June 14, 2006
WASHINGTON -- A senior government scientist pocketed hundreds of thousands of dollars as a drug company consultant in exchange for human tissue samples that cost the federal government millions to acquire, congressional investigators said yesterday .
The House Energy and Commerce Committee report, the culmination of a one-year inquiry, was released hours before a two-day hearing began to explore the government's practices for procuring human tissue samples. According to congressional investigators, the National Institutes of Health's Dr. Trey Sunderland agreed to collaborate with Pfizer Inc. , the world's largest drug company. Sunderland, chief of the geriatric psychiatry branch of the National Institute for Mental Health , sent Pfizer 3,200 tubes of spinal fluid and 388 tubes of plasma collected for Alzheimer's research.
The government spent $6.4 million to obtain the 3,500 samples that showed how Alzheimer's disease progressed in 538 subjects.
Pfizer paid Sunderland $285,000 in consulting fees related to the samples, investigators said. In total, Pfizer paid him more than $600,000 from 1998 to 2004 for outside consulting and speaking fees. Sunderland is scheduled to testify today at the hearing.
``Contrary to the House committee report, Dr. Sunderland did not receive any payments from Pfizer for human tissue samples," said Robert F. Muse, the scientist's Washington, D.C., attorney. ``He acted properly, ethically, and legally in his relationship with Pfizer."
Pfizer spokeswoman Kate Robins said the company had a transfer agreement endorsed by the NIH that permitted Sunderland to send cerebrospinal fluid from research participants with Alzheimer's, the participant's relatives who were at higher risk of developing the neurological disease, and elderly adults with normal Alzheimer's risk.
Sunderland's consulting role tapped his Alzheimer's disease expertise to look for signals in the samples that could help identify and diagnose the disease.
``The payments over a six-year period were reasonable and customary for an expert of Dr. Sunderland's stature, and reflect the fair-market value of his consulting services," Robins said.
The report said the tissue transfers, reported by a government whistleblower, raised serious questions about how the government ensures its scientists do not abuse their positions and about the agency's ability to track the valuable samples.
``NIH tells us it has no centralized inventory system that could tell the NIH director how many vials of tissues are in freezers at a particular institute," said Representative Joe Barton , Republican of Texas and House Energy and Commerce Committee chairman . ``It would really be a shame if we find out that the National Institutes of Health has more control over its paper clips and trash cans than it has over its human tissue samples."
John T. Burklow , a NIH spokesman, said the agency shares ``the committee's concerns in regard to the ethical management of human tissue samples."
Sunderland's arrangement with the drug maker -- made without NIH knowledge, according to Burklow -- occurred after the agency relaxed its ethics policy covering scientists' outside activities and ended before the agency enacted more stringent rules.
The NIH, pressured by Barton's committee, on Aug. 25 curbed outside consulting deals between its scientists and pharmaceutical and biotechnology companies.
Diedtra Henderson can be reached at [log in to unmask]
© Copyright 2006 Globe Newspaper Company.
NIH Scientist Exploited Human Tissue Samples For Personal Gain, Report Finds
Academic Shipped 3,500 Tubes to Pfizer; Pocketed $285,000
WASHINGTON - A senior scientist at the National Institutes of Health (NIH) shared thousands of valuable human tissue samples with the drug giant Pfizer, netting himself at least $285,000 and raising serious questions about existing government safeguards against abuse, a new House Energy and Commerce Committee staff report has found.
The report was released today in conjunction with a two-day Subcommittee on Oversight and Investigations hearing entitled "Human Tissue Samples: NIH Research Policies and Practices."
According to the committee's investigation, Dr. Trey Sunderland, chief of the Geriatric Psychiatry Branch of the National Institute for Mental Health, shipped 3,200 tubes of spinal fluid and 388 tubes of plasma collected for Alzheimer's research. As a result, Dr. Sunderland was paid $285,000 by Pfizer for consulting work related to the samples. All told, Dr. Sunderland received more than $600,000 in payments from Pfizer from 1998 to 2004 for outside consulting and speaking with any record of prior approval or disclosure in his government financial report filings.
Scientists considered the 3,500 samples, chronicling the progression of Alzheimer's disease on the same subjects over several years, to be priceless. An analysis by committee staff determined that simply procuring the samples from 538 subjects cost U.S. taxpayers $6.4 million. "It was unlikely that anywhere but at the clinics of NIH could this unique historical collection of human tissue samples be assembled," the report reads.
Committee investigators concluded that there were "reasonable grounds to believe" that obtaining the samples was a "primary reason for Pfizer's interest in collaborating with Dr. Sunderland."
The NIH's Office of Management Assessment has found that Dr. Sunderland's misconduct violated ethics rules as well as federal law and regulation. Yet, years after the transactions, he has not been prosecuted or disciplined to any measurable degree. He remains an employee at NIH and a member of the Public Health Service Corps.
The committee's year-long inquiry into the collections, storage, tracking and use of human tissue samples in the NIH's intramural research program was prompted in part by concerns raised by a former NIH scientist, Dr. Susan Molchan, in April 2005.
Human tissues include everything from DNA to organs to blood and they play a critical role in modern scientific research. According to the RAND Corporation, there are more than 307 million tissue samples from more than 178 million people stored in the United States .
Among the report's other findings:
The National Institutes of Health have "no uniform, centralized, and mandatory authority regulating the handling of human tissue samples. Some NIH laboratories kept a written record on the maintenance of these samples, but other NIH laboratories did not." NIH had "no formal inventory control or tracking system. ... Moreover, the lack of accountability left NIH wholly vulnerable to theft and diversion of valuable human tissue samples. These preliminary inquiries raised serious concerns over what was described to committee staff by NIH officials of a fairly loose, ad-hoc approach to controlling human tissue samples." http://energycommerce.house.gov/108/News/06132006_1941.htm
Barton: NIH Must Regain Control of Tissue Samples, Ethical Lapses 'It would really be a shame if we find out that the National Institute of Health has more control over its paper clips and trash cans than it has over its human tissue samples.' - Chairman Joe Barton
WASHINGTON - U.S. Rep. Joe Barton, R-Texas, chairman of the House Energy and Commerce Committee, issued the following statement today as part of an Oversight and Investigations Subcommittee hearing entitled "Human Tissue Samples: NIH Research Policies And Practices:"
"As I said, at the last set of oversight hearings on NIH, the hallmark of this committee has always been its oversight responsibility and its willingness and ability to hold agencies responsible under its jurisdiction and that produce results in better government and better services for the American people.
"Two years ago, we found that there were weaknesses in the system at that time and that those weaknesses were more severe than had been previously recognized. To his credit, Dr. Zerhouni, had a ringside seat at these hearings and took the facts of the hearings seriously and changed and reformed the NIH ethics system.
"Today we are going to take a look at how NIH protects its most precious assets -- the material that is at the core of the NIH research mission: human tissue samples. Once again, after extensive investigation on a bipartisan basis, we have found deeper concerns regarding human tissue samples at NIH than we first believed. We have found a lack of a centralized database and a lack of oversight at NIH that could, and probably does, leave NIH laboratory vulnerable to the risks of theft and abuse. We know from previous investigations that NIH has an inventory system, but NIH tells us it has no centralized inventory system that could tell the NIH director how many vials of tissues are in freezers at a particular institute. It would really be a shame if we find out that the National Institute of Health has more control over its paper clips and trash cans than it has over its human tissue samples.
"The committee has investigated a case and found evidence of a serious breach of trust. This case has focused on Dr. Trey Sunderland, who is supposed to be a witness later in this hearing. He's a very noted and respected researcher in the field of Alzheimer's disease. I wish we were here to discuss some great discovery he made to cure, or at least alleviate, the hazards of Alzheimer's. Instead, we are discussing the ways he used his position to use NIH spinal fluid samples to further his undisclosed, personal consulting. The information provided to the committee shows that Pfizer paid Dr. Sunderland $285,000 during the 1998-2003 time period to consult on two projects involving spinal fluid samples that Dr. Sunderland sent to Pfizer. During this same time period, Pfizer also paid Dr. Sunderland approximately $300,000 for lectures. These figures don't even count almost $200,000 additional for undisclosed activities with other companies. There is also evidence that he advised his subordinate to conceal these consulting activities involving the samples. This is from an official who for 10 years chaired a committee that reviews the ethics of conducting mental health research on human beings. This certainly appears to be a betrayal of the public trust that NIH stands for.
