CREUTZFELDT JAKOB DISEASE

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

From: TERRY SINGELTARY
To: TERRY SINGELTARY
Sent: Friday, August 08, 2008 5:30 PM
Subject: Biosafety in Microbiological and Biomedical Laboratories Fifth Edition 2007

Biosafety in Microbiological and Biomedical Laboratories*

U.S. Department of Health and Human Services Public Health Service

Centers for Disease Control and Prevention and National Institutes of Health

Fifth Edition 2007

U. S. Government Printing Office Washington: 2007

Section VIII-H: Prion Diseases

Transmissible spongiform encephalopathies (TSE) or prion diseases are neurodegenerative diseases which affect humans and a variety of domestic and wild animal species (Tables 1 and 2).1,2 A central biochemical feature of prion diseases is the conversion of normal prion protein (PrP) to an abnormal, misfolded, pathogenic isoform designated PrPSc (named for “scrapie,” the prototypic prion disease). The infectious agents that transmit prion diseases are resistant to inactivation by heat and chemicals and thus require special biosafety precautions. Prion diseases are transmissible by inoculation or ingestion of infected tissues or homogenates, and infectivity is present at high levels in brain or other central nervous system tissues, and at slightly lower levels in lymphoid tissues including spleen, lymph nodes, gut, bone marrow, and blood. Although the biochemical nature of the infectious TSE agent, or prion, is not yet proven, the infectivity is strongly associated with the presence of PrPSc, suggesting that this material may be a major component of the infectious agent.

A chromosomal gene encodes PrPC (the cellular isoform of PrP) and no PrP genes are found in purified preparations of prions. PrPSc is derived from PrPC by a posttranslational process whereby PrPSc acquires a high beta-sheet content and a resistance to inactivation by normal disinfection processes. The PrPSc is less soluble in aqueous buffers and, when incubated with protease (proteinase K), the PrPC is completely digested (sometimes indicated by the “sensitive” superscript, PrPsen) while PrPSc is resistant to protease (PrPres). Neither PrP-specific nucleic acids nor virus-like particles have been detected in purified, infectious preparations.

OCCUPATIONAL INFECTIONS

No occupational infections have been recorded from working with prions. No increased incidence of Creutzfeldt-Jakob disease (CJD) has been found amongst pathologists who encounter cases of the disease post-mortem.

NATURAL MODES OF INFECTION

The recognized diseases caused by prions are listed under Table 1 (human diseases) and Table 2 (animal diseases). The only clear risk-factor for disease transmission is the consumption of infected tissues such as human brain in the case of kuru, and meat including nervous tissue in the case of bovine spongiform encephalopathy and related diseases such as feline spongiform encephalopathy. It is also possible to acquire certain diseases such as familial CJD by inheritance through the germline.

Agent Summary Statements – Prion Diseases

TABLE 1

THE HUMAN PRION DISEASES

DISEASE ABBREVIATION MECHANISM OF PATHOGENESIS

Kuru Infection through ritualistic cannibalism Creutzfeldt-Jakob disease CJD Unknown mechanism Sporadic CJD sCJD Unknown mechanism; possibly somatic mutation or spontaneous conversion of PrPC to PrPSc Variant CJD vCJD Infection presumably from consumption of BSE-contaminated cattle products and secondary bloodborne transmission Familial CJD fCJD Germline mutations in PrP gene Iatrogenic CJD iCJD Infection from contaminated corneal and dural grafts, pituitary hormone, or neurosurgical equipment Gerstmann-Sträussler- Scheinker syndrome GSS Germline mutations in PrP gene Fatal familial insomnia FFI Germline mutations in PrP gene TABLE 2 THE ANIMAL PRION DISEASES DISEASE ABBREVIATION NATURAL HOST MECHANISM OF PATHOGENESIS Scrapie Sheep, goats and mouflon Infection in genetically susceptible sheep Bovine spongiform encephalopathy BSE Cattle Infection with prion-contaminated feedstuffs Chronic wasting disease CWD Mule deer, white-tailed deer and Rocky Mountain elk Unknown mechanism; possibly from direct animal contact or indirectly from contaminated feed and water sources Exotic ungulate encephalopathy EUE Nyala, greater kudu and oryx Infection with BSE-contaminated feedstuffs Feline spongiform encephalopathy FSE Domestic and wild cats in captivity Infection with BSE-contaminated feedstuffs Transmissible mink encephalopathy TME Mink (farm raised) Infection with prion-contaminated feedstuffs

Agent Summary Statements – Prion Diseases

Species-specificity of prions. Most TSE agents, or prions, have a preference for infection of the homologous species, but cross-species infection with a reduced efficiency is also possible. After cross-species infection there is often a gradual adaptation of specificity for the new host; however, infectivity for the original host may also be propagated for several passages over a time-span of years. The process of cross-species adaptation can also vary among individuals in the same species and the rate of adaptation and the final species specificity is difficulty to predict with accuracy. Such considerations help to form the basis for the biosafety classification of different prions.

LABORATORY SAFETY

Biosafety level classification In the laboratory setting prions from human tissue and human prions propagated in animals should be manipulated at BSL-2. BSE prions can likewise be manipulated at BSL-2. Due to the high probability that BSE prions have been transmitted to humans, certain circumstances may require the use of BSL-3 facilities. All other animal prions are considered BSL-2 pathogens. However, when a prion from one species is inoculated into another the resultant infected animal should be treated according to the guidelines applying to the source of the inoculum. Contact APHIS National Center for Import and Export at (301) 734- 5960 for specific guidance.

Although the exact mechanism of spread of scrapie among sheep and goats developing natural scrapie is unknown, there is considerable evidence that one of the primary sources is oral inoculation with placental membranes from infected ewes. There has been no evidence for transmission of scrapie to humans, even though the disease was recognized in sheep for over 200 years. The diseases TME, BSE, FSE, and EUE are all thought to occur after the consumption of prion-infected foods.1,2 The exact mechanism of CWD spread among mule deer, white-tailed deer and Rocky Mountain elk is unknown. There is strong evidence that CWD is laterally transmitted and environmental contamination may play an important role in local maintenance of the disease.2

Human prion diseases In the care of patients diagnosed with human prion disease, Standard Precautions are adequate. However, the human prion diseases in this setting are not communicable or contagious.3 There is no evidence of contact or aerosol transmission of prions from one human to another. However, they are infectious under some circumstances, such as ritualistic cannibalism in New Guinea causing kuru, the administration of prion-contaminated growth hormone causing iatrogenic CJD, and the transplantation of prion-contaminated dura mater and corneal grafts. It is highly suspected that variant CJD can also be transmitted by blood transfusion.4 However, there is no evidence for bloodborne transmission of non-variant forms of CJD. Familial CJD, GSS, and FFI are all dominantly inherited prion diseases; many different mutations of the PrP gene have been shown to be genetically linked to the development of inherited prion disease. Prions from many cases of inherited prion disease have been transmitted to apes, monkeys, and mice, especially those carrying human PrP transgenes.

SPECIAL ISSUES

Inactivation of prions Prions are characterized by resistance to conventional inactivation

Agent Summary Statements – Prion Diseases

procedures including irradiation, boiling, dry heat, and chemicals (formalin, betapropiolactone, alcohols). While prion infectivity in purified samples is diminished by prolonged digestion with proteases, results from boiling in sodium dodecyl sulfate and urea are variable. Likewise, denaturing organic solvents such as phenol or chaotropic reagents such as guanidine isothiocyanate have also resulted in greatly reduced but not complete inactivation. The use of conventional autoclaves as the sole treatment has not resulted in complete inactivation of prions.5 Formalin-fixed and paraffin-embedded tissues, especially of the brain, remain infectious. Some investigators recommend that formalin-fixed tissues from suspected cases of prion disease be immersed for 30 min in 96% formic acid or phenol before histopathologic processing (Table 3), but such treatment may severely distort the microscopic neuropathology.

The safest and most unambiguous method for ensuring that there is no risk of residual infectivity on contaminated instruments and other materials is to discard and destroy them by incineration.6 Current recommendations for inactivation of prions on instruments and other materials are based on the use of sodium hypochlorite, NaOH, Environ LpH and the moist heat of autoclaving with combinations of heat and chemical being most effective (See Table 4).5,6

Surgical procedures Precautions for surgical procedures on patients diagnosed with prion disease are outlined in an infection control guideline for transmissible spongiform encephalopathies developed by a consultation convened by the WHO in 1999.6 Sterilization of reusable surgical instruments and decontamination of surfaces should be performed in accordance with recommendations described by the CDC (www.cdc.gov) and the WHO infection control guidelines.6 Table 4 summarizes the key recommendations for decontamination of reusable instruments and surfaces. Contaminated disposable instruments or materials should be incinerated.

