Thursday, April 07, 2016

What is the risk of chronic wasting disease being introduced into Great Britain? An updated Qualitative Risk Assessment March 2016

What is the risk of chronic wasting disease being introduced into Great Britain? An updated Qualitative Risk Assessment March 2016

 


 

Summary

 

The previous assessment concentrated on the incursion of disease from North America through the imports of animal feed or the movement of contaminated clothing, footwear and equipment. The results suggested that import of pet feed was a non-negligible risk, but given the unlikely contact of resident deer in GB with such non-ruminant feed, this was considered overall a negligible to very low risk. The movement of contaminated clothing, footwear or equipment (particularly hunting equipment) could pose a very low risk, although the volume of contaminated soil which would need to be ingested to give rise to an infection is likely to be higher than would be present. There is a variable level uncertainty in all these assessments.

 

The new assessment focuses on an additional potential route of entry: the importation of natural deer urine lures. The main conclusions from this assessment are:

 

 In areas of North America where CWD has been reported, given that CWD is excreted in faeces, saliva, urine and blood, and survives in the environment for several years there is a medium probability that the deer urine in North America contains CWD (high uncertainty; depends on the source of deer used for production).

 

 The risk of a deer in GB being infected per 30 ml bottle of urine imported from the USA is very low, albeit with high uncertainty. Overall it is concluded that the risk of at least one infection of deer in the UK with CWD per year from deer urine lures imported from the USA is medium. This assumes a high number of 30 ml bottles imported per year from all areas of the USA.

 

 None of the species affected by CWD in North America are present in GB. For a British species to become infected with CWD following exposure, the dose and inherent susceptibility of the species will be important. Based on current scientific evidence Red deer (Cervus elaphus elaphus) are susceptible to CWD, Fallow deer (Dama dama) are likely to be less susceptible and Roe deer (Capreolus capreolus) have a gene conferring susceptibility. Therefore, it is likely that given exposure to an infectious dose of CWD, deer in GB could become infected with CWD.

 

Overall, the probability of importing CWD into GB from North America and causing infection in British deer is uncertain but likely to be negligible to very low via movement of deer hunters, other tourists and British servicemen and very low via imported (non-

 

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ruminant) animal feed and medium for the use of lures. However, if it was imported and (a) deer did become infected with CWD, the consequences would be severe as eradication of the disease is impossible, it is clinically indistinguishable from BSE infection in deer (Dalgleish et al., 2008) and populations of wild and farmed deer would be under threat.

 

The USA has implemented a Herd Certification Programme for farmed and captive cervids. So far, 29 States are approved for HCP status (APHIS, 2015). The list includes States such as Colorado, where CWD is present, therefore it is recommended that any sourcing of such natural urine lures should be not only from States with an HCP programme, but also from a herd which is registered as being regularly tested free of CWD.

 

Animal urine is not considered a commodity which is subject to animal by-products legislation for imports. Internet sales are common and although a license would be required, there are no conditions for the safe sourcing of such products. Deer urine lures are also available in Europe and may be produced from carcases of hunted deer. The use of deer urine produced from a species not present in Europe (such as white tailed deer) is questioned for its value with native GB deer according to the British Deer Society survey.

 

Background

 

Chronic wasting disease (CWD) is a highly infectious transmissible spongiform encephalopathy (TSE) that is circulating in the wild and farmed cervid populations of North America. It is the only TSE maintained in free-ranging wild animal populations. A feature of CWD is that it is able to transmit both directly (animal-to-animal) and indirectly via the contaminated environment. In particular, CWD prions are able to bind to and survive in the soil in a bio-available form for many years without any decrease in infectivity. This makes eradication of the disease from a wild population increasingly challenging. Thus far, there have been no reported cases of CWD or other TSE in deer in Great Britain (GB). This is based on surveys of wild and farmed red deer (Cervus elaphus elaphus) (EFSA, 2011). Given the consequences of CWD observed in North America, it is of high importance that GB remains free of the disease. Further, as the clinical signs of CWD in deer are similar to those of deer experimentally infected with bovine spongiform encephalopathy (BSE), all infected deer would need to be tested to differentiate if they were infected with CWD or BSE to minimise the risk of BSE entering the human food chain via affected venison. In 2015, the British Deer Society (BDS) carried out an online survey of BDS and BASC members to gather evidence about the use of deer urine as a lure. Fifteen percent of respondents (~1,800) answered yes about knowing that deer urine was used as a lure. Of the respondents, less than 2% responded yes to using such a product themselves. Of those that use the product, 50% had sourced the product from the USA, while 20% use more than a litre in volume a year and ~70% is natural (as opposed to synthetic).