"These hearings underscore the need to enact NIH reauthorization and reform legislation. The NIH director must have some baseline of information about NIH assets. If we are going to gain new efficiencies and hopefully more effective ways to translate research into better healthcare, enacting NIH reauthorization legislation is of the highest importance.
"Out of this investigation of disturbing questions and concerns, we can use these hearings to make NIH stronger and better. The National Institute of Health is indeed a national treasure. It must be cherished, protected, nourished and allowed to flourish. Today's hearings are a first step toward strengthening public trust in NIH research and preserving confidence in its integrity.
"I thank you, Mr. Chairman, and Mr. Stupak for the bipartisan nature of this investigation. I also thank Mr. Dingell for his support of this investigation. Finally, I look forward to working with Dr. Zerhouni and the leadership of NIH on this matter and helping NIH become better managed -- and thus deliver the results for the health of America that we so depend on NIH to do."
####
--------------------------------------------------------------------------------
FOR IMMEDIATE RELEASE Tuesday, June 13, 2006
CONTACT: OD Office of Communications and Public Liaison 301-496-5787
NIH Statement Regarding House Hearing on Human Tissue Samples
Attribution: John Burklow, NIH spokesman
NIH’s position on ethics is clear: any conflict of interest resulting in an individual personally profiting from official government research activities cannot be tolerated. We are committed to maintaining the public’s trust in NIH and its scientists as an unbiased source of biomedical research guidance and advice. The case under consideration concerns events that began in 1998 — after the NIH ethics rules concerning outside activities were relaxed — and that ended before the new rules were put in place. NIH has previously referred this case to the relevant authorities for appropriate action.
It is important to note that the specific consulting arrangements in question, had they been known to NIH, would not have been approved under the present or previous ethics regulations. Outside consulting connected to an NIH employee’s official government duties has always been prohibited at NIH.
NIH has undertaken a comprehensive review of its activities and conflict of interest policies in the last few years. As a result of that process, on August 25, 2005, NIH implemented comprehensive ethics rules that make it clear what NIH scientists can and cannot do in regard to outside activities. These new rules removed any ambiguity about what is allowed or not allowed. Here are two important points:
Under new NIH regulations, all NIH employees are now prohibited from engaging in outside employment with pharmaceutical companies and biotechnology companies in their private capacities — period. Collaboration and partnership with industry can nonetheless be very valuable in scientific pursuits and NIH rules allow such activities, as long as they are undertaken through an officially approved Cooperative Research and Development Agreement (CRADA). Although we cannot discuss this particular case because it remains under investigation, we can speak to the relevant issues that it raises.
Collaborations among scientists that involve human tissue samples are common and essential for science. There are, however, stringent rules in place to protect the participants who donated their samples, and to ensure that there is full informed consent.
We share the Committee’s concerns in regard to the ethical management of human tissue samples and the development of rigorous and uniform policies to protect the public’s trust and interests, while advancing science to address important public health problems. The thousands of scientists who work at NIH have always been and remain committed to these principles.
The Office of the Director, the central office at NIH, is responsible for setting policy for NIH, which includes 27 Institutes and Centers. This involves planning, managing, and coordinating the programs and activities of all NIH components. The Office of the Director also includes program offices which are responsible for stimulating specific areas of research throughout NIH. Additional information is available at http://www.nih.gov/icd/od/.
The National Institutes of Health (NIH) — The Nation's Medical Research Agency — includes 27 Institutes and Centers and is a component of the U.S. Department of Health and Human Services. It is the primary federal agency for conducting and supporting basic, clinical and translational medical research, and it investigates the causes, treatments, and cures for both common and rare diseases. For more information about NIH and its programs, visit www.nih.gov.
THE LEGALITY OF STEALING ORGAN/TISSUE...TEXAS STATUTES
Friday, August 07, 2009
CJD Human Cornea Tissue, Recall END OF ENFORCEMENT REPORT FOR AUGUST 5, 2009
Trump administration tightens rules for federal scientists talking to reporters
Trump administration tightens rules for federal scientists talking to reporters A new directive from the Trump administration instructs federal scientists with the U.S. Geological Survey to get approval from its parent agency before agreeing to most interview requests from reporters, according to employees and emails from officials with the Department of the Interior and USGS.
USGS employees who spoke with The Times on condition of anonymity because they were unauthorized to do so say the new protocol represents a dramatic change in decades of past media practices at the scientific agency and will interfere with scientists’ ability to quickly respond to reporters’ questions. They expected that taxpayers would see less of the USGS’ scientific expertise as reporters seek scientific comment elsewhere.
The new protocol also permits the Department of the Interior’s communications office to reject interview requests on scientific matters.
Documents reviewed by The Times show that the Department of the Interior’s press secretary, Heather Swift, on April 25 sent an email saying that the standard protocol is “that interviews with a national outlet — such as the Washington Post, Discovery, NYT, The Atlantic, CNN, etc — go through approval. Additionally, topics that are either very controversial or that are likely to become a national story even if a regional reporter is asking (such as Houston Chronicle doing a story about an USGS employee discovering life on Mars) also go through approval.”
snip...
Current and former federal employees suggested the new protocols are an unwieldy attempt to control the voices of workers in the Department of the Interior, which employs some 70,000 people, including thousands of scientists at the top of their fields.
“This is really quite troubling. … In the 44 years I was with the agency, I was never required to go through anyone for authorization to speak with a reporter,” said William Ellsworth, former chief scientist of the USGS’ earthquake hazards team and now a professor of geophysics at Stanford University. “The USGS is a nonpolitical science agency. … These new roadblocks will not help them fulfill their mission.”
“You’re hamstringing the ability of the organization to listen to the needs of the public,” said Ross Stein, a USGS scientist emeritus and adjunct professor of geophysics at Stanford University.
Kate Kelly, a former director of communications at the Department of the Interior during the Obama administration, said it’s a problem when political appointees are put in a position where they can require scientists to obtain approval before speaking to journalists.
“This policy, if it’s in fact being implemented as such, has a lot of concerning implications. It essentially gives political appointees veto power over science, scientists and information that the American people should have access to,” Kelly said. “That introduces questions about what scientists are able to say, and whether what they’re sharing is some mangled version of the truth.”
Some also expressed concern about how the work of federal scientists is being affected in the Trump administration. The Center for Investigative Reporting wrote about the deletion of every mention of humans causing climate change in a draft report for the National Park Service; the references were later restored. The Washington Post reported on the deletionfrom a USGS news release about the role climate change played in rising sea levels and reductions in the number of scientists allowed to attend key conferences where they can share their work and learn new things.
Scientists at the United States Geological Survey (USGS) must now get approval first through their parent office
By David Grossman Jun 22, 2018 usgs official officeDOI/USGS If they want to talk to reporters, scientists at the United States Geological Survey (USGS) must now get approval first through their parent office, the Department of Interior (DOI). That's what anonymous USGS employees have told the Los Angeles Times.
The new policy allows for the DOI communications office to reject interview requests on scientific matters. The anonymous employees say that the new policies present "a dramatic change" in the office's media practices and will interfere with scientists’ ability to quickly respond to reporters’ questions.
snip...
“This policy, if it’s in fact being implemented as such, has a lot of concerning implications. It essentially gives political appointees veto power over science, scientists and information that the American people should have access to,” says Kate Kelly, a former DOI director of communications, to the Times.
Perhaps the biggest flashpoint between scientists and the higher ups at DOI would stem from the Administration's view of global warming.
Both Secretary Zinke and President Trump have repeatedly given incorrect informationregarding wind energy's effects on global warming, and President Trump has falsely statedthat global warming "was created by and for the Chinese." The DOI has removed factually true information about climate change from press releases and the agency has also curtailed the number of scientists on staff who can attend scientific conferences.
Source: L.A Times
CWD TSE PRION, USGS, FUTURE POLICY ON NEWS PUBLICATION FROM SCIENTIST, what will it be???
when will these type publications start MIA???