Autopsies Routine autopsies and the processing of small amounts of formalin-fixed tissues containing human prions can safely be done using BSL-2 precautions.7 The absence of any known effective treatment for prion disease demands caution. The highest concentrations of prions are in the central nervous system and its coverings. Based on animal studies, it is likely that prions are also found in spleen, thymus, lymph nodes, and intestine. The main precaution to be taken by laboratorians working with prion-infected or contaminated material is to avoid accidental puncture of the skin.3 Persons handling contaminated specimens should wear cutresistant gloves if possible. If accidental contamination of unbroken skin occurs, the area should be washed with detergent and abundant quantities of warm water (avoid scrubbing); brief exposure (1 minute to 1N NaOH or a 1:10 dilution of bleach) can be considered for maximum safety.6 Additional guidance related to occupational injury are provided in the WHO infection control guidelines.6 Unfixed samples of brain, spinal cord, and other tissues containing human prions should be processed with extreme care at least in a BSL-2 facility.

Bovine spongiform encephalopathy Although the eventual total number of variant CJD cases resulting from BSE transmission to humans is unknown, a review of the epidemiological data from the United Kingdom indicates that BSE transmission to humans is not efficient.8 The most prudent approach is to study BSE prions at a minimum in a BSL-2 facility. When performing necropsies on large animals where there is an opportunity that the worker may be accidentally splashed or have contact with high-risk materials (e.g., spinal column, brain, etc.) personnel

Agent Summary Statements – Prion Diseases

should wear full body coverage personal protective equipment (e.g., gloves, rear closing gown and face shield). Disposable plasticware, which can be discarded as a dry regulated medical waste, is highly recommended. Because the paraformaldehyde vaporization procedure does not diminish prion titers, BSCs must be decontaminated with 1N NaOH and rinsed with water. HEPA filters should be bagged out and incinerated. Although there is no evidence to suggest that aerosol transmission occurs in the natural disease, it is prudent to avoid the generation of aerosols or droplets during the manipulation of tissues or fluids and during the necropsy of experimental animals. It is further strongly recommended that impervious gloves be worn for activities that provide the opportunity for skin contact with infectious tissues and fluids. Animal carcasses and other tissue waste can be disposed by incineration with a minimum secondary temperature of 1000oC (1832oF).6 Pathological incinerators should maintain a primary chamber temperature in compliance with design and applicable state regulations, and employ good combustion practices. Medical waste incinerators should be in compliance with applicable state and federal regulations.

The alkaline hydrolysis process, using a pressurized vessel that exposes the carcass or tissues to 1 N NaOH or KOH heated to 150oC, can be used as an alternative to incineration for the disposal of carcasses and tissue.5,9 The process has been shown to completely inactive TSEs (301v agent used) when used for the recommended period of time.

TABLE 3

TISSUE PREPARATION FOR HUMAN CJD AND RELATED DISEASES

1. Histology technicians wear gloves, apron, laboratory coat, and face protection. 2. Adequate fixation of small tissue samples (e.g., biopsies) from a patient with suspected prion disease can be followed by post-fixation in 96% absolute formic acid for 30 minutes, followed by 48 hours in fresh 10% formalin. 3. Liquid waste is collected in a 4L waste bottle initially containing 600 ml 6N NaOH. 4. Gloves, embedding molds, and all handling materials are disposed as regulated medical waste. 5. Tissue cassettes are processed manually to prevent contamination of tissue processors. 6. Tissues are embedded in a disposable embedding mold. If used, forceps are decontaminated as in Table 4. 7. In preparing sections, gloves are worn, section waste is collected and disposed in a regulated medical waste receptacle. The knife stage is wiped with 2N NaOH, and the knife used is discarded immediately in a "regulated medical waste sharps" receptacle. Slides are labeled with "CJD Precautions." The sectioned block is sealed with paraffin. 8. Routine staining: a. slides are processed by hand; b. reagents are prepared in 100 ml disposable specimen cups; c. after placing the coverslip on, slides are decontaminated by soaking them for 1 hour in 2N NaOH; d. slides are labeled as "Infectious-CJD." 9. Other suggestions: a.disposable specimen cups or slide mailers may be used for reagents; b. slides for immunocytochemistry may be processed in disposable petri dishes; c. equipment is decontaminated as described above or disposed as regulated medical waste. Agent Summary Statements – Prion Diseases Handling and processing of tissues from patients with suspected prion disease The special characteristics of work with prions require particular attention to the facilities, equipment, policies, and procedures involved.9 The related considerations outlined in Table 3 should be incorporated into the laboratory's risk management for this work. TABLE 4 PRION INACTIVATION METHODS FOR REUSABLE INSTRUMENTS AND SURFACES 1. Immerse in 1 N NaOH, and heat in a gravity displacement autoclave at 121oC for 30 minutes. Clean and sterilize by conventional means. 2. Immerse in 1 N NaOH or sodium hypochlorite (20,000 ppm) for 1 hour. Transfer into water and autoclave (gravity displacement) at 121oC for 1 hour. Clean and sterilize by conventional means. 3. Immerse in 1N NaOH or sodium hypochlorite (20,000 ppm) for 1 hour. Rinse instruments with water, transfer to open pan and autoclave at 121oC (gravity displacement) or 134oC (porous load) for 1 hour. Clean and sterilize by conventional means. 4. Surfaces or heat-sensitive instruments can be treated with 2N NaOH or sodium hypochlorite (20,000 ppm) for 1 hour. Ensure surfaces remain wet for entire time period, then rinse well with water. Before chemical treatment, it is strongly recommended that gross contamination of surfaces be reduced because the presence of excess organic material will reduce the strength of either NaOH or sodium hypochlorite solutions. 5. Environ LpH (EPA Reg. No. 1043-118) may be used on washable, hard, non-porous surfaces (such as floors, tables, equipment, and counters), items (such as non-disposable instruments, sharps, and sharp containers), and/or laboratory waste solutions (such as formalin or other liquids). This product is currently being used under FIFRA Section 18 exemptions in a number of States. Users should consult with the State environmental protection office prior to use. (Adapted from www.cdc.gov, 10,11) Working Solutions 1 N NaOH equals 40 grams of NaOH per liter of water. Solution should be prepared daily. A stock solution of 10 N NaOH can be prepared and fresh 1:10 dilutions (1 part 10 N NaOH plus 9 parts water) used daily. 20,000 ppm sodium hypochlorite equals a 2% solution. Most commercial household bleach contains 5.25% sodium hypochlorite, therefore make a 1:2.5 dilution (1 part 5.25% bleach plus 1.5 parts water) to produce a 20,000 ppm solution. This ratio can also be stated as two parts 5.25% bleach to three parts water. Working solutions should be prepared daily. CAUTION: Above solutions are corrosive and require suitable personal protective equipment and proper secondary containment. These strong corrosive solutions require careful disposal in accordance with local regulations. Precautions in using NaOH or sodium hypochlorite solutions in autoclaves NaOH spills or gas may damage the autoclave if proper containers are not used. The use of containers with a rim and lid designed for condensation to collect and drip back into the pan is recommended. Persons who use this procedure should be cautious in handling hot NaOH solution (post-autoclave) and in avoiding potential exposure to gaseous NaOH, exercise caution during all sterilization steps, and allow the autoclave, instruments, and solutions to cool down before removal. Immersion in sodium hypochlorite bleach can cause severe damage to some instruments. Agent Summary Statements – Prion Diseases REFERENCES 1. Prusiner SB. Prion diseases and the BSE crisis. Science. 1997;278:245-51. 2. Williams ES, Miller MW. Transmissible spongiform encephalopathies in non-domestic animals: origin, transmission and risk factors. Rev Sci Tech Off Int Epiz. 2003;22:145- 56. 3. Ridley RM, Baker HF. Occupational risk of Creutzfeldt-Jakob disease. Lancet. 1993;341:641-2. 4. Llewelyn CA, Hewitt PE, Knight RS, et al. Possible transmission of variant Creutzfeldt- Jakob disease by blood transfusion. Lancet. 2004;7;363:417-21. 5. Taylor DM, Woodgate SL. Rendering practices and inactivation of transmissible spongiform encephalopathy agents. Rev Sci Tech Off Int Epiz. 2003;22:297-310. 6. World Health Organization. [http://www.who.int/en/]. Geneva (Switzerland): The Organization; [updated 2006 Sept 21; cited 2006 Sept 21]. 2000. WHO Infection Control Guidelines for Transmissible Spongiform Encephalopathies. Report of a WHO Consultation, Geneva, Switzerland, 23-26 March 1999. Available from:

http://www.who.int/csr/resources/publications/bse/WHO_CDS_CSR_APH_2000_3/en/

7. Ironside JW and JE Bell. The 'high-risk' neuropathological autopsy in AIDS and Creutzfeldt-Jakob disease: principles and practice. Neuropathol Appl Neurobiol. 1996;22:388-393. 8. Hilton DA. Pathogenesis and prevalence of variant Creutzfeldt-Jakob disease. Pathol J. 2006;208:134-41. 9. Richmond JY, Hill RH, Weyant RS, et al. What’s hot in animal biosafety? ILAR J. 2003;44:20-7. 10. Ernst DR, Race RE. Comparative analysis of scrapie agent inactivation methods. J. Virol. Methods. 1994; 41:193-202. 11. Race RE, Raymond GJ. Inactivation of transmissible spongiform encephalopathy (prion) agents by Environ LpH. J Virol. 2004;78:2164-5.

http://www.cdc.gov/OD/OHS/biosfty/bmbl5/BMBL_5th_Edition.pdf

No occupational infections have been recorded from working with prions ???