 

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Hazard identification The hazard is identified as Chronic Wasting Disease

 

Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy (TSE) affecting cervids. It is the only TSE maintained in free-ranging wild animal populations; other TSE’s are mostly restricted to captive domestic livestock populations or humans. Chronic wasting disease was first identified as a clinical disease of captive mule deer in Colorado in 1967 and later classified as a TSE in 1978 (Williams & Miller, 2003). The origin of the disease is unknown and may have been a spontaneous TSE that arose in deer. Currently, natural infections of CWD have been reported in mule deer (Odocoileus hemionus hemionus), black-tailed deer (Odocoileus hemionus columbianus), white-tailed deer (Odocoileus virginianus), Rocky Mountain elk (Cervus elphus nelsoni), Shira’s moose (Alces alces shirasi) and mule deer and white-tailed deer hybrids (Hamir et al., 2008). Other species of elk may also be susceptible. The disease is restricted to North America except for isolated cases of infected elk being exported from Canada to South Korea (Williams & Miller, 2003). In the last decade, with increased CWD testing, a more widespread distribution of CWD within North America has been observed and the geographic distribution is increasing (APHIS, 2015) (Figure 1).

 

Figure 1: Current range of CWD in North America (USGS, 2016)

 

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This more widespread distribution may be due to enhanced surveillance but also to natural migration of cervids and translocation of infected animals by humans (EFSA, 2011). Within affected areas, the prevalence varies. In the endemic area of Wyoming, for example, the prevalence of CWD in mule deer has increased from approximately 11% in 1997 to 36% in 2007 (Almberg et al., 2011). In such areas, population declines of deer of up to 30 to 50% have been observed (Almberg et al., 2011). In areas of Colorado, the prevalence can be as high as 30% (EFSA, 2011).

 

The clinical signs of CWD in affected adults are weight loss and behavioural changes that can span weeks or months (Williams, 2005). In addition, signs might include excessive salivation, behavioural alterations including a fixed stare and changes in interaction with other animals in the herd, and an altered stance (Williams, 2005). These signs are indistinguishable from cervids experimentally infected with bovine spongiform encephalopathy (BSE). Given this, if CWD was to be introduced into countries with BSE such as GB, for example, infected deer populations would need to be tested to differentiate if they were infected with CWD or BSE to minimise the risk of BSE entering the human food-chain via affected venison.

 

The duration of clinical disease is highly variable and death can occur within 4 weeks but some infected animals may survive as long as a year (Williams, 2005). The incubation period is a minimum of approximately 16 months and is more likely to be between 2 and 4 years (Williams, 2005). In affected elk, the incubation period is between 1.5 and 3 years after which they become clinically affected and may succumb less than 12 months after initial clinical signs appear (Miller et al., 1998). During the pre-clinical period, the animal is infectious (Almberg et al., 2011).

 

The CWD agent or Prion Protein (PrPCWD) in affected animals is distributed firstly in the gut associated lymphoid tissues, digestive tract (e.g. tonsils, Peyer’s patches, mesenteric lymph nodes) and then in the brain and spinal cord as the disease progresses (Sigurdson, 2008). Prions of CWD have also been found in muscle tissue (Angers et al., 2006) (see Figure 2). The distribution and levels of PrPCWD in tissues differ between species (e.g. elk versus deer).