Exploring: Chronic Wasting Disease 690 ALL
TUESDAY, JUNE 05, 2018
sporadic CJD or BSe diagnosis of one in a million, the truth of the matter
this just went MIA after about 16 or 17 years being online www, i was right from day one, that is why it was removed...so sad.
remember two things about the federal government, big business, and corporate interest, and remember these two things and how many people was killed, asbestos and tobacco. now think TSE Prion aka mad cow type disease possibly in all species eventually. $$$
TUESDAY, JUNE 5, 2018
Sent: Monday, January 08,2001 3:03 PM
WOW, my submission held up on the www for 17 years, and was proven to be true, and now, it has been removed from the www, the same url does not work anymore and it was just working this year. nothing like the FDA et al cleaning up any evidence of truth with their mad cow debacle and sporadic cjd cover up contineus...so sad$$$
let's review the truth about sporadic cjd shall we;
Prion Scientific Advisors and Consultants Staff Meeting Singeltary Submission Freas Monday, January 08,2001 3:03 PM
Discussion: The C, L and H type BSE cases in Canada exhibit molecular characteristics similar to those described for classical and atypical BSE cases from Europe and Japan.
*** This supports the theory that the importation of BSE contaminated feedstuff is the source of C-type BSE in Canada.
*** It also suggests a similar cause or source for atypical BSE in these countries. ***
see page 176 of 201 pages...tss
*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply;
Wednesday, July 15, 2015
Additional BSE TSE prion testing detects pathologic lesion in unusual brain location and PrPsc by PMCA only, how many cases have we missed?
***however in 1 C-type challenged animal, Prion 2015 Poster Abstracts S67 PrPsc was not detected using rapid tests for BSE.
***Subsequent testing resulted in the detection of pathologic lesion in unusual brain location and PrPsc detection by PMCA only.
*** IBNC Tauopathy or TSE Prion disease, it appears, no one is sure ***
Posted by Terry S. Singeltary Sr. on 03 Jul 2015 at 16:53 GMT
ONE DECADE POST MAD COW FEED BAN OF AUGUST 1997...2007
2007
10,000,000 POUNDS 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.
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
PAGE NOT FOUND
ALABAMA MAD COW FEED IN COMMERCE 2006
RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINE -- CLASS II
______________________________
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
______________________________
PRODUCT
Bulk custom dairy feds manufactured from concentrates, Recall # V-113-6
CODE
All dairy feeds produced between 2/1/05 and 6/16/06 and containing H. J. Baker recalled feed products.
RECALLING FIRM/MANUFACTURER
Vita Plus Corp., Gagetown, MI, by visit beginning on June 21, 2006. Firm initiated recall is complete.
REASON
The feed was manufactured from materials that may have been contaminated with mammalian protein.
VOLUME OF PRODUCT IN COMMERCE
27,694,240 lbs
DISTRIBUTION
MI
______________________________
PRODUCT
Bulk custom made dairy feed, Recall # V-114-6
CODE
None
RECALLING FIRM/MANUFACTURER
Burkmann Feeds LLC, Glasgow, KY, by letter on July 14, 2006. Firm initiated recall is ongoing.
REASON
Custom made feeds contain ingredient called Pro-Lak, which may contain ruminant derived meat and bone meal.
VOLUME OF PRODUCT IN COMMERCE
?????
DISTRIBUTION
KY
END OF ENFORCEMENT REPORT FOR AUGUST 2, 2006
###
=====
PRODUCT
Bulk Whole Barley, Recall # V-256-2009
CODE
No code or lot number.
RECALLING FIRM/MANUFACTURER
Mars Petcare US, Clinton, OK, by telephone on May 21, 2009. Firm initiated recall is complete.
REASON
Product may have contained prohibited materials without cautionary statement on the label.
VOLUME OF PRODUCT IN COMMERCE
208,820 pounds
DISTRIBUTION
TX
END OF ENFORCEMENT REPORT FOR AUGUST 26, 2009
###
Subject: MAD COW FEED RECALL KY VOLUME OF PRODUCT IN COMMERCE ?????
Date: August 6, 2006 at 6:19 pm PST
PRODUCT Bulk custom made dairy feed, Recall # V-114-6
CODE None
RECALLING FIRM/MANUFACTURER Burkmann Feeds LLC, Glasgow, KY, by letter on July 14, 2006.
Firm initiated recall is ongoing. REASON Custom made feeds contain ingredient called Pro-Lak, which may contain ruminant derived meat and bone meal.
VOLUME OF PRODUCT IN COMMERCE ?????
DISTRIBUTION KY
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
###
Subject: MAD COW FEED BAN WARNING LETTER ISSUED MAY 17, 2006
Date: June 27, 2006 at 7:42 am PST Public Health Service Food and Drug Administration
New Orleans District 297 Plus Park Blvd. Nashville, TN 37217
Telephone: 615-781-5380 Fax: 615-781-5391
May 17, 2006
WARNING LETTER NO.. 2006-NOL-06
FEDERAL EXPRESS OVERNIGHT DELIVERY
Mr. William Shirley, Jr., Owner Louisiana.DBA Riegel By-Products 2621 State Street Dallas, Texas 75204
Dear Mr. Shirley:
On February 12, 17, 21, and 22, 2006, a U.S. Food & Drug Administration (FDA) investigator inspected your rendering plant, located at 509 Fortson Street, Shreveport, Louisiana. The inspection revealed significant deviations from the requirements set forth in Title 21, Code of Federal Regulations, Part 589.2000 [21 CFR 589.2000], Animal Proteins Prohibited in Ruminant Feed. This regulation is intended to prevent the establishment and amplification of Bovine Spongiform Encephalopathy (BSE). You failed to follow the requirements of this regulation; products being manufactured and distributed by your facility are misbranded within the meaning of Section 403(a)(1) [21 USC 343(a)(1)] of the Federal Food, Drug, and Cosmetic Act (the Act).
Our investigation found you failed to provide measures, including sufficient written procedures, to prevent commingling or cross-contamination and to maintain sufficient written procedures [21 CFR 589.2000(e)] because:
You failed to use clean-out procedures or other means adequate to prevent carryover of protein derived from mammalian tissues into animal protein or feeds which may be used for ruminants. For example, your facility uses the same equipment to process mammalian and poultry tissues. However, you use only hot water to clean the cookers between processing tissues from each species. You do not clean the auger, hammer mill, grinder, and spouts after processing mammalian tissues.
You failed to maintain written procedures specifying the clean-out procedures or other means to prevent carryover of protein derived from mammalian tissues into feeds which may be used for ruminants.
As a result . the poultry meal you manufacture may contain protein derived from mammalian tissues prohibited in ruminant feed. Pursuant to 21 CFR 589.2000(e)(1)(i), any products containing or may contain protein derived from mammalian tissues must be labeled, "Do not feed to cattle or other ruminants." Since you failed to label a product which may contain protein derived from mammalian tissues with the required cautionary statement. the poultry meal is misbranded under Section 403(a)(1) [21 USC 343(a)(1)] of the Act.
This letter is not intended as an all-inclusive list of violations at your facility. As a manufacturer of materials intended for animal feed use, you are responsible for ensuring 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 violations do not recur. Failure to promptly correct these violations may result in regulatory action, such as seizure and/or injunction, without further notice.
You should notify this office in writing within 15 working days of receiving this letter, outlining the specific steps you have taken to bring your firm into compliance with the law. Your response should include an explanation of each step taken to correct the violations and prevent their recurrence. If corrective action cannot be completed within 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 corrections have been made.
Your reply should be directed to Mark W. Rivero, Compliance Officer, U.S. Food and Drug Administration, 2424 Edenborn Avenue, Suite 410, Metairie, Louisiana 70001. If you have questions regarding any issue in this letter, please contact Mr. Rivero at (504) 219-8818, extension 103.
Sincerely,
/S
Carol S. Sanchez Acting District Director New Orleans District
PLEASE NOTE, THE FDA URLS FOR OLD WARNING LETTERS ARE OBSOLETE AND DO NOT WORK IN MOST CASES.. I LOOKED UP THE OLD ONE ABOVE AND FOUND IT, BUT HAVE NOT DONE THAT FOR THE OTHERS TO FOLLOW. THE DATA IS VALID THOUGH!
Subject: MAD COW PROTEIN IN COMMERCE USA 2006 RECALL UPDATE
From: "Terry S. Singeltary Sr." <[log in to unmask]>
Reply-To: SAFETY <[log in to unmask]>
Date: Mon, 9 Oct 2006 14:10:37 -0500
Subject: MAD COW FEED RECALL USA SEPT 6, 2006 1961.72 TONS
IN COMMERCE AL, TN, AND WV
Date: September 6, 2006 at 7:58 am PST
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
______________________________
snip...