I beg to differ. i would say that none has been documented yet, for various reasons.

incubation time period, surveillance just to name a few. how can you document something that is not reportable, and or with an incubation period that can last as long as decades.

i am reminded of our own CJD/TSE surveillance in the USA, and or, the lack of.

USA WRITTEN CJD QUESTIONNAIRE ???

http://cjdquestionnaire.blogspot.com/

vCJD questionnaire

http://www.bseinquiry.gov.uk/report/volume8/pdf/1stquestionnaire.pdf

WHAT are officials so afraid of, as to make cjd reportable of all age groups, and to have a written CJD questionnaire ???

what are they afraid of that they may find $$$

why is it they continue to rely on 'death certificates', when they themselves say it is inaccurate $$$

same with surveillance of mad cow diseae in the USA $$$ and i think this is the reason. they dont want to find either one, and or document.

SAD THAT OLDER FOLKS SEEM TO BE EXPENDABLE IN RELATIONS TO TSEs.IF YOUR NOT YOUNG, IF IT's NOT THE UKBSENVCJD ONLY STRAIN, OR IF YOU NOT A DOG OR CAT, YOUR OUTA LUCK. YOUR SPORADIC, YOUR SPONTANEOUS, in short, your expendable, you are an acceptable death. it's a corporate decission. ...tss

One reason for this was the _inaccuracy_ in coding of cases correctly certified as CJD Coding is carried out by staff who are not medically qualified and it is not surprising that coding errors occur in the processing of large numbers of certificates. In 1982, 12,000 certificates per week were processed at the office of population censuses and surveys bu 15 coders and 6 checkers (Alderson et al., 1983). The occurrence of both inter- and intra-observer coding errors has been described (Curb et al., 1983) and the _inaccuracies_ of BOTH certification and coding discovered in this study _support_ the introduction of a more accurate system of death certificates and a more detailed and specific coding system...

snip...

http://www.bseinquiry.gov.uk/files/mb/m26/tab01.pdf

ii. Reliance on death certificates was no help, as ‘CJD is written on death certificates in quite a reckless way!’

http://www.bseinquiry.gov.uk/files/yb/1990/02/00006001.pdf

The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.http://www.cjdfoundation.org/fact.html

POLICY IN CONFIDENCE; CONFIDENTIAL; CJD IN FARMER WITH BSE COW ie OCCUPATIONAL EXPOSURE

Subject: POLICY IN CONFIDENCE: CJD IN FARMER WITH BSE COW

POLICY IN CONFIDENCE: CJD IN FARMER WITH BSE COW

LIKELY TO ATRACT MEDIA ATTENTION

http://www.bseinquiry.gov.uk/files/yb/1992/08/13002001.pdf

http://www.bseinquiry.gov.uk/files/yb/1992/08/21002001.pdf

http://www.bseinquiry.gov.uk/files/yb/1992/08/21005001.pdf

CONFIRMED CJD IN FARMER WITH BSE COW

line to take, sporadic CJD

http://www.bseinquiry.gov.uk/files/yb/1992/10/22004001.pdf

http://www.bseinquiry.gov.uk/files/yb/1992/10/22005001.pdf

http://www.bseinquiry.gov.uk/files/yb/1992/10/22001001.pdf

http://www.bseinquiry.gov.uk/files/yb/1992/11/05002001.pdf

http://www.bseinquiry.gov.uk/files/yb/1992/11/05003001.pdf

SECOND CASE CJD IN DAIRY FARMER

http://www.bseinquiry.gov.uk/files/yb/1993/00/00001001.pdf

CJD IN AN INDIVIDUAL OCCUPATIONALLY EXPOSED TO BSE

ii. on page 2 the sentence ''He had drunk pooled milk from the herd which included that from the affected animal'' will mislead the uninformed. It needs to be made clear that milk from a cow which is suspected to be affected with BSE cannot be drunk or added to the bulk milk produced by the rest of the herd.

iii. in the final paragraph I suggest that the phrase ''and a causal link with BSE is at most conjectural'' BE DELETED: the first paragraph of the sentence would then stand as a clear statement that the CJD case was likely to have been a CHANCE PHENOMENON.

http://www.bseinquiry.gov.uk/files/yb/1993/02/15003001.pdf

''DH is aware of a second case of CJD in a dairy farmer who has had BSE in his herd. We cannot comment on the details of the case, but we know of nothing to suggest this is anthing other than a sporadic case of CJD. .........

http://www.bseinquiry.gov.uk/files/yb/1993/07/12001001.pdf

IF PRESSED:

The numbers concerned are very small, and it is not possible to draw any conclusions from such small numbers. This issue is being considered by the Government's expert advisers....

http://www.bseinquiry.gov.uk/files/yb/1993/07/12002001.pdf

THE FARMER IS THOUGHT TO HAVE HAD AT LEAST TWO CASES OF BSE IN HIS HERD, which were diagnosed in 1992. The farmer is reported to have asssisted in calving and to have drunk milk from his herd. This does not suggest that this is anything other than a sporadic case of CJD. ...

http://www.bseinquiry.gov.uk/files/yb/1993/07/12003001.pdf

CONFIDENTIAL

CONFIRMED CASE OF CJD IN DAIRY FARMER

http://www.bseinquiry.gov.uk/files/yb/1993/07/14003001.pdf

3. Neither Dr Will nor the CJD surveillance unit intend to disclose the existence of this case or make any comment at present unless it attracts media attention.

snip...

HUMAN CASE DETAILS CONFIDENTIAL

snip...

6. CJD IN FARMERS

The second annual report on CJD surveillance in the UK, which is about to be published, gives occupational history details of 29 definite and probable CJD cases recorded in people who had a history of employment at any time in particular occupational groups of potential significance for the occurrence of the disease. The 29 cases were amongst 95 diagnosed over a 3 year period: the other 66 cases did not fall into such occupational groups.

These relevant details are:-

MEDICAL/PARAMEDICAL/DENTISTRY 7

ANIMAL LABORATORY 1

PHARMACEUTICAL LABORATORY 0

RESEARCH LABORATORY 0

FARMERS/VETERINARY SURGEONS 7

BUTCHERS/ABATTOIR WORKERS/OCCUPATION INVOLVING DIRECT CONTACT WITH ANIMAL OR CARCASES 5

OCCUPATION INVOLVING ANIMAL PRODUCTS 9

snip... full text ;

http://www.bseinquiry.gov.uk/files/yb/1993/07/19001001.pdf

Rocky Mountain oysters, mountain oysters, prairie oysters, Montana tendergroin or swinging sirloin

POLICY IN CONFIDENCE

1. The article in the Daily Mail of 12 August again raises the question of a CAUSATIVE LINK BETWEEN BSE AND CJD. This follows the death of a second farmer from CJD...

snip...

I am, however, concerned about how DH and MAFF would respont to public concern generated if there are further CJD cases among farmers.

snip...

4. Unwelcome, though it maybe to the Tyrrell Committee, I think they must be asked at their next meeting to give further thought to what they might advise the Department and MAFF if ANOTHER FARMER (or TWO) DEVELOPS CJD. OR, if a butcher or abattoir worker develops the disease.

5. Although the Committee were given plenty of advance warning about the second farmer, they may NOT BE SO FORTUNATE NEXT TIME ROUND. Some Contingency planning on the Committee's response to a further case of CJD in a farmer seems essential. At the same time the Committee should consider if there is SPECIAL RISK TO FARMERS, FOR EXAMPLE THEIR HISTORICAL HABIT OF CHEWING CATTLE NUTS, that might be implicated. .....(oh my GOD...tss)

http://www.bseinquiry.gov.uk/files/yb/1993/08/12002001.pdf

Ministers will note from this that experts are of the view, that there is unlikely to be a direct link between the cases of BSE, and the occurance of CJD in the farmer.