 

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Figure 2: Diagram displaying the main organs affected by CWD in infected cervids (http://www.dnr.state.mn.us/mammals/deer/cwd/index.html)

 

Given its propensity to colonise the digestive tract, evidence suggests the prion is excreted in faeces (Safar et al., 2008), urine and saliva potentially leading to direct and indirect transmission between cervid species. Indeed, the disease is transmitted horizontally with high efficiency and circumstantial evidence suggests that environmental contamination with CWD prions contributes to the maintenance of CWD in affected areas (Safar et al., 2008). The rate of transmission of CWD has been reported to be as high as 30% and can approach 100% among captive animals in endemic areas (Safar et al., 2008). The efficiency of CWD transmission is unparalleled among TSE diseases (EFSA, 2011). Trifilo et al., (2007), using a murine tg mouse model, established that CWD can be transmitted via the oral route. Indeed, the distribution of PrPres in the orally infected mice (e.g. in the spleen and lymph nodes) mimicked what has been reported in deer developing CWD via natural infection (Trifilo et al., 2007). Modelling studies also support the theory that transmission of CWD in deer herds is maintained by contact with a prion contaminated environment (Almberg et al., 2011). Scavenging of CWD-infected carcasses provides another route of releasing the prion into the environment and exposure of non-cervid species (Sigurdson, 2008). This indirect transmission route is problematic as it not only increases the basic reproductive number but also because there are very few effective mitigation strategies for reducing the risk from indirect transmission. This is due to the fact that the agent is extremely resistant in the environment and able to bind to soil particles making eradication and control of CWD a major obstacle in both farmed and free-ranging cervid populations.

 

Sheep and cattle may be exposed to CWD via common grazing areas with affected deer but so far, appear to be poorly susceptible to mule deer CWD (Sigurdson, 2008). In contrast, cattle are highly susceptible to white-tailed deer CWD and mule deer CWD in experimental conditions but no natural CWD infections in cattle have been reported (Sigurdson, 2008; Hamir et al., 2006). It is not known how susceptible humans are to CWD but given that the prion can be present in muscle, it is likely that humans have been exposed to the agent via consumption of venison (Sigurdson, 2008). Initial experimental research, however, suggests that human susceptibility to CWD is low and there may be a robust species barrier for CWD transmission to humans (Sigurdson, 2008). It is apparent, though, that CWD is affecting wild and farmed cervid populations in endemic areas with some deer populations decreasing as a result.

 

Thus far, CWD is restricted to North America with the exception of imported infected animals into South Korea from Canada. Surveys of wild and farmed cervid populations in the European Union between 2006 and 2010 did not identify any TSEs (EFSA, 2011). As part of this survey, 601 farmed and 598 wild red deer (Cervus elaphus elaphus) were tested (EFSA, 2010). These included clinical/sick animals, fallen stock, healthy shot/slaughtered animals and road killed animals. Based on the survey results, it can be concluded that the prevalence of CWD in the EU is less than 0.5%. It is important, therefore, to ensure that the disease is not introduced into Europe and establish within the

 

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EU wild and farmed cervid population as the probability of being able to eradicate the disease would be very small.

 

Risk Question

 

This risk assessment considers the risk posed to the Great Britain (GB) deer population if chronic wasting disease (CWD) was imported from North America (i.e. Canada and the United States). The specific risk question addressed is: What is the risk of CWD being introduced into Great Britain (GB) from North America and causing infection in deer?

 

To answer the above question, the risk assessment follows the OIE framework of release (or entry), exposure and consequence assessment. Specifically, it is divided into the three key areas:

 

1. What is the probability of introducing CWD into GB from North America?

 

2. What is the probability of a deer species in GB being exposed to the CWD prion?

 

3. What is the probability of a GB deer species becoming infected with CWD upon exposure to the prion?

 

Risk Assessment

 

Terminology related to the assessed level of risk

 

For the purpose of the risk assessment, the following terminology will apply (OIE, 2004):

 

Negligible

 

So rare that it does not merit to be considered

 

Very low

 

Very rare but cannot be excluded

 

Low

 

Rare but does occur

 

Medium

 

Occurs regularly

 

High

 

Occurs often

 

Very high

 

Event occurs almost certainly

 

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Entry assessment

 

The routes by which CWD may be introduced into GB from North America include:

 

 Importation of live deer

 

 Importation of deer urine lures

 

 Importation of meat and other products derived from cervid species (e.g. trophy items including antlers, semen)

 

 Importation of animal feed

 

 Hunters and other tourists and British servicemen travelling from affected areas to GB with contaminated equipment (e.g. boots, clothing, knives)

 

Currently, according to the European Union Trade Control and Expert System (TRACES) database, GB does not import live cervids, ‘other’ animal meat products or raw hides and skins. This was the same conclusion drawn by EFSA (2004) who stated GB does not import cervids or products from North America. Therefore, the three routes which this assessment focuses on are:

 

1) importation of animal feed

 

2) importation of deer urine lures

 

3) importation of CWD prion on contaminated equipment and clothing/footwear of hunters or other tourists and British servicemen

 

Importation of animal feed

 

Animal feed encompasses all feed fed to farmed livestock, horses, pets, farmed fish, zoo and circus animals and also animals living freely in the wild. Currently, legislation for animal feed relating to production, and labelling and composition is harmonised at the EU level and, in GB, is the responsibility of the Food Standards Agency (FSA). In addition, Defra is responsible for ABP Regulations which includes pet food manufacturing regulation.