Subject: MAD COW FEED RECALLS ENFORCEMENT REPORT FOR AUGUST 9, 2006 KY, LA, MS, AL, GA, AND TN 11,000+ TONS
Date: August 16, 2006 at 9:19 am PST RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINE - CLASS II
______________________________
snip...
______________________________
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
###
Product Details
Product Description:
CalDensity Black Label, CalDensity White Label with HA, packaged in white plastic 5, 15, 25, 40, 60 lb pails with plastic liner and white plastic lid. Reason for Recall:
During an FDA inspection it was found that the CalDensity Black label and CalDensity White Label with HA product containers did not include the precautionary statement DO NOT FEED TO CATTLE OR OTHER RUMINANTS
Product Quantity: 50,935 lbs
Recall Number: V-209-2012
Code Information: 042009, 051009, 061209, 071509, 091009, 011510, 030310, 031610, 052610, 092410, 120110, 011211, 020111, 030911, 050111, 071111 & 090111. Classification: Class II Event Details
Event ID: 61880
Voluntary / Mandated:
Voluntary: Firm Initiated
Product Type:
Veterinary
Initial Firm Notification of Consignee or Public:
E-Mail
Status:
Terminated
Distribution Pattern:
Nationwide distribution: AL, AR, AZ, CA, CO, FL, GA, IA, ID, IL, KY, LA, MD, MI, MN, MO, MS, NC, NE, NJ, NM, NY, OH, OK, PA, SC, TX, UT, VA, WA & WV. No shipments were made to foreign countries including Canada.
Recalling Firm:
Process Managers LLC
485 Gawthrope Dr
Winchester, KY 40391-8910
United States
Recall Initiation Date:
1/6/2012
Center Classification Date:
9/7/2012
Date Terminated:
1/24/2014
Product Details
Product Description:
Regular Chicken 50# Ingredients: Corn, Wheat, Oats, Oyster shells, Medium Grit, CCC, ADS, Plant Protein Products, Animal Protein Products, Processed Grain By-Products, Roughage Products, Animal Fat procession with DHA, etc
Reason for Recall:
During an FDA sample collection, the firms 50# Regular Chicken Feed was found to contain mammalian protein. The label does not contain the warning statement.
Product Quantity:
5400lbs (50lb bags)
Recall Number:
V-137-2013
Code Information:
8/6/2012
Classification:
Class III
Event Details
Event ID:
63743
Voluntary / Mandated:
Voluntary: Firm Initiated
Product Type:
Veterinary
Initial Firm Notification of Consignee or Public:
Other
Status:
Terminated
Distribution Pattern:
Midland MI area only.
Recalling Firm:
Cohoons Elevator Inc.
802 Townsend St
Midland, MI 48640-5362
United States
Recall Initiation Date:
11/21/2012
Center Classification Date:
2/8/2013
Date Terminated:
2/12/2013
V. Use in animal feed of material from deer and elk NOT considered at high risk for CWD
FDA continues to consider materials from deer and elk NOT considered at high risk for CWD to be acceptable for use in NON-RUMINANT animal feeds in accordance with current agency regulations, 21 CFR 589.2000.
Deer and elk not considered at high risk include:
(1) deer and elk from areas not declared by State officials to be endemic for CWD and/or to be CWD eradication zones; and
(2) deer and elk that were not at some time during the 60-month period immediately before the time of slaughter in a captive herd that contained a CWD-positive animal.
2017 Section 21 C.F.R. 589.2000, Animal Proteins Prohibited in Ruminant Feed
Subject: MICHIGAN FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE BREACH APRIL 4, 2017
MICHIGAN FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEEDVIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE BREACH APRIL 4, 2017
FDA BSE/Ruminant Feed Inspections Firms Inventory
11998 DET-DO MI 48846-847 OPR 4/4/2017 OAI
http://www.accessdata.fda.gov/scripts/BSEInspect/bseinspections.csv
NAI = NO ACTION INDICATED
OAI = OFFICIAL ACTION INDICATED
VAI = VOLUNTARY ACTION INDICATED
RTS = REFERRED TO STATE
OAI (Official Action Indicated) when inspectors find significant objectionable conditions or practices and believe that regulatory sanctions are warranted to address the establishment’s lack of compliance with the regulation. An example of an OAI classification would be findings of manufacturing procedures insufficient to ensure that ruminant feed is not contaminated with prohibited material. Inspectors will promptly re-inspect facilities classified OAI after regulatory sanctions have been applied to determine whether the corrective actions are adequate to address the objectionable conditions...end...TSS
TUESDAY, APRIL 18, 2017
*** EXTREME USA FDA PART 589 TSE PRION FEED LOOP HOLE STILL EXIST, AND PRICE OF POKER GOES UP ***
TUESDAY, JANUARY 17, 2017
FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEEDVIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE 2016 to 2017 BSE TSE PRION
FY 2016 Inspectional Observation Summaries
4132 21 CFR 589.2000(d)(1) Protein blenders, feed manufacturers, distributors Products that contain or may contain prohibited material fail to bear the caution statement, "Do not feed to cattle or other ruminants." Specifically, *** 2
4131 21 CFR 589.2000(c)(1)(i) Renderers Products that contain or may contain prohibited material fail to bear a label containing the caution statement, "Do not feed to cattle or other ruminants." Specifically, *** 1
FY 2015 Inspectional Observation Summaries
4132 21 CFR 589.2000(d)(1) Protein blenders, feed manufacturers, distributors Products that contain or may contain prohibited material fail to bear the caution statement, "Do not feed to cattle or other ruminants." Specifically, *** 2
FY 2014 Inspectional Observation Summaries
4146 21 CFR 589.2000(e)(1) Written clean-out procedures Failure to maintain written clean-out procedures to prevent carryover of protein derived from mammalian tissues to animal protein or feeds that may be used for ruminants. Specifically, *** 2
4131 21 CFR 589.2000(c)(1)(i) Renderers Products that contain or may contain prohibited material fail to bear a label containing the caution statement, "Do not feed to cattle or other ruminants." Specifically, *** 1
4132 21 CFR 589.2000(d)(1) Protein blenders, feed manufacturers, distributors Products that contain or may contain prohibited material fail to bear the caution statement, "Do not feed to cattle or other ruminants." Specifically, *** 1
4145 21 CFR 589.2000(e)(1) Use of clean-out procedures Failure to use clean-out procedures or other means adequate to prevent carryover of protein derived from mammalian tissues to animal protein or feeds that may be used for ruminants. Specifically, *** 1
FY 2013 Inspectional Observation Summaries
4131 21 CFR 589.2000(c)(1)(i) 5 Renderers Products that contain or may contain prohibited material fail to bear a label containing the caution statement, "Do not feed to cattle or other ruminants." Specifically, ***
4132 21 CFR 589.2000(d)(1) 5 Protein blenders, feed manufacturers, distributors Products that contain or may contain prohibited material fail to bear the caution statement, "Do not feed to cattle or other ruminants." Specifically, ***
4145 21 CFR 589.2000(e)(1) 1 Use of clean-out procedures Failure to use clean-out procedures or other means adequate to prevent carryover of protein derived from mammalian tissues to animal protein or feeds that may be used for ruminants. Specifically, ***
4146 21 CFR 589.2000(e)(1) 1 Written clean-out procedures Failure to maintain written clean-out procedures to prevent carryover of protein derived from mammalian tissues to animal protein or feeds that may be used for ruminants. Specifically, ***
FY 2012 Inspectional Observation Summaries
4131 21 CFR 589.2000(c)(1)(i) 5 Renderers Products that contain or may contain prohibited material fail to bear a label containing the caution statement, "Do not feed to cattle or other ruminants." Specifically, ***
4132 21 CFR 589.2000(d)(1) 4 Protein blenders, feed manufacturers, distributors Products that contain or may contain prohibited material fail to bear the caution statement, "Do not feed to cattle or other ruminants." Specifically, ***
FY 2011 Inspectional Observation Summaries
4132 21 CFR 589.2000(d)(1) 5 Protein blenders, feed manufacturers, distributors Products that contain or may contain prohibited material fail to bear the caution statement, "Do not feed to cattle or other ruminants."Specifically, ***
4131 21 CFR 589.2000(c)(1)(i) 4 Renderers Products that contain or may contain prohibited material fail to bear a label containing the caution statement, "Do not feed to cattle or other ruminants."Specifically, ***