(NOTE CJD increasing over 3 years. ...TSS)

http://www.bseinquiry.gov.uk/files/yb/1993/08/18004001.pdf

'AGE AT ONSET' is therefore likely to be a reflection of particulary aetiological factors, about which, for sporadic CJD at least, much is yet unknown. IT has therefore been suggested that examination of the f/d i/p of other groups with TSE's, and comparison with that of CJD subsets might help to elucidate aetiological mechanisms for sporadic CJD in particular; i.e. ALMOST A REVERSAL OF THE ORIGINAL UNDERTAKING.

http://www.bseinquiry.gov.uk/files/yb/1993/08/26001001.pdf

OCCUPATIONAL EXPOSURE TO BSE AND CJD

2. The Tyrrell Committee met on 7 October and the significance of the two cases of CJD reported in dairy farmers who had BSE-affected animals on their farms was discussed at some length, AS WERE THE IMPLICATIONS OF A THIRD (OR FORTH) similar case.

3. The Committee were unable to identify any possible risk factors over and above those that they had already considered, both in general and with particular of TASTING THE FEED does continue but there was no consensus about the value of advising farmers to discontinue this practice. Feed currently in use does not pose a risk because of the ruminant-ruminant feed ban.

http://www.bseinquiry.gov.uk/files/yb/1993/10/11001001.pdf

MRC

STRAIN CHARACTERISATION OF THE CREUTZFELDT-JAKOB DISEASE AGENT BY TRANSMISSION TO MICE

In view of the CONCERN that exposue to BSE OR SCRAPIE MAY POSE A RISK TO HUMANS, it is proposed investigate the relationship between sporadic creutzfeldt-jakob disease.....

http://www.bseinquiry.gov.uk/files/yb/1993/10/12001001.pdf

3. While Committee may have no leads to pursue on why farmers might be at increased risk, I hope they understand the urgency with which they will need to respond if or when a THIRD FARMER DEVELOPS CJD.

http://www.bseinquiry.gov.uk/files/yb/1993/10/18001001.pdf

INCREASE IN SPORADIC CJD

http://www.bseinquiry.gov.uk/files/yb/1993/11/11001001.pdf

occupational

http://www.bseinquiry.gov.uk/files/yb/1994/02/16001001.pdf

Dealler gets ''dixie chicked' again ;

http://www.bseinquiry.gov.uk/files/yb/1993/11/22001001.pdf

http://www.bseinquiry.gov.uk/files/yb/1993/12/08003001.pdf

http://www.bseinquiry.gov.uk/files/yb/1993/12/10006001.pdf

http://www.bseinquiry.gov.uk/files/yb/1993/12/14003001.pdf

http://www.bseinquiry.gov.uk/files/yb/1993/12/16006001.pdf

http://www.bseinquiry.gov.uk/files/yb/1993/12/17003001.pdf

STACKING THE DECK AGAINST SPORADIC CJD AND SOUND SCIENCE

APPOINTMENTS IN CONFIDENCE

MEMBERSHIP TO SEAC

snip...

I have informed Dr Tyrrell that we have now written to Dr Hueston to invite him to serve on the Committee and he was very pleased to hear this. He was also in favour of our idea of having a deputy Chairman who could take any emergency meetings eg IF THERE WERE TO BE ANOTHER CJD CASE IN AGRICULTURE. I suggested that either Dr. WIll or Dr Kimberlin were likely candidates and he thought that this was about right. He felt that on balance he would prefer Dr Will who he thought took a more cautious line and was LESS DOGMATIC. .....

http://www.bseinquiry.gov.uk/files/yb/1993/12/01003001.pdf

http://www.bseinquiry.gov.uk/files/yb/1994/01/00005001.pdf

CHANGING SCIENCE TO FIT YOUR INDUSTRY NEEDS COVER-UP IN FULL MODE NOW

PROBLEM

7. The main findings in the case-control study were STATISTICALLY SIGNIFICANT ASSOCIATIONS BETWEEN CONSUMPTION OF VEAL OR VENISON AND THE DEVELOPMENT OF CJD (INCREASED RISKS OF 2-13x).

IP PS(L) wishes to probe this further we think it best to explain the matter VERBALLY. The problem is how to present the findings in this year's annual report in a way which avoids unnecessary public alarm and limits the scope for media scare stores. (or the facts...TSS)

http://www.bseinquiry.gov.uk/files/yb/1994/07/00001001.pdf

A REVISED VERSION WHERE THE FOLLOWING WAS MADE TO BE REMOVED FROM SCIENTIFIC FINDINGS. ...TSS

''This year's findings show a number of associations but the strongest is for veal.''

A BIG LINE WAS DRAWN THROUGH THAT SENTENCE TO BE REMOVED DUE TO THE FOLLOWING. THIS IS THE NEXT SENTENCE ;

''This is of considerable concern given recent developments. In particular, Ministers will be particularly concerned about the European dimension given the recent troubles with the Germans.''

YOU can see the beginning of the ukbsenvCJD only theory beginning to unfold now. full text of this ukbsenvcjd only conspiracy can be seen here. ...TSS

POLICY RESTRICTED

http://www.bseinquiry.gov.uk/files/yb/1994/07/00001001.pdf

BRITISH DEER FARMERS ASSOCIATION

OCTOBER 1994

Dear Mr Elmhirst,

CREUTZFELDT-JAKOB DISEASE (CJD) SURVEILLANCE UNIT REPORT

Thank you for your recent letter concerning the publication of the third annual report from the CJD Surveillance Unit. I am sorry that you are dissatisfied with the way in which this report was published.

The Surveillance Unit is completely independent outside body and the Department of Health is committed to publishing their reports as soon as they become available. In the circumstances it is not the practice to circulate the report for comment since the findings of the report would not be amended. In future we can ensure that the British Deer Farmers Association receives a copy of the report in advance of publication.

snip...

The statistical results regarding the consumption of venison was put into perspective in the body of the report and was NOT MENTIONED AT ALL IN THE PRESS RELEASE. Media attention regarding this report was low key but gave a realistic presentation of the statistical findings of the Unit. This approach to publication was successful in that consumption of VENISON was highlighted only by the media i.e. in the News at one television programme.

I believe that a further statement about the report, or indeed statistical links between CJD and consumption of Venison, would increase, and quite possibly GIVE DAMAGING CREDENCE, to the whole issue. From the low key media reports of which I am aware it seems unlikely that venison consumption will suffer adversely, if at all.

http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf

see buttered and watered down report here that caters to industry instead of human safety...TSS

http://www.bseinquiry.gov.uk/files/yb/1994/10/00004001.pdf

SEE WHERE THIS ;

''This year's findings show a number of associations but the strongest is for veal.''

WENT TO THIS;

In conclusion, an analysis of dietary histories revealed statistical associations between various meats/animal products and increased risk of CJD. When some account was taken of possible confounding, the association between veal eating and risk of CJD emerged as the strongest of these associations statistically. These findings concerning dietary history are particulary difficult to interpret for two reasons.

1. .........BSeee...........TSS

2. .........BSeee...........TSS

(I.E. BSeee = bull sh!t encephalopathy or government cover-up i.e. God save the industry at all cost, including human health. ...TSS)

THUS, the reported veal eating habits of confirmed CJD cases appear virtually identical to suspect cases later judged not to have the disease. This provides good circumstantial evidence to support the hypothesis that the apparent association between veal consumption and CJD is due to recall bias. Analysis of other apparent dietary risk factors for CJD, including venison, has provided similar evidence of recall bias.

snip...

In summary, the analysis of the dietary case-control study demonstrates a strong association between a lifetime history of veal consumption and the risk of developing Creutzfeldt-Jakob disease. HOWEVER this result may well be due to recall bias and analysis of clinical and molecular bilogical features does not provide supportive evidence for the hypothesis that veal eating is a risk factor for CJD. ...

snip...

MORE OF THIS BSeee CAN BE READ IN THE FINAL GOVERNMENT DICTATED CJD REPORT OF 1994

http://www.bseinquiry.gov.uk/files/yb/1994/08/00004001.pdf

BSE SCIENTIST WAS 'CENSORED'

He says that when he worked at MAFF, ''the way it was structurally set up was not that science would drive the politics, but that the politics will drive the science. And that's wrong.''

http://www.bseinquiry.gov.uk/files/yb/1997/12/11001001.pdf

11/3/96 DGRC alerts DTI Ministers and CSA to discovery of nvCJD on basis of letter from MRC Chief Executive dated 11/3/96

BIRTH OF THE UKBSEnvCJD ONLY THEORY, the birth of the 'BIG LIE' begins. ...tss

http://www.bseinquiry.gov.uk/files/db/do01/tab03.pdf

REPORT OF 16 YEAR OLD GIRL WITH CJD

5. This case may raise fears of a link between BSE and CJD. Current advice is that there is no scientific evidence of a link between BSE in cattle and CJD in humans. Furthermore advice from the Spongiform Encephalopathy Advisory Committee is that they are satisified that all necessary safeguards are in place to minimise further spread of spongiform encephalopathies in animals and to prevent any risk of transmission to humans. ...

http://www.bseinquiry.gov.uk/files/yb/1994/01/14005001.pdf

To ask the Secretary of State for Health, how many people under the age of 20 years in each of the last five years have suffered from Creutzfeldt-Jakob disease; and of these how many had not had any growth treatment previously.