 

Pet food containing material of animal origin, according to EU Regulation (EC) No. 142/2011 on Animal By-Products, must be derived from animals inspected and passed as fit for human consumption prior to slaughter (Category 3 material). Further, the products are subject to strict microbiological criteria for Enterobacteriaceae and Salmonella. Under the EU Regulation, imported pet food produced using Category 3 processed animal proteins (PAP) must adhere to the same standards as that produced within the EU. More specifically, the imported pet food must satisfy the following criteria:

 

 The PAP must have been produced in accordance with the same requirements as PAP for placing on the market in the EU

 

 The PAP must have been sampled and tested to satisfy certain bacteriological criteria in accordance with the Regulations before release onto the EU market

 

 The product must enter the EU under correct Health Certification

 

 The Health Certification signed by the veterinarian or official inspector responsible for the rendering plant in the exporting country must verify that EU standards of

 

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sourcing of animal by-products, processing and sampling are met for each consignment.

 

These requirements apply to canned pet food, processed pet food other than canned pet food, dog chews, raw pet food and flavouring innards.

 

According to TRACES, GB imports processed pet food from Canada and the United States of America (USA). In November and December 2015, for example, GB imported 13.6112 tonnes of processed cat and dog food (including dog chews) containing products of ungulate origin from Canada and USA. There are a limited number of processed pet food products made in the USA containing (roasted) venison for the cat and dog food market that are available in GB (e.g. Taste of the Wild pet food). Venison is high in iron content and considered a good alternative meat product for pets with intolerance to certain meat proteins. The specific amount of pet food products imported into GB from North America containing deer protein is unknown and not specified in the TRACES system, but is likely to be a small percentage of the overall amount of processed pet food imported. The TRACES database indicates that in the same period (Nov – Dec 2015) 751,418 tonnes of other products for pet food were imported into the UK from USA and Canada.

 

In the USA, under the Food and Drug Administration’s BSE Feed Regulation (21 CFR 589.2000) most material (exceptions include milk, tallow, and gelatin) from deer and elk is prohibited for use in feed for ruminant animals. With regards to feed for non-ruminant animals, under FDA law, CWD positive deer may not be used for any animal feed or feed ingredients. ***For elk and deer considered at high risk for CWD, the FDA recommends that these animals do not enter the animal feed system. ***However, this recommendation is guidance and not a requirement by law.

 

***Animals considered at high risk for CWD include:

 

***1) animals from areas declared to be endemic for CWD and/or to be CWD eradication zones and

 

***2) deer and elk that at some time during the 60-month period prior to slaughter were in a captive herd that contained a CWD-positive animal.

 

***Therefore, in the USA, materials from cervids other than CWD positive animals may be used in animal feed and feed ingredients for non-ruminants. The amount of animal PAP that is of deer and/or elk origin imported from the USA to GB cannot be determined, however, as it is not specified in TRACES. It may constitute a small percentage of the very low tonnage of non-fish origin processed animal proteins that were imported from US into GB.

 

*** Overall, therefore, it is considered there is a greater than negligible risk that (non-ruminant) animal feed and pet food containing deer and/or elk protein is imported into GB. There is uncertainty associated with this estimate given the lack of data on the amount of deer and/or elk protein possibly being imported in these products.