4146 21 CFR 589.2000(e)(1) 1 Written clean-out procedures Failure to maintain written clean-out procedures to prevent carryover of protein derived from mammalian tissues to animal protein or feeds that may be used for ruminants. Specifically, ***
FY 2010 Inspectional Observation Summaries
4131 21 CFR 589.2000(c)(1)(i) 3 Renderers Products that contain or may contain prohibited material fail to bear a label containing the caution statement, "Do not feed to cattle or other ruminants." Specifically, *** 4132 21 CFR 589.2000(d)(1) 3 Protein blenders, feed manufacturers, distributors Products that contain or may contain prohibited material fail to bear the caution statement, "Do not feed to cattle or other ruminants." Specifically, ***
4146 21 CFR 589.2000(e)(1) 1 Written clean-out procedures Failure to maintain written clean-out procedures to prevent carryover of protein derived from mammalian tissues to animal protein or feeds that may be used for ruminants. Specifically, ***
FY 2009 Inspectional Observation Summaries
4132 21 CFR 589.2000(d)(1) 10 Protein blenders, feed manufacturers, distributors Products that contain or may contain prohibited material fail to bear the caution statement, "Do not feed to cattle or other ruminants." Specifically, ***
4146 21 CFR 589.2000(e)(1) 4 Written clean-out procedures Failure to maintain written clean-out procedures to prevent carryover of protein derived from mammalian tissues to animal protein or feeds that may be used for ruminants. Specifically, ***
4145 21 CFR 589.2000(e)(1) 3 Use of clean-out procedures Failure to use clean-out procedures or other means adequate to prevent carryover of protein derived from mammalian tissues to animal protein or feeds that may be used for ruminants. Specifically, ***
FY 2008 Inspectional Observation Summaries
4132 21 CFR 589.2000(d)(1) 7 Protein blenders, feed manufacturers, distributors Products that contain or may contain prohibited material fail to bear the caution statement, "Do not feed to cattle or other ruminants." Specifically, ***
4145 21 CFR 589.2000(e)(1) 1 Use of clean-out procedures Failure to use clean-out procedures or other means adequate to prevent carryover of protein derived from mammalian tissues to animal protein or feeds that may be used for ruminants. Specifically, *** 4146 21 CFR 589.2000(e)(1) 1 Written clean-out procedures Failure to maintain written clean-out procedures to prevent carryover of protein derived from mammalian tissues to animal protein or feeds that may be used for ruminants. Specifically, ***
FY 2007 Inspectional Observation Summaries
4132 21 CFR 589.2000(d)(1) 3 Protein blenders, feed manufacturers, distributors Products that contain or may contain prohibited material fail to bear the caution statement, "Do not feed to cattle or other ruminants." Specifically, ***
4146 21 CFR 589.2000(e)(1) 3 Written clean-out procedures Failure to maintain written clean-out procedures to prevent carryover of protein derived from mammalian tissues to animal protein or feeds that may be used for ruminants. Specifically, ***
4131 21 CFR 589.2000(c)(1)(i) 2 Renderers Products that contain or may contain prohibited material fail to bear a label containing the caution statement, "Do not feed to cattle or other ruminants." Specifically, ***
4145 21 CFR 589.2000(e)(1) 1 Use of clean-out procedures Failure to use clean-out procedures or other means adequate to prevent carryover of protein derived from mammalian tissues to animal protein or feeds that may be used for ruminants. Specifically, ***
FY 2006 Inspectional Observation Summaries
4132 21 CFR 589.2000(d)(1) 6 Protein blenders, feed manufacturers, distributors Products that contain or may contain prohibited material fail to bear the caution statement, "Do not feed to cattle or other ruminants."Specifically, ***
4146 21 CFR 589.2000(e)(1) 5 Written clean-out procedures Failure to maintain written clean-out procedures to prevent carryover of protein derived from mammalian tissues to animal protein or feeds that may be used for ruminants. Specifically, ***
4145 21 CFR 589.2000(e)(1) 4 Use of clean-out procedures Failure to use clean-out procedures or other means adequate to prevent carryover of protein derived from mammalian tissues to animal protein or feeds that may be used for ruminants. Specifically, ***
4131 21 CFR 589.2000(c)(1)(i) 2 Renderers Products that contain or may contain prohibited material fail to bear a label containing the caution statement, "Do not feed to cattle or other ruminants." Specifically, ***
*** PLEASE SEE THIS URGENT UPDATE ON CWD AND FEED ANIMAL PROTEIN ***
Sunday, March 20, 2016
Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed ***UPDATED MARCH 2016*** Singeltary Submission
SEE MAD COW FEED VIOLATIONS AFER MAD COW FEED VIOLATIONS ;
Tuesday, April 19, 2016
Docket No. FDA-2013-N-0764 for Animal Feed Regulatory Program Standards Singeltary Comment Submission
17 years post mad cow feed ban August 1997
Monday, October 26, 2015
FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE October 2015
Tuesday, December 23, 2014
FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEEDVIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE DECEMBER 2014 BSE TSE PRION
16 years post mad cow feed ban August 1997 2013
Sunday, December 15, 2013
FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE DECEMBER 2013 UPDATE
Saturday, August 29, 2009
FOIA REQUEST FEED RECALL 2009 Product may have contained prohibited materials Bulk Whole Barley, Recall # V-256-2009
Friday, September 4, 2009
FOIA REQUEST ON FEED RECALL PRODUCT 429,128 lbs. feed for ruminant animals may have been contaminated with prohibited material Recall # V-258-2009
Thursday, March 19, 2009
MILLIONS AND MILLIONS OF POUNDS OF MAD COW FEED IN COMMERCE USA WITH ONGOING 12 YEARS OF DENIAL NOW, WHY IN THE WORLD DO WE TO TALK ABOUT THIS ANYMORE $$$
Office of Inspector General Semiannual Report to Congress FY 2007 - 2nd Half
Two Texas Companies Sentenced and Fined for Misbranding Meat Products In April 2007, two closely held and related Texas companies pled guilty in Federal court and were sentenced to 12 months of probation and ordered to pay $10,250 in fines for misbranding meat products. One of the companies sold adulterated meat products to a retail store in New Mexico. Additionally, portions of the invoices failed to properly and consistently identify the meat products as being from cattle more than 30 months old at time of slaughter. This information is required to be disclosed because of bovine spongiform encephalopathy (BSE, or "mad cow disease") concerns. No adulterated meat reached consumers.
THE USDA JUNE 2004 ENHANCED BSE SURVEILLANCE PROGRAM WAS TERRIBLY FLAWED ;
CDC DR. PAUL BROWN TSE EXPERT COMMENTS 2006
In an article today for United Press International, science reporter Steve Mitchell writes:
Analysis: What that mad cow means
By STEVE MITCHELL UPI Senior Medical Correspondent
WASHINGTON, March 15 (UPI) -- The U.S. Department of Agriculture was quick to assure the public earlier this week that the third case of mad cow disease did not pose a risk to them, but what federal officials have not acknowledged is that this latest case indicates the deadly disease has been circulating in U.S. herds for at least a decade.
The second case, which was detected last year in a Texas cow and which USDA officials were reluctant to verify, was approximately 12 years old.
These two cases (the latest was detected in an Alabama cow) present a picture of the disease having been here for 10 years or so, since it is thought that cows usually contract the disease from contaminated feed they consume as calves. The concern is that humans can contract a fatal, incurable, brain-wasting illness from consuming beef products contaminated with the mad cow pathogen.
"The fact the Texas cow showed up fairly clearly implied the existence of other undetected cases," Dr. Paul Brown, former medical director of the National Institutes of Health's Laboratory for Central Nervous System Studies and an expert on mad cow-like diseases, told United Press International. "The question was, 'How many?' and we still can't answer that."
Brown, who is preparing a scientific paper based on the latest two mad cow cases to estimate the maximum number of infected cows that occurred in the United States, said he has "absolutely no confidence in USDA tests before one year ago" because of the agency's reluctance to retest the Texas cow that initially tested positive.