SUGGESTED REPLY

We are not aware of any people under the age of 20 in the UK suffering from Creutzfeldt-Jakob Disease in the last five years.

http://www.bseinquiry.gov.uk/files/yb/1994/01/20001001.pdf

STATEMENT FROM HOSPITAL

http://www.bseinquiry.gov.uk/files/yb/1994/01/20005001.pdf

http://www.bseinquiry.gov.uk/files/yb/1994/01/25001001.pdf

PREPARING FOR THE STORM 'LINE TO TAKE'

http://www.bseinquiry.gov.uk/files/yb/1994/01/25003001.pdf

BARONESS CUMBERLEGE TRYING TO MANIPULATE THE MEDIA

http://www.bseinquiry.gov.uk/files/yb/1994/01/25006001.pdf

http://www.bseinquiry.gov.uk/files/yb/1994/01/25006001.pdf

MAD COW MEAL DESTROYED MY DAUGHTERS LIFE

A TEENAGE GIRL may have caught the human form of MAD COW DISEASE by eating a contaminated burger it was claimed last night.

VICKY RIMMER, 16, has the killer Creutzfeldt-Jakob disease (CJD).

http://www.bseinquiry.gov.uk/files/yb/1994/01/25007001.pdf

GIVE ME BACK MY LIFE

http://www.bseinquiry.gov.uk/files/yb/1994/01/25008001.pdf

HUSH UP! GOVERNMENT TOLD GRAN: ''YOU MUST THINK OF THE ECONOMY''

http://www.bseinquiry.gov.uk/files/yb/1994/01/25009001.pdf

WHY IS MY GIRL DYING ? '' IT WAS LIKE SOMEBODY OLD INSIDE A YOUNG PERSON'S BODY

http://www.bseinquiry.gov.uk/files/yb/1994/01/25010001.pdf

I have interviewed Mrs Rimmer at my constituency surgery

IF there is nothing to hide, why is there so much SECRECY? WHY is the Government and other Bodies trying to stop any CHANCE OF PEOPLE CONNECTING THE TWO DISEASES. The B.S.E. problem is obvious, but if the correct measures are taken, surely the problem could be contained, however, as it stands the lack of investigation and interest of the possibility of B.S.E. and C.J.D. being linked is open for speculation and surely someone has to account for peoples lives! WHY is so much trouble being taken to convice people that B.S.E. and C.J.D. are not linked? Guilty Conscience perhaps ? - or cover up?

HOUSE OF COMMONS

FROM BARRY JONES, M.P.

22 FEBRUARY 1994

http://www.bseinquiry.gov.uk/files/yb/1994/02/22009001.pdf

Alleged Case of Creutzfeld Jakob Disease: Victoria Rimmer.

(now story changes that biopsy shows she does not have CJD...tss)

http://www.bseinquiry.gov.uk/files/yb/1994/06/06004001.pdf

now story changes to ;

Advice

7. The Parliamentary Secretary is invited to note the recent statements made on __________ and the present position which remains that CJD cannot be confirmed, in this case at this stage.

http://www.bseinquiry.gov.uk/files/yb/1994/06/08004001.pdf

3. The Medical Director at ___________________ Hospital advised the Department on 6 June that the results of ___________________ brain biopsy had been received and that it showed NO EVIDENCE OF CJD. ______________ Hospital subsequently issued a statement to the press to this effect and this was publicised widely in the press (doc 1). News coverage which followed suggested that the statement made by ________________ Hospital had been misleading (doc 2). Enquires have been made of the Medical Director at _______________ Hospital who has CONFIRMED THAT THE STATEMENT ISSUED BY THE HOSPITAL WAS ISSUED IN ERROR. The facts are that two pathology reports on the same piece of brain tissue were recieved. The first report indicated that CJD was unlikely, The second report indicated that CJD was possible, PERHAPS EVEN LIKELY, but that no definitive diagnosis could be made before a post mortem was undertaken.

http://www.bseinquiry.gov.uk/files/yb/1994/06/08006001.pdf

(ONLY PROBLEM IS, VICKY RIMMER, 16, DID NOT DIE FROM nvCJD, SHE DIED FROM SPORADIC CJD, the same damn thing. ...TSS)

IN light of Asante/Collinge et al findings that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest _sporadic_ CJD;

-------- Original Message -------- Subject: re-BSE prions propagate as

either variant CJD-like or sporadic CJD Date: Thu, 28 Nov 2002 10:23:43

-0000 From: "Asante, Emmanuel A" To: "[log in to unmask]"

Dear Terry,

I have been asked by Professor Collinge to respond to your request. I am

a Senior Scientist in the MRC Prion Unit and the lead author on the

paper. I have attached a pdf copy of the paper for your attention. Thank

you for your interest in the paper.

In respect of your first question, the simple answer is, yes. As you

will find in the paper, we have managed to associate the alternate

phenotype to type 2 PrPSc, the commonest sporadic CJD.

It is too early to be able to claim any further sub-classification in

respect of Heidenhain variant CJD or Vicky Rimmer's version. It will

take further studies, which are on-going, to establish if there are

sub-types to our initial finding which we are now reporting. The main

point of the paper is that, as well as leading to the expected new

variant CJD phenotype, BSE transmission to the 129-methionine genotype

can lead to an alternate phenotype which is indistinguishable from type

2 PrPSc.

I hope reading the paper will enlighten you more on the subject. If I

can be of any further assistance please to not hesitate to ask. Best wishes.

Emmanuel Asante

<> ____________________________________

Dr. Emmanuel A Asante MRC Prion Unit & Neurogenetics Dept. Imperial

College School of Medicine (St. Mary's) Norfolk Place, LONDON W2 1PG

Tel: +44 (0)20 7594 3794 Fax: +44 (0)20 7706 3272 email:

[log in to unmask] (until 9/12/02)

New e-mail: [log in to unmask] (active from now)

____________________________________

snip...

full text ;

http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.htm

WHAT ABOUT U.S.A. ???

CJD YOUNG PEOPLE

in the USA, a 16 year old in 1978;

ALSO IN USA;

(20 year old died from sCJD in USA in 1980 and a 16 year old in 1981. see second url below)

in France, a 19 year old in 1982;

in Canada, a 14 year old of UK origin in 1988;

in Poland, cases in people aged 19, 23, and 27 were identified in a retrospective study (published 1991), having been originally misdiagnosed with a viral encephalitis;

Creutzfeldt's first patient in 1923 was aged 23.

http://www.bseinquiry.gov.uk/files/yb/1995/10/27013001.pdf

20 year old died from sCJD in USA in 1980 and a 16 year old in 1981. A 19 year old died from sCJD in France in 1985. There is no evidence of an iatrogenic cause for those cases....

http://www.bseinquiry.gov.uk/files/yb/1995/10/04004001.pdf

NOW BACK TO THOSE FARMERS WITH BSE HERDS THAT DIED FROM SPORADIC CJD

CJD FARMERS WIFE 1989

http://www.bseinquiry.gov.uk/files/yb/1989/10/13007001.pdf

http://www.bseinquiry.gov.uk/files/yb/1989/10/13003001.pdf

cover-up of 4th farm worker ???

http://www.bseinquiry.gov.uk/files/yb/1995/10/23006001.pdf

http://www.bseinquiry.gov.uk/files/yb/1995/10/20006001.pdf

CONFIRMATION OF CJD IN FOURTH FARMER

http://www.bseinquiry.gov.uk/files/yb/1995/11/03008001.pdf

now story changes from;

SEAC concluded that, if the fourth case were confirmed, it would be worrying, especially as all four farmers with CJD would have had BSE cases on their farms.

to;

This is not unexpected...

was another farmer expected?

http://www.bseinquiry.gov.uk/files/yb/1995/11/13010001.pdf

4th farmer, and 1st teenager

http://www.bseinquiry.gov.uk/files/yb/1996/02/27003001.pdf

2. snip...

Over a 5 year period, which is the time period on which the advice from Professor Smith and Dr. Gore was based, and assuming a population of 120,000 dairy farm workers, and an annual incidence of 1 per million cases of CJD in the general population, a DAIRY FARM WORKER IS 5 TIMES MORE LIKELY THAN an individual in the general population to develop CJD. Using the actual current annual incidence of CJD in the UK of 0.7 per million, this figure becomes 7.5 TIMES.