 

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Movement of hunters, other tourists and British servicemen

 

Probability that the environment in North America is contaminated with CWD

 

As outlined in Figure 1, there are 21 states and provinces in the USA and Canada where CWD has been detected in farmed and wild cervids. These include: Alberta (Canada), Arkansas, Colorado, Illinois, Kansas, Maryland, Minnesota, Missouri, Nebraska, New Mexico, New York, North Dakota, Saskatchewan (Canada), South Dakota, Utah, Virginia, West Virginia, Wisconsin and Wyoming. In these areas, the environment is likely to be contaminated with CWD prions from direct excretion of the prion in various bodily fluids of infected animals, and leaching of prions into the soil from decaying carcasses of infected animals. A summary of the current studies on CWD in faeces, urine, blood and other bodily fluids or organs is summarised in Table 1.

 

Table 1: Summary of the studies on CWD prion excretion

 

Fluid/organ

 

Study summary

 

Reference

 

Faeces

 

 Faeces were the source of CWD infection in Syrian hamsters under experimental conditions.

 

 Prions remain intact after being passed through the digestive tract and, therefore, are a viable source of infectivity in the environment.

 

 CWD prions are excreted in the faeces of infected mule deer 7 to 11 months prior to the onset of neurological signs (i.e. during the incubation period)

 

Safar et al., (2008) Tamguney et al. (2009)

 

Saliva

 

 During studies of oral transmission using a murine tg mouse model, it was observed that prior to and during clinical disease, serous and mucous glands contained PrPres.

 

 Three naïve fawns were orally inoculated with 50ml of saliva from an infected deer in 3 doses over a 3 day period. Eighteen months post inoculation, CWD prions were detected in all 3 fawns during tonsil biopsy.

 

 Pooled saliva from five terminally CWD infected white-tailed deer was inoculated into nine tg1536 mice. Eight of the nine mice developed disease consistent with a TSE at 342 ± 109 days post inoculation suggesting infectious prions are present in saliva of infected cervids.

 

Trifilo et al., (2007)

 

Mathiason et al., (2006)

 

Haley et al., (2009)

 

Blood

 

 Two naïve white-tailed deer were inoculated intraperitoneally with 250 ml of frozen citrated blood and a further fawn with an intravenous transfusion of 250 ml freshly citrated whole blood. After 18 months post inoculation, all three fawns had CWD prions in their tonsils and retropharyngeal lymph node.

 

Mathiason et al., (2006)

 

Urine

 

 Pooled urine from five terminally CWD infected white-tailed deer was inoculated into nine tg Haley et al., (2009)

 

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mice. Two of the nine mice developed disease consistent with a TSE at 370 and 376 days post inoculation suggesting infectious prions are present in the urine of infected cervids but at a lower infectivity than other bodily fluids such as saliva.

 

 Henderson et al. (2015) report urine from CWD-infected deer contained 1 intracerebral LD50 per 10-20 ml. But their LD50 is estimated from amyloid formation rate and may be difficult to relate to an oral LD50 for live deer.

 

 Henderson et al. (2015) cite Gonzalez-Romero et al. 2008 which used PMCA to show that levels of prions in neat urine were approximately equivalent to a 10-10 to 10-11 dilution of brain.

 

 The levels in urine may be so low as to be difficult to quantify by bioassay.

 

 Assumed estimate below that the oral LD50 for a deer may be 1,000 litres of urine from a CWD-infected deer. This assumes the oral route is 100,000-fold less efficient than the ic route. Henderson et al. (2015)

 

Antler velvet

 

 Antlers are covered by a layer of skin, velvet, which is shed after an increase in testosterone and ossification of antlers. CWD is present at low, but detectable, amounts in antler velvet from infected elk.

 

Angers et al., (2009)

 

It is clear from Table 1 that CWD is excreted in several different bodily fluids and, as demonstrated in experimental studies, can be a source for onward transmission to naïve animals. Infected carcasses decaying naturally in confined areas can also lead to new CWD infections in naïve deer (Sigurdson, 2008). This was proved, experimentally, by Miller et al., (2004) during a study of environmental transmission. Specifically, 3 naïve mule deer were stocked in an 800m2 paddock in which a naturally infected mule deer had died and decomposed approximately 1.8 years prior. In a second paddock, a further 3 naïve mule deer were placed where infected mule deer had resided 2.2 years earlier and contaminated the environment with their faeces (Miller et al., 2004). The experiment was conducted in 3 replicates. In total, 3 out of 12 and 1 out of 9 deer were infected by being exposed to an infected decomposed carcass or residual excreta, respectively.