USDA officials finally retested the cow and confirmed it was infected seven months later, but only at the insistence of the agency's inspector general.
"Everything they did on the Texas cow makes everything they did before 2005 suspect," Brown said.
Despite this, Brown said the U.S. prevalence of mad cow, formally known as bovine spongiform encephalopathy, or BSE, did not significantly threaten human or cattle health.
"Overall, my view is BSE is highly unlikely to pose any important risk either in cattle feed or human feed," he said.
However, Jean Halloran of Consumers Union in Yonkers, N.Y., said consumers should be troubled by the USDA's secrecy and its apparent plan to dramatically cut back the number of mad cow tests it conducts.
"Consumers should be very concerned about how little we know about the USDA's surveillance program and the failure of the USDA to reveal really important details," Halloran told UPI. "Consumers have to be really concerned if they're going to cut back the program," she added.
Last year the USDA tested more than 300,000 animals for the disease, but it has proposed, even in light of a third case, scaling back the program to 40,000 tests annually.
"They seem to be, in terms of actions and policies, taking a lot more seriously the concerns of the cattle industry than the concerns of consumers," Halloran said. "It's really hard to know what it takes to get this administration to take action to protect the public."
The USDA has insisted that the safeguards of a ban on incorporating cow tissue into cattle feed (which is thought to spread the disease) and removal of the most infectious parts of cows, such as the brain and spinal cord, protect consumers. But the agency glosses over the fact that both of these systems have been revealed to be inadequately implemented.
The feed ban, which is enforced by the Food and Drug Administration, has been criticized by the Government Accountability Office in two reports, the most recent coming just last year. The GAO said the FDA's enforcement of the ban continues to have weaknesses that "undermine the nation's firewall against BSE."
USDA documents released last year showed more than 1,000 violations of the regulations requiring the removal of brains and spinal cords in at least 35 states, Puerto Rico and the Virgin Islands, with some plants being cited repeatedly for infractions. In addition, a violation of similar regulations that apply to beef exported to Japan is the reason why Japan closed its borders to U.S. beef in January six weeks after reopening them.
Other experts also question the adequacy of the USDA's surveillance system. The USDA insists the prevalence of mad cow disease is low, but the agency has provided few details of its surveillance program, making it difficult for outside experts to know if the agency's monitoring plan is sufficient.
"It's impossible to judge the adequacy of the surveillance system without having a breakdown of the tested population by age and risk status," Elizabeth Mumford, a veterinarian and BSE expert at Safe Food Solutions in Bern, Switzerland, a company that provides advice on reducing mad cow risk to industry and governments, told UPI.
"Everybody would be happier and more confident and in a sense it might be able to go away a little bit for (the USDA) if they would just publish a breakdown on the tests," Mumford added.
UPI requested detailed records about animals tested under the USDA's surveillance plan via the Freedom of Information Act in May 2004 but nearly two years later has not received any corresponding documents from the agency, despite a federal law requiring agencies to comply within 30 days. This leaves open the question of whether the USDA is withholding the information, does not have the information or is so haphazardly organized that it cannot locate it.
Mumford said the prevalence of the disease in U.S. herds is probably quite low, but there have probably been other cases that have so far gone undetected. "They're only finding a very small fraction of that low prevalence," she said.
Mumford expressed surprise at the lack of concern about the deadly disease from American consumers. "I would expect the U.S. public to be more concerned," she said.
Markus Moser, a molecular biologist and chief executive officer of Prionics, a Swiss firm that manufactures BSE test kits, told UPI one concern is that if people are infected, the mad cow pathogen could become "humanized" or more easily transmitted from person to person.
"Transmission would be much easier, through all kinds of medical procedures" and even through the blood supply, Moser said.
© Copyright 2006 United Press International, Inc. All Rights Reserved
CDC - Bovine Spongiform Encephalopathy and Variant Creutzfeldt ... Dr. Paul Brown is Senior Research Scientist in the Laboratory of Central Nervous System ... Address for correspondence: Paul Brown, Building 36, Room 4A-05, ...
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." ........TSS
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
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
TUESDAY, JANUARY 17, 2017
FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEEDVIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE 2016 to 2017 BSE TSE PRION
Thursday, November 16, 2017
Texas Natural Meats Recalls Beef Products Due To Possible Specified Risk Materials Contamination
PAGE NOT FOUND
IF you will notice, archived information has now been deleted before 2008. please be aware, 2006 was a banner year for tons and tons of banned mad cow protein fed out into commerce. i have some archived, but not all. the mad cow feed ban by the FDA et al was and is nothing but ink on paper...terry
TUESDAY, JANUARY 17, 2017
FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEEDVIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE 2016 to 2017 BSE TSE PRION
FRIDAY, NOVEMBER 3, 2017
BSE MAD COW TSE PRION DISEASE PET FOOD FEED IN COMMERCE INDUSTRY VS TERRY S. SINGELTARY Sr. A REVIEW
''I have a neighbor who is a dairy farmer. He tells me that he knows of several farmers who feed their cattle expired dog food. These farmers are unaware of any dangers posed to their cattle from the pet food contents. For these farmers, the pet food is just another source of protein.''
IN CONFIDENCE
2017
FRIDAY, DECEMBER 22, 2017
Detection of PrPBSE and prion infectivity in the ileal Peyer’s patch of young calves as early as 2 months after oral challenge with classical bovine spongiform encephalopathy
WEDNESDAY, OCTOBER 4, 2017
EFSA Scientific Report on the Assessment of the Geographical BSE-Risk (GBR) of the United States of America (USA) a review 2017
TUESDAY, JULY 18, 2017
***> USDA announces Alabama case of Bovine Spongiform Encephalopathy Alabama
THURSDAY, JULY 20, 2017
***> USDA OIE Alabama Atypical L-type BASE Bovine Spongiform Encephalopathy BSE animal feeds for ruminants rule, 21 CFR 589..200
SUNDAY, JULY 23, 2017
***> atypical L-type BASE Bovine Amyloidotic Spongiform Encephalopathy BSE TSE PRION
SUNDAY, JULY 23, 2017
***> Experimental Infection of Cattle With a Novel Prion Derived From Atypical H-Type Bovine Spongiform Encephalopathy
WEDNESDAY, OCTOBER 4, 2017
*** EFSA Scientific Report on the Assessment of the Geographical BSE-Risk (GBR) of the United States of America (USA) a review 2017 ***
TUESDAY, AUGUST 8, 2017
Concurrence With OIE Risk Designations for Bovine Spongiform Encephalopathy [Docket No. APHIS-2016-0092]
SUNDAY, JUNE 3, 2018
Clinical, pathological, and molecular features of classical and L-type atypical-BSE in goats
Wednesday, December 21, 2016
TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES 2016 ANNUAL REPORT ARS RESEARCH
Tuesday, September 06, 2016
A comparison of classical and H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism in wild type and EK211 cattle following intracranial inoculation
Saturday, July 23, 2016
BOVINE SPONGIFORM ENCEPHALOPATHY BSE TSE PRION SURVEILLANCE, TESTING, AND SRM REMOVAL UNITED STATE OF AMERICA UPDATE JULY 2016
Tuesday, July 26, 2016
Atypical Bovine Spongiform Encephalopathy BSE TSE Prion UPDATE JULY 2016
Monday, June 20, 2016
Specified Risk Materials SRMs BSE TSE Prion Program
Thursday, December 08, 2016
USDA APHIS National Scrapie Eradication Program October 2016 Monthly Report Fiscal Year 2017 atypical NOR-98 Scrapie
UESDAY, JULY 18, 2017
MINK FARMING USA TRANSMISSIBLE MINK ENCEPHALOPATHY TSE PRION DISEASE SURVEILLANCE AND TESTING
WEDNESDAY, MARCH 21, 2018
World Animal Organization (OIE) Appoints Veterinary Institute as first European reference laboratory for land animal health field of CWD or skrantesjuke scratch disease
WEDNESDAY, JUNE 13, 2018
National Scrapie Eradication Program April 2018 Monthly Report Fiscal Year 2018
I urge everyone to watch this video closely...terry
*** you can see video here and interview with Jeff's Mom, and scientist telling you to test everything and potential risk factors for humans ***
*** Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery ***
Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC.
Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892. Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8006664&dopt=Abstract
Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC.
Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892. Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8006664&dopt=Abstract
Morbidity and Mortality Weekly Report
274 MMWR / March 9, 2018 / Vol. 67 / No. 9 US Department of Health and Human Services/Centers for Disease Control and Prevention
Update: Dura Mater Graft–Associated Creutzfeldt-Jakob Disease — Japan, 1975–2017
Ryusuke Ae, MD, PhD1; Tsuyoshi Hamaguchi, MD, PhD2; Yosikazu Nakamura, MD1; Masahito Yamada, MD, PhD2; Tadashi Tsukamoto, MD, PhD3; Hidehiro Mizusawa, MD, PhD3; Ermias D. Belay, MD4; Lawrence B. Schonberger, MD4
The cases described in this report indicate that recipients of prion-contaminated grafts could remain at risk for CJD for at least 30 years after receiving grafts. Given the known potential for even longer latency periods for prion diseases, this outbreak is expected to continue. The dCJD cases underscore the importance of establishing measures to eliminate or greatly reduce the possibility of CJD transmissions (e.g., strict donor screening, appropriate record keeping, prevention of crosscontaminations, and ideally, the use of validated sterilization methods) whenever human tissues, particularly of cadaveric origin, might be used to treat other patients. In addition, a system of human disease surveillance to detect the possible emergence of new sources of prion disease transmissions is needed. Furthermore, physicians maintaining a high index of suspicion for unusual prion disease cases, as well as a system of human disease surveillance to detect the emergence of new sources of prion disease transmissions, is needed to enable the prevention of infections Finally, maintaining surveillance for CJD in Japan is important to better assess the impact of the outbreak of dCJD and to identify additional cases.
SUNDAY, MARCH 11, 2018
Dura Mater Graft–Associated Creutzfeldt-Jakob Disease — Japan, 1975–2017 Update
Re-Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy
>>> The only tenable public line will be that "more research is required’’ <<<
>>> possibility on a transmissible prion remains open<<<
O.K., so it’s about 23 years later, so somebody please tell me, when is "more research is required’’ enough time for evaluation ?
Re-Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy
Nature 525, 247?250 (10 September 2015) doi:10.1038/nature15369 Received 26 April 2015 Accepted 14 August 2015 Published online 09 September 2015 Updated online 11 September 2015 Erratum (October, 2015)
snip...see full Singeltary Nature comment here;
Alzheimer's disease
let's not forget the elephant in the room. curing Alzheimer's would be a great and wonderful thing, but for starters, why not start with the obvious, lets prove the cause or causes, and then start to stop that. think iatrogenic, friendly fire, or the pass it forward mode of transmission. think medical, surgical, dental, tissue, blood, related transmission. think transmissible spongiform encephalopathy aka tse prion disease aka mad cow type disease...
Commentary: Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy
Self-Propagative Replication of Ab Oligomers Suggests Potential Transmissibility in Alzheimer Disease
*** Singeltary comment PLoS ***
Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ?
Posted by flounder on 05 Nov 2014 at 21:27 GMT
IN CONFIDENCE
5 NOVEMBER 1992
TRANSMISSION OF ALZHEIMER TYPE PLAQUES TO PRIMATES
[9. Whilst this matter is not at the moment directly concerned with the iatrogenic CJD cases from hgH, there remains a possibility of litigation here, and this presents an added complication.
There are also results to be made available shortly
(1) concerning a farmer with CJD who had BSE animals,
(2) on the possible transmissibility of Alzheimer’s and
(3) a CMO letter on prevention of iatrogenic CJD transmission in neurosurgery, all of which will serve to increase media interest.]
snip...see full Singeltary Nature comment here;
re-Evidence for human transmission of amyloid-? pathology and cerebral amyloid angiopathy Nature 525, 247?250 (10 September 2015) doi:10.1038/nature15369 Received 26 April 2015 Accepted 14 August 2015 Published online 09 September 2015 Updated online 11 September 2015 Erratum (October, 2015)
I would kindly like to comment on the Nature Paper, the Lancet reply, and the newspaper articles.
First, I applaud Nature, the Scientist and Authors of the Nature paper, for bringing this important finding to the attention of the public domain, and the media for printing said findings.
Secondly, it seems once again, politics is getting in the way possibly of more important Transmissible Spongiform Encephalopathy TSE Prion scientific findings. findings that could have great implications for human health, and great implications for the medical surgical arena. but apparently, the government peer review process, of the peer review science, tries to intervene again to water down said disturbing findings.
where have we all heard this before? it's been well documented via the BSE Inquiry. have they not learned a lesson from the last time?
we have seen this time and time again in England (and other Country's) with the BSE mad cow TSE Prion debacle.
That 'anonymous' Lancet editorial was disgraceful. The editor, Dick Horton is not a scientist.
The pituitary cadavers were very likely elderly and among them some were on their way to CJD or Alzheimer's. Not a bit unusual. Then the recipients, who got pooled extracts injected from thousands of cadavers, were 100% certain to have been injected with both seeds. No surprise that they got both diseases going after thirty year incubations.
That the UK has a "system in place to assist science journalists" to squash embargoed science reports they find 'alarming' is pathetic.
Sounds like the journalists had it right in the first place: 'Alzheimer's may be a transmissible infection' in The Independent to 'You can catch Alzheimer's' in The Daily Mirror or 'Alzheimer's bombshell' in The Daily Express
if not for the journalist, the layperson would not know about these important findings.
where would we be today with sound science, from where we were 30 years ago, if not for the cloak of secrecy and save the industry at all cost mentality?
when you have a peer review system for science, from which a government constantly circumvents, then you have a problem with science, and humans die.
to date, as far as documented body bag count, with all TSE prion named to date, that count is still relatively low (one was too many in my case, Mom hvCJD), however that changes drastically once the TSE Prion link is made with Alzheimer's, the price of poker goes up drastically.
so, who makes that final decision, and how many more decades do we have to wait?
the iatrogenic mode of transmission of TSE prion, the many routes there from, load factor, threshold from said load factor to sub-clinical disease, to clinical disease, to death, much time is there to spread a TSE Prion to anywhere, but whom, by whom, and when, do we make that final decision to do something about it globally? how many documented body bags does it take? how many more decades do we wait? how many names can we make up for one disease, TSE prion?
Professor Collinge et al, and others, have had troubles in the past with the Government meddling in scientific findings, that might in some way involve industry, never mind human and or animal health.
FOR any government to continue to circumvent science for monetary gain, fear factor, or any reason, shame, shame on you.
in my opinion, it's one of the reasons we are at where we are at to date, with regards to the TSE Prion disease science i.e. money, industry, politics, then comes science, in that order.
greed, corporate, lobbyist there from, and government, must be removed from the peer review process of sound science, it's bad enough having them in the pharmaceutical aspect of healthcare policy making, in my opinion.
my mother died from confirmed hvCJD, and her brother (my uncle) Alzheimer's of some type (no autopsy?). just made a promise, never forget, and never let them forget, before I do.
I kindly wish to remind the public of the past, and a possible future we all hopes never happens again. ...
2012
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 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 (most of the time) than the TSE prion disease. Symptoms are very similar, and pathology is very similar.
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
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. 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.
Conclusions
There should be a Global Congressional Science round table event 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 lets 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.
end...tss
Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ?
source references ...end...tss
Hello Nicole,
by all means, please do use my poster. but I thought this was already taken care of, and I could not attend for my poster presentation, therefore, it was not going to be presented. I have some health issues and could not make the trip.
please see old correspondence below...
From: Nicole Sanders Sent: Tuesday, April 10, 2012 5:37 PM To: Terry S. Singeltary Sr. Subject: RE: re-submission
Dear Terry,
The decline of proposal number 30756 is registered in the system. Thank you for your consideration.
Best Regards,
Nicole
Nicole Sanders
Senior Specialist, Membership & Conference Programming
______________________________________
From: xxxx
To: Terry Singeltary
Sent: Saturday, December 05, 2009 9:09 AM
Subject: 14th ICID - abstract accepted for 'International Scientific Exchange'
Your preliminary abstract number: 670
Dear Mr. Singeltary,
On behalf of the Scientific Committee, I am pleased to inform you that your abstract
'Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009'
WAS accepted for inclusion in the INTERNATIONAL SCIENTIFIC EXCHANGE (ISE) section of the 14th International Congress on Infectious Diseases. Accordingly, your abstract will be included in the "Intl. Scientific Exchange abstract CD-rom" of the Congress which will be distributed to all participants.