3. You will recall that the advice provided by Professor Smith in 1993 and by Dr. Gore this month used the sub-population of dairy farm workers who had had a case of BSE on their farms - 63,000, which is approximately half the number of dairy farm workers - as a denominator. If the above sums are repeated using this denominator population, taking an annual incidence in the general population of 1 per million the observed rate in this sub-population is 10 TIMES, and taking an annual incidence of 0.7 per million, IT IS 15 TIMES (THE ''WORST CASE'' SCENARIO) than that in the general population...

http://www.bseinquiry.gov.uk/files/yb/1995/01/31004001.pdf

IN CONFIDENCE

CJD IN FARMERS WIFE

Locally, they made quite an association with BSE, since she was a farmers wife on a farm that, atypically for that area of s Yorkshire, had several BSE cases.

http://www.bseinquiry.gov.uk/files/yb/1989/10/13003001.pdf

A CASE OF CJD - (NOT FROM A BSE FARM)

Dr. R G Will has looked into the case about which I wrote a month ago, and concludes ''any connection between this patient and any bovine condition was tenuous''. No further action is called for.

http://www.bseinquiry.gov.uk/files/yb/1989/11/20011001.pdf

WE concluded that it would be a mistake to involve CDSC, since this would encourage people to believe CJD was somehow directly communicable and would increase the noise in the reporting, just as making CJD notifiable would.

http://www.bseinquiry.gov.uk/files/yb/1989/11/29003001.pdf

http://www.bseinquiry.gov.uk/files/yb/1989/12/01004001.pdf

http://www.bseinquiry.gov.uk/files/yb/1989/12/01003001.pdf

DOES ANYONE BESIDES ME SEE A PATTERN YET ???

Vickey Rimmer, 16, DID NOT DIE FROM nvCJD, she died from a form of sporadic CJD, whatever the hell that is. and there have been 16 year old die from sporadic CJD in the USA as well.

SIMPLY PUT, the ukbsenvcjd only theory was wrong from day one. the elderly are expendable, pets and kids are not.

Science was dictated by 'big buisness' after the Vickey Rimmer case with the ukbsenvcjd only myth.

YOU CAN SEE FULL TEXT ;

Thursday, July 10, 2008 A New Prionopathy OR more of the same old BSe

http://creutzfeldt-jakob-disease.blogspot.com/2008/08/new-prionopathy-or-more-of-same-old-bse.html

CONFIDENTIAL

6. CJD IN FARMERS

The second annual report on CJD surveillance in the UK, which is about to be published, gives occupational history details of 29 definite and probable CJD cases recorded in people who had a history of employment at any time in particular occupational groups of potential significance for the occurrence of the disease. The 29 cases were amongst 95 diagnosed over a 3 year period: the other 66 cases did not fall into such occupational groups.

These relevant details are:-

MEDICAL/PARAMEDICAL/DENTISTRY 7

ANIMAL LABORATORY 1

PHARMACEUTICAL LABORATORY 0

RESEARCH LABORATORY 0

FARMERS/VETERINARY SURGEONS 7

BUTCHERS/ABATTOIR WORKERS/OCCUPATION INVOLVING DIRECT CONTACT WITH ANIMAL OR CARCASES 5

OCCUPATION INVOLVING ANIMAL PRODUCTS 9

snip... full text ;

http://www.bseinquiry.gov.uk/files/yb/1993/07/19001001.pdf

http://bseinquiry.blogspot.com/2008/05/sporadic-cjd-in-farmers-farmers-wives.html

CJD toll among farmers `too high for mere chance' August 15, 1997 PA News John von Radowitz and Andrew Woodcock Microbiologist Richard Lacey, billed in this story as the first to suggest a link between CJD and BSE seven years ago, was cited in this story as saying that the number of cattle farmers falling victim to Creutzfeld-Jakob Disease is much too high to be mere chance, adding that, "Where the CJD Surveillance Unit come unstuck is in trying to explain what happened to these six farmers. This is just too many to have occurred by chance. Unfortunately they don't want to consider the possibility that these farmers in this country and other countries were infected by cattle before BSE developed."

The story notes that professor Lacey believes sporadic CJD itself originates from a cattle infection - possibly a precursor to BSE that has not yet been detected, adding that,

"For years I have suggested that the cause is a rare disease in cattle world wide. Both BSE and the new variant CJD are a new and different disease. What has probably happened is that BSE is a variant of the old type of disease, which could have been missed because it's symptom free. It would explain why such an unusually high number of dairy farmers are being affected by CJD both here and abroad." He also said that cases of sporadic CJD had been recorded as far back as the 1920s. Professor Lacey went on to add that he thought the new variant pattern was alarming, adding, "It's rising, and that is a concern. Unfortunately we can't predict the scale of the problem. If the disease doubled each year up to the year 2020 you'd have hundreds of thousands of cases."

http://www.mad-cow.org/~tom/cousens_gore.html#toll

Prions
Iatrogenic Disease
Source of Contamination N=
n Growth Hormone 139
n Dura Mater Grafts 114
n Neurosurgery 5
n Gonadotropin 4
n Corneal Transplants 3
n Stereotactic EEG 2
Total 267
Brown et al: Neurology 2000; 55:1075

1: J Neurosci Nurs. 1991 Apr;23(2):116-9.

Recommended Biosafety Practices for Handling Prions andPrion-Infected TissuesUpdated May 2007

hazard is from accidental parenteral inoculation or ingestion. Cuts and punctures should be avoided and the use of sharp knives, scalpels, blades and needles should be minimized. If the use of sharps cannot be avoided, cut-resistant gloves should be worn (CFIA 2005). Wherever possible, the laboratory and equipment used for work with prions should be dedicated to that task alone. All employees should be informed and aware that prion research is being conducted in the lab. The entrance to the lab should allow for the separation of PPE/lab clothing and staff clothing. An exposure protocol should be developed, posted and communicated to all employees (CFIA 2005, UCSD 2002). Procedures should be in place for the effective decontamination of all waste, re-usable equipment, surfaces and other lab space (CFIA 2005, UCSD 2002).

http://www.biosafety.msu.edu/current_topic/Prions/working_with_prions.pdf

Precautions prevent spread of Creutzfeldt-Jakob disease. Mocsny N. Veterans Administration Medical Center, Cincinnati, Ohio.

In addition, there are now three cases of laboratory workers with CJD from exposure in the workplace.

http://www.ncbi.nlm.nih.gov/pubmed/1831471?ordinalpos=7&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum

NEXT ;

Q J Med 2000; 93: 617-631 © 2000 Association of Physicians

--------------------------------------------------------------------------------

Commentary papers

Is there evidence for exogenous risk factors in the aetiology and spread of Creutzfeldt-Jakob disease? C. E. M. Hillier and R. L. Salmon From the Welsh Combined Centres for Public Health, University of Wales College of Medicine, Cardiff, UK

snip...

Occupational exposure: observations from case reports and series Case reports and series focus on two main occupational groups; health professionals and those who are exposed to farm animals, in particular sheep and cattle.

There are at least 26 reports of sporadic CJD in health-care workers world-wide.69–71 These include seven physicians, including a neurologist and head of an intensive care unit, two neurosurgeons, an orthopaedic surgeon and a pathologist, three dentists, a dental surgeon, nine nurses, three nursing assistants and two histopathology technicians. Clinical details are available for six of the cases.

Berger70 describes a 58-year-old physician who died of definite CJD and who, 20 years previously, had frequently performed autopsies. Weber71 described a case in a 55-year-old orthopaedic surgeon who died of definite CJD. The clinical picture was suggestive of a peripheral route of infection. Twenty years previously, he had handled both sheep and human dura mater. The specimens of dura mater were later sent to a company that sold dura mater preparations that subsequently transmitted CJD on six occasions. His wife did not remember any definite injury during the time he was working with dura mater. Schoene72 reported a case with an atypical clinical presentation including necrotizing cutaneous lesions with vasculitis in a 54-year-old neurosurgeon, which was later confirmed, as CJD, by experimental transmission. There was no definite history of exposure to any case of CJD. In a case in a pathologist,73 he is known to have performed over 14 000 autopsies, but it is not known if any of them were cases of CJD. There is no information on the dentists or nurses. One of the two histopathology technicians had been a neuropathology technician for 22 years74 and had come into contact with two cases of CJD, 16 and 11 years before the onset of her disease. The other technician75 had been exposed to animal and human brain.

A wide range of occupations have been reported among cases of sporadic CJD.6,21 Several case series quote an excess number of farmers and farmers' wives.24,30 In an Italian study,24 the incidence was three times the expected. An analysis of epidemiological surveillance data in the UK from 1970–9616 revealed a statistically significant excess of cases among dairy farm workers and their spouses and among people with greater degrees of contact with live cattle infected with BSE. No such excess was found in abattoir workers butchers or meat cutters.

snip...

http://qjmed.oxfordjournals.org/cgi/content/full/93/9/617

WHO/CDS/CSR/APH/2000.3 10 WHO Infection Control Guidelines for Transmissible Spongiform Encephalopathies Section 4.

OCCUPATIONAL INJURY 4.1

Occupational exposure Although there have been no confirmed cases of occupational transmission of TSE to humans, cases of CJD in healthcare workers have been reported in which a link to occupational exposure is suggested. Therefore, it is prudent to take a precautionary approach. In the context of occupational exposure, the highest potential risk is from exposure to high infectivity tissues through needle-stick injuries with inoculation; however exposure to either high or low infectivity tissues through direct inoculation (e.g. needle-sticks, puncture wounds, .sharps. injuries, or contamination of broken skin) must be avoided. Exposure by splashing of the mucous membranes (notably the conjunctiva) or unintentional ingestion may be considered a hypothetical risk and must also be avoided. Healthcare personnel who work with patients with confirmed or suspected TSEs, or with their high or low infectivity tissues, should be appropriately informed about the nature of the hazard, relevant safety procedures, and the high level of safety which will be provided by the proposed procedures described throughout this document.'

http://www.who.int/csr/resources/publications/bse/whocdscsraph2003.pdf

PMCID: PMC1769932

Copyright © Copyright 2003 Journal of Clinical Pathology

Health and safety at necropsy

J L Burton Correspondence to: Dr J L Burton, Academic Unit of Pathology, E-Floor Medical School, Beech Hill Road, Sheffield S10 2RX, UK; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000134/!x-usc:mailto:j.l.burton@shef.ac.uk Accepted October 16, 2002.

Transmissible spongiform encephalopathies The risk of acquiring other category 3 risk pathogens, notably the prions responsible for TSE (including v-CJD) is considerably less. However, it should be remembered that the agents responsible for TSE are extremely resilient. They are not “killed” by formalin or phenolised formalin fixation, and are resistant to routine methods of physical and chemical decontamination.3,21,81 Furthermore, v-CJD can be transmitted from archived, formalin fixed, paraffin wax embedded tissues,82 and can survive reduction to ash at 360°C.3 Decontamination requires disinfection with sodium hypochlorite (20 000 parts per million chlorine for at least one hour), 1–2M sodium hydroxide, or steam autoclaving at 134°C for at least 18 minutes.3 Given the prolonged latency of these disorders, evidence of an occupational risk to postmortem room workers remains circumstantial. However, a single case of v-CJD has been reported in a laboratory technician whose work included handling formalin fixed brains.83

http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1769932

http://www.ncbi.nlm.nih.gov/pubmed/3281004

CREUTZFELDT-JAKOB DISEASE (CJD)

The risk of occupational exposure is considered low however reported cases include one neurosurgeon, one pathologist and three pathology technicians.

http://ag.ca.gov/cci/content/bbpsafty.php#cjd

Infection Control Policy for the Management of Patients with, Suspected, or ‘At Risk’ of Having Creutzfeldt-Jakob Disease or Any Transmissible Spongiform Encephalopathy (TSE) (Interim Policy)

http://www.lcrpct.nhs.uk/site/Internet/PoliciesAndProcedures/Clinical/InfectionControlCommunityHospitals/0/CH023%20Patients%20with%20Suspected%20or%20at%20Risk%20of%20Having%20CJD%20Policy%201.pdf

Blackpool Fylde and Wyre Hospitals NHS Trust Revision No: 2 Review Date:01/02/2008 I.D. No: CORP/PROC/031 Title: Healthcare Workers Exposed To Infective Tissues And Body Fluids From Patients Known To Have, Or At High Risk Of Having A Transmissible Spongiform Encephalopathy (Tse)

http://www.bfwhospitals.nhs.uk/about/foi/part_two/cat8/documents/ic_docs/Corp_Proc_031.pdf

TRANSMISSABLE SPONGIFORM ENCEPHALOPATHIES (TSEs) POLICY LEAD DIRECTOR: Alex Horne, Medical Director POLICY APPROVED BY: Executive Management Team DATE POLICY APPROVED: June 2007 IMPLEMENTATION DATE: June 2007 REVIEW DATE: June 2009

snip...

6.0 Occupational Exposure

Although cases of CJD/vCJD have been reported in healthcare workers, there have been no confirmed cases linked to occupational exposure. It is important to take a precautionary approach. The highest potential risk is from exposure to high infectivity tissues through direct inoculation as a result of sharps injuries, puncture wounds or contamination of broken skin or exposure of the mucous membranes.

If a TSE were to develop as a result of occupational exposure, it may only become apparent decades later. It is a requirement of the COSHH regulations that employers keep a list of employees exposed to human TSE agents for 40 years following the last known exposure (for known and suspected). This list is not required for those involved in routine clinical care of patients with CJD or a related disorder, but does include those who have been involved in any invasive procedure.

An official record will be made of all incidents with infectious or potentially infectious material involving the exposure of individuals. This will apply whether or not the accident is reportable under RIDDOR.

The COSHH regulations provide the framework to control the risk from a range of hazardous substances including biological agents. Schedule 9 of the regulations includes the TSE agents.

All employees must have a clear understanding of identifiable risks to their health arising from work and the actions to be taken in dealing with situations in which exposure may occur.

If there is any incident with a known exposure of staff to potentially infectious material, then there must be an urgent incident meeting to consider up-to-date evidence on treatment options, along with infection control aspects of the incident.

This will include: Infection Control Doctor - Infection Control Nurse - Occupational Health - Consultant Communicable Disease Control - Manager of Staff Member

snip...

http://www.nelmht.nhs.uk/downloads/TSEs%20Policy%20CG036.pdf

Sunday, December 16, 2007 Risk factors for sporadic Creutzfeldt-Jakob disease Sunday, December 16, 2007 Risk factors for sporadic Creutzfeldt-Jakob disease Published Online: 11 Dec 2007

Copyright © 2007 American Neurological Association

http://creutzfeldt-jakob-disease.blogspot.com/2007/12/risk-factors-for-sporadic-creutzfeldt.html

SEAC 99th meeting on Friday 14th December 2007

snip...

© SEAC 2007

New research

4. Preliminary, unpublished results of research from the Health Protection Agency, aimed at addressing some of the uncertainties in the risk assessments, were reviewed by SEAC (SEAC 97, May 2007). The prion agent used in these studies is closely related to the vCJD agent. This research, using a mouse model, shows that following inoculation of mouse-adapted bovine spongiform encephalopathy (BSE) directly into the gut, infectivity subsequently becomes widespread in tissues of the oral cavity, including dental pulp, salivary glands and gingiva, during the preclinical as well as clinical stage of disease.

5. It is not known how closely the level and distribution of infectivity in the oral cavity of infected mice reflects those of humans infected with vCJD, as there are no comparable data from oral tissues, in particular dental pulp and gingiva, from human subclinical or clinical vCJD cases. Although no abnormal prion protein was found in a study of human dental tissues, including dental pulp, salivary glands and gingiva from vCJD cases22, the relationship between levels of infectivity and abnormal prion protein is unclear23. Infectivity studies underway using the mouse model and oral tissues that are presently available from human vCJD cases will provide some comparable data. On the basis of what is currently known, there is no reason to suppose that the mouse is not a good model for humans in respect to the distribution of infectivity in oral tissues. Furthermore, the new data are consistent with published results from experiments using a hamster scrapie model24.

6. A second set of experiments using the same mouse model showed that non-invasive and transient contact between gingival tissue and fine dental files contaminated with mouse-adapted BSE brain homogenate transmits infection very efficiently. It is not known how efficient gingival transmission would be if dental files were contaminated with infectious oral tissues and then subsequently cleaned and sterilised, a situation which would more closely model human dental practice. Further studies using the mouse model that would be more representative of the human situation, comparing oral tissues with a range of doses of infectivity, cleaned and sterilised files and the kind of tissue contact with instruments that occurs during dentistry, should be considered.

7. SEAC considered that the experiments appear well designed and the conclusions justified and reliable, while recognising that the research is incomplete and confirmatory experiments have yet to be completed. It is recommended that the research be completed, submitted for peer-review and widely disseminated as soon as possible so others can consider the implications. Nevertheless, these preliminary data increase the possibility that some oral tissues of humans infected with vCJD may potentially become infective during the preclinical stage of the disease. In addition, they increase the possibility that infection could potentially be transmitted not only via accidental abrasion of the lingual tonsil or endodontic procedures but a variety of routine dental procedures.

20 Department of Health (2006) Dentistry and vCJD: the implications of a carrier-state for a self-sustaining epidemic. Unpublished. 21 SEAC (2006) Position statement on vCJD and endodontic dentistry.

http://www.seac.gov.uk/statements/statement0506.htm

22 Head et al. (2003) Investigation of PrPres in dental tissues in variant CJD. Br. Dent. J. 195, 339-343. 23 SEAC 90 reserved business minutes.