Abstracts accepted for INTERNATIONAL SCIENTIFIC EXCHANGE are NOT PRESENTED in the oral OR poster sessions.
Your abstract below was accepted for: INTERNATIONAL SCIENTIFIC EXCHANGE
#0670: Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009
Author: T. Singeltary; Bacliff, TX/US
Topic: Emerging Infectious Diseases Preferred type of presentation: International Scientific Exchange
This abstract has been ACCEPTED.
#0670: Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009
Authors: T. Singeltary; Bacliff, TX/US
Title: Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009
Body: Background
An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.
Methods
12 years independent research of available data
Results
I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.
Conclusion
I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries.
I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.
Keywords: Transmissible Spongiform Encephalopathy Creutzfeldt Jakob Disease Prion
page 114 ;
http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf
http://www.isid.org/14th_icid/
http://www.isid.org/publications/ICID_Archive.shtml
http://ww2.isid.org/Downloads/IMED2009_AbstrAuth.pdf
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 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 (most of the time) than the TSE prion disease. Symptoms are very similar, and pathology is very similar.
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
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. 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.
Conclusions
There should be a Global Congressional Science round table event 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 lets 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.
end...tss
Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ?
source references ...end...tss
Hello Nicole,
by all means, please do use my poster. but I thought this was already taken care of, and I could not attend for my poster presentation, therefore, it was not going to be presented. I have some health issues and could not make the trip.
please see old correspondence below...
From: Nicole Sanders Sent: Tuesday, April 10, 2012 5:37 PM To: Terry S. Singeltary Sr. Subject: RE: re-submission
Dear Terry,
The decline of proposal number 30756 is registered in the system. Thank you for your consideration.
Best Regards,
Nicole
Nicole Sanders
Senior Specialist, Membership & Conference Programming
______________________________________
From: xxxx
To: Terry Singeltary
Sent: Saturday, December 05, 2009 9:09 AM
Subject: 14th ICID - abstract accepted for 'International Scientific Exchange'
Your preliminary abstract number: 670
Dear Mr. Singeltary,
On behalf of the Scientific Committee, I am pleased to inform you that your abstract
'Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009'
WAS accepted for inclusion in the INTERNATIONAL SCIENTIFIC EXCHANGE (ISE) section of the 14th International Congress on Infectious Diseases. Accordingly, your abstract will be included in the "Intl. Scientific Exchange abstract CD-rom" of the Congress which will be distributed to all participants.
Abstracts accepted for INTERNATIONAL SCIENTIFIC EXCHANGE are NOT PRESENTED in the oral OR poster sessions.
Your abstract below was accepted for: INTERNATIONAL SCIENTIFIC EXCHANGE
#0670: Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009
Author: T. Singeltary; Bacliff, TX/US
Topic: Emerging Infectious Diseases Preferred type of presentation: International Scientific Exchange
This abstract has been ACCEPTED.
#0670: Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009
Authors: T. Singeltary; Bacliff, TX/US
Title: Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009
Body: Background
An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.
Methods
12 years independent research of available data
Results
I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.
Conclusion
I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries.
I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.
Keywords: Transmissible Spongiform Encephalopathy Creutzfeldt Jakob Disease Prion
page 114 ;
http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf
http://www.isid.org/14th_icid/
http://www.isid.org/publications/ICID_Archive.shtml
http://ww2.isid.org/Downloads/IMED2009_AbstrAuth.pdf
BMJ Case Reports 2017; doi:10.1136/bcr-2017-220907
Rare disease
CASE REPORT
Gerstmann-Sträussler-Scheinker disease with atypical presentation
Sarah E Keuss1, James W Ironside2, Jonathan O’Riordan1
+ Author Affiliations
1Department of Neurology, Ninewells Hospital, Dundee, Tayside, UK
2Department of Clinical Brain Sciences, National Creutzfeldt-Jakob Disease Research and Surveillance Unit, Edinburgh, UK
Correspondence to Dr Jonathan O’Riordan, joriordan@nhs.net
Accepted 6 September 2017
Published 1 November 2017
Summary
We describe a 37-year-old woman who presented with progressive deafness, visual loss and ataxia. She latterly developed neuropsychiatric problems, including cognitive impairment, paranoid delusions and episodes of altered consciousness. She was found to be heterozygous for the Q212P mutation in the prion protein gene. She died over a decade after initial presentation and a diagnosis of prion disease was confirmed at postmortem.
Gerstmann-Sträussler-Scheinker disease subtypes efficiently transmit in bank voles as genuine prion diseases
Published online: 04 February 2016
Tuesday, November 29, 2016
Transmissibility of Gerstmann–Sträussler–Scheinker syndrome in rodent models: new insights into the molecular underpinnings of prion infectivity
2015 PRION CONFERENCE
*** RE-P.164: Blood transmission of prion infectivity in the squirrel monkey: The Baxter study
***suggest that blood donations from cases of GSS (and perhaps other familial forms of TSE) carry more risk than from vCJD cases, and that little or no risk is associated with sCJD. ***
P.164: Blood transmission of prion infectivity in the squirrel monkey: The Baxter study
Paul Brown1, Diane Ritchie2, James Ironside2, Christian Abee3, Thomas Kreil4, and Susan Gibson5 1NIH (retired); Bethesda, MD USA; 2University of Edinburgh; Edinburgh, UK; 3University of Texas; Bastrop, TX USA; 4Baxter Bioscience; Vienna, Austria; 5University of South Alabama; Mobile, AL USA
Five vCJD disease transmissions and an estimated 1 in 2000 ‘silent’ infections in UK residents emphasize the continued need for information about disease risk in humans. A large study of blood component infectivity in a non-human primate model has now been completed and analyzed. Among 1 GSS, 4 sCJD, and 3 vCJD cases, only GSS leukocytes transmitted disease within a 5–6 year surveillance period. A transmission study in recipients of multiple whole blood transfusions during the incubation and clinical stages of sCJD and vCJD in ic-infected donor animals was uniformly negative. These results, together with other laboratory studies in rodents and nonhuman primates and epidemiological observations in humans, suggest that blood donations from cases of GSS (and perhaps other familial forms of TSE) carry more risk than from vCJD cases, and that little or no risk is associated with sCJD. The issue of decades-long incubation periods in ‘silent’ vCJD carriers remains open.
ran across an old paper from 1984 ;
***The occurrence of contact cases raises the possibility that transmission in families may be effected by an unusually virulent strain of the agent. ***
snip...see full text ;
Variably protease-sensitive prionopathy (VPSPr), a recently identified and seemingly sporadic human prion disease, is distinct from Creutzfeldt-Jakob disease (CJD) but shares features of Gerstmann-Sträussler-Scheinker disease (GSS). However, contrary to exclusively inherited GSS, no prion protein (PrP) gene variations have been detected in VPSPr, suggesting that VPSPr might be the long-sought sporadic form of GSS. snip...
In conclusion, we propose that VPSPr is transmissible and, therefore, is an authentic prion disease. However, transmissibility cannot be sustained through serial passages presumably because human PrPC (or the mouse brain environment) cannot efficiently convert and propagate the VPSPr PrPSc species. If this is the case, uncovering the properties of human PrP that are required to replicate more efficiently the prion strains associated with VPSPr may help clarify the PrPSc mode of formation in this intriguing disease.
Friday, January 10, 2014
vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type prion disease, what it ???
WEDNESDAY, NOVEMBER 09, 2011
Case report Sporadic fatal insomnia in a young woman: A diagnostic challenge: Case Report TEXAS
HOW TO TURN A POTENTIAL MAD COW VICTIM IN THE USA, INTO A HAPPENSTANCE OF BAD LUCK, A SPONTANEOUS MUTATION FROM NOTHING.
OR WAS IT $$$
FRIDAY, NOVEMBER 3, 2017
GSS Gerstmann-Sträussler-Scheinker disease with atypical presentation
Sunday, June 17, 2018
Reviews Prion-like Propagation of α-synuclein, Parkinson, and tse prion
Terry S. Singeltary Sr.
posted by Terry S. Singeltary Sr. | 7:57 PM
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