Implications for transmission risks

snip...PLEASE SEE DISTURBING FINDINGS FULL TEXT HERE ;

http://seac992007.blogspot.com/

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

Nadège Bourvis1,2, Pierre-Yves Boelle1,2,3, Jean-Yves Cesbron4,5,6, Alain-Jacques Valleron1,2,3*

1 Université Pierre et Marie Curie-Paris6, Unité de Recherche Epidémiologie-Systèmes d'information-Modélisation, UMR S 707, Paris, France, 2 INSERM, U707, Paris, France, 3 Assistance Publique-Hôpitaux de Paris (AP-HP), Unité de Santé Publique, Hôpital St Antoine, Paris, France, 4 Laboratoire Adaptation et de Pathogénie des Micro-organismes, Université Joseph Fourier, UMR 5163, Grenoble, France, 5 Centre National de la Recherche Scientifique (CNRS), UMR 5163, Grenoble, France, 6 Centre hospitalier universitaire (CHU) de Grenoble, Laboratoire d'Immunologie, Grenoble, France

Abstract

Background

Experimental results evidenced the infectious potential of the dental pulp of animals infected with transmissible spongiform encephalopathies (TSE). This route of iatrogenic transmission of sporadic Creutzfeldt-Jakob disease (sCJD) may exist in humans via reused endodontic instruments if inadequate prion decontamination procedures are used.

Methodology/Principal Findings

To assess this risk, 10 critical parameters in the transmission process were identified, starting with contamination of an endodontic file during treatment of an infectious sCJD patient and ending with possible infection of a subsequent susceptible patient. It was assumed that a dose-risk response existed, with no-risk below threshold values. Plausible ranges of those parameters were obtained through literature search and expert opinions, and a sensitivity analysis was conducted. Without effective prion-deactivation procedures, the risk of being infected during endodontic treatment ranged between 3.4 and 13 per million procedures. The probability that more than one case was infected secondary to endodontic treatment of an infected sCJD patient ranged from 47% to 77% depending on the assumed quantity of infective material necessary for disease transmission. If current official recommendations on endodontic instrument decontamination were strictly followed, the risk of secondary infection would become quasi-null.

Conclusion

The risk of sCJD transmission through endodontic procedure compares with other health care risks of current concern such as death after liver biopsy or during general anaesthesia. These results show that single instrument use or adequate prion-decontamination procedures like those recently implemented in dental practice must be rigorously enforced.

snip...

http://www.plosone.org/article/info:doi/10.1371/journal.pone.0001330

http://www.plosone.org/article/fetchObjectAttachment.action;jsessionid=A92C286CBD5A9668069613D3CD070D90?uri=info%3Adoi%2F10.1371%2Fjournal.pone.0001330&representation=PDF

Conclusions 14. Preliminary research findings suggest that the potential risk of transmission of vCJD via dental procedures may be greater than previously anticipated. Although this research is incomplete, uses an animal model exposed to relatively high doses of infectivity, and there are no data from infectivity studies on human oral tissues, these findings suggest an increased possibility that vCJD may be relatively efficiently transmitted via a range of dental procedures. Ongoing infectivity studies using human oral tissues and the other studies suggested here will enable more precise assessment of the risks of vCJD transmission through dental procedures.

15. Guidance was issued to dentists earlier this year recommending that endodontic files and reamers be treated as single use which, provided it is adhered to, will remove any risk of a self-sustaining epidemic arising from re-use of these instruments. To minimise risk it is critical that appropriate management and audit is in place, both for NHS and private dentistry.

16. It is also critical that a detailed and comprehensive assessment of the risks of all dental procedures be conducted as a matter of urgency. While taking into account the continuing scientific uncertainties, this will allow a more thorough consideration of the possible public health implications of vCJD transmission via dentistry and the identification of possible additional precautionary risk reduction measures. The assessment will require continued updating as more evidence becomes available on the transmissibility of vCJD by dental routes, and on the prevalence of infection within the population. A DH proposal to convene an expert group that includes dental professionals to expedite such an assessment is welcomed. Given the potential for transmission via dentistry, consideration should be given to the urgent assessment of new decontamination technologies which, if proved robust and effective, could significantly reduce transmission risks.

SEAC June 2007

References 1Smith et al. (2003) Prions and the oral cavity. J. Dent. Res. 82, 769-775.

2Smith et al. (2005) Residual protein levels on reprocessed dental instruments. J. Hosp. Infect. 61, 237-241.

3Everington et al. (2007) Dental treatment and risk of variant CJD – a case control study. Brit. Den. J. 202, 1-3.

4Department of Health. (2003) Risk assessment for vCJD and dentistry.

5 Department of Health (2006) Dentistry and vCJD: the implications of a carrier-state for a self-sustaining epidemic. Unpublished.

6SEAC (2006) Position statement on vCJD and endodontic dentistry.

http://www.seac.gov.uk/statements/statement0506.htm

7Head et al. (2003) Investigation of PrPres in dental tissues in variant CJD. Br. Dent. J. 195, 339-343.

8SEAC 90 reserved business minutes.

9Ingrosso et al. (1999) Transmission of the 263K scrapie strain by the dental route. J. Gen. Virol. 80, 3043-3047.

10Department of Health (2006) Dentistry and vCJD: the implications of a carrier-state for a self-sustaining epidemic. Unpublished.

11Clarke & 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.

12SEAC Epidemiology Subgroup (2006) position statement of the vCJD epidemic.

http://www.seac.gov.uk/statements/st...06subgroup.htm

13DH (2007) Precautionary advice given to dentists on re-use of instruments

http://www.gnn.gov.uk/environment/fu...partment=False

Page updated: 8 June, 2007

http://www.seac.gov.uk/statements/state-vcjd-dentrstry.htm

Subject: MASTER DENTIST FALLS VICTIM TO CJD Date: March 31, 2007 at 1:27 pm PST

''It was in the cards a long time ago,'' she says. ''We've put it in the hands of God.''

- Crystal Harmon can be reached at 894-9643 or by e-mail at mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000134/!x-usc:mailto:charmon@bc-times.com.

http://www.mlive.com/news/bctimes/index.ssf?/base/news-9/1175181333132150.xml&coll=4

Occupational risk factors for the sporadic form of Creutzfeldt-Jakob disease. Cocco PL, Caperna A, Vinci F. Dipartimento di Sanità Pubblica, Sezione di Medicina del Lavoro, Università di Cagliari, via San Giorgio 12, 09124 Cagliari. mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000134/!x-usc:mailto:coccop@pacs.unica.it

1: Med Lav. 2003 Jul-Aug;94(4):353-63.

snip...

CJD cases represented a wide variety of occupations (159) and industries (147). Among occupations and industries, for which previous reports suggested potential exposure to a transmissible spongiform encephalopathy (TSE) agent, the OR for CJD was significantly increased among butchers (OR = 6.8, 95% C.I. 1.5, 30.1, based on 4 cases and 3 controls), and persons working in offices of physicians (OR = 4.6, 95% C.I. 1.2, 17.6 based on 5 cases and 4 controls). Nine other occupations and seven other industries, for which no previous suggestion existed in the literature, also showed significant associations. Overall, our results suggest that occupational exposures are not an important source of sCJD infection. However, as the excess among butchers and some workers in health occupations was consistent with previous reports, more indepth research is warranted to address the hypothesis.

http://www.ncbi.nlm.nih.gov/pubmed/14526494

Saturday, December 08, 2007 Transfusion Transmission of Human Prion Diseases

http://vcjdblood.blogspot.com/2006/12/vcjd-case-study-highlights-blood.html

Thursday, July 24, 2008Prion diseases are efficiently transmitted by blood transfusion in sheep Submitted April 18, 2008 Accepted June 28, 2008

http://vcjdblood.blogspot.com/2008/07/prion-diseases-are-efficiently.html

Saturday, January 20, 2007 Fourth case of transfusion-associated vCJD infection in the United Kingdom

http://vcjdtransfusion.blogspot.com/2007/01/fourth-case-of-transfusion-associated.html http://vcjdblood.blogspot.com/

OCCUPATIONAL EXPOSURE FROM TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES TSEs IN THE USA

let me count the ways. ...tss

Manuscript Draft Manuscript Number: Title: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory Article Type: Personal View Corresponding Author: Mr. Terry S. Singeltary, Corresponding Author's Institution: na First Author: Terry S Singeltary, none Order of Authors: Terry S Singeltary, none; Terry S. Singeltary Abstract: TSEs have been rampant in the USA for decades in many species, and they all have been rendered and fed back to animals for human/animal consumption. 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 2007.

http://www.regulations.gov/fdmspublic/ContentViewer?objectId=090000648027c28e&disposition=attachment&contentType=pdf

Scrapie

http://scrapie-usa.blogspot.com/

NOR-98

http://nor-98.blogspot.com/2008_04_01_archive.html

Friday, July 18, 2008
TSE risk assessment from carcasses of ovine and caprine animals below 6 months of age from TSE infected flocks intended for human consumption

http://nor-98.blogspot.com/2008/07/tse-risk-assessment-from-carcasses-of.html

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 ;

http://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html

Transmissible Mink Encephalopathy TME

http://transmissible-mink-encephalopathy.blogspot.com/

Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518

Labels: bse; cwd, occupational exposure, PRION, SCRAPIE, SPORADIC CJD, TME

CREUTZFELDT JAKOB DISEASE

Tuesday, August 12, 2008

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

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