 

The CWD prion has also been detected in water. Specifically, very low levels (below infectious levels) were detected in a water sample from melting winter snow-pack from an endemic area (Nichols et al., 2009). The data showed persistence of CWD prions in water, accumulated levels of which, it is hypothesised, may promote transmission within deer herds.

 

Once in the environment, TSE prions can bind to soil particles and remain infectious (Saunder et al., 2010). Indeed, Johnson et al., (2006) demonstrated that the disease-

 

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associated form of the prion protein can bind to all soil mineral surfaces and is preserved in a bioavailable form. Further, in a later study, Johnson et al., (2007) observed that prions bound to the soil mineral montmorillonite (Mte) significantly enhanced disease penetrance and reduced the incubation period compared to unbound prions. The reason why binding to Mte or other soil components enhances transmissibility is unclear but it may provide some protection for the prion in the gut against denaturation allowing more agent to be absorbed by the animal (Johnson et al., 2007). Further, binding to the soil particles maintains prions near the soil surface increasing the probability of animal exposure (Russo et al., 2009).

 

In addition to the enhanced infectivity, prions can remain in the soil for several years as the agents are resistant to inactivation by most chemical agents, radiation and heat (Johnson et al., 2006). Seidel et al. (2007), for example, demonstrated that scrapie agent (strain 263K) remains persistent in soil over a period of at least 29 months and remains highly infectious to Syrian hamsters in oral inoculation experiments. In Iceland during an epidemiological investigation of scrapie, a TSE of sheep and goats, Georgsson et al., (2006) reported that the scrapie agent survived on a farm for at least 16 years. However, Russo et al., (2009) demonstrated experimentally that reactive soil components such as manganese oxides may contribute to the inactivation process of TSE prions in soil. The authors did not study CWD prion specifically but the study highlights the complexity of the effect the inorganic and organic constituents in soil may have on prion survival and infectivity.

 

*** In summary, in endemic areas, there is a medium probability that the soil and surrounding environment is contaminated with CWD prions and in a bioavailable form. In rural areas where CWD has not been reported or only very recently reported and deer are present, there is a greater than negligible risk the soil is contaminated with CWD prion.

 

Movement of deer hunters, other outdoor tourists and British servicemen between North America and GB

 

The probability a person comes into contact with CWD prions varies depending upon their place of residence and/or their involvement with outdoor pursuits (e.g. hunting). In this assessment, focus is given to the following groups of people:

 

 Residents in CWD affected areas travelling to GB (particularly the countryside) and British tourists travelling to CWD affected areas

 

 North American hunters travelling to GB to hunt/stalk deer and British hunters travelling to North America to hunt deer

 

 British servicemen training in and/or near CWD affected areas

 

All other people (e.g. city tourists and residents) are considered to pose a lower risk of being exposed to CWD in North America and, therefore, arriving in GB with contaminated clothing, footwear and/or equipment.

 

There are limited data on the number of North American tourists, stratified by location of residence, arriving in the UK. Therefore, it is not possible to ascertain of the 3.89 million

 

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visitors from the United States (US) to the United Kingdom (UK) in 2006, for example, how many were from CWD affected areas. Likewise, there is no breakdown of where in the USA the 4.5 million UK visitors travelled in 2008. This is a significant data gap in the assessment.

 

Hunting in the US is a popular sport with 4% of the population (10.1 million) involved in deer hunting in 2006 (USFWS, 2011). Of these people, 4.7 million only hunted deer whilst the remainder hunted deer and other species (e.g. small game, bears). Further, 58% of deer hunters were involved in wildlife watching activities and other outdoor pursuits (e.g. hiking, fishing); this is compared to 31% of the general public (USFWS, 2011; TAMS, 2007). Individuals that hunted whilst on a trip were likely to come from northern and western states (e.g. Alaska, Wyoming, North Dakota, South Dakota, Idaho and Montana) compared to highly urbanised states. In Canada, those individuals participating in hunting activities are most likely to do so within Canada with the majority taking a trip within their own province or region (TAMS, 2006). Canadian hunters were also more likely than other Canadian pleasure travellers to participate in wilderness activities and hiking (TAMS, 2006). There are no data collated on the number of hunters from North America travelling to the UK to stalk/hunt deer and, vice versa, there are no data on the number of UK residents hunting in North America. However, in order to hunt in GB with your own rifle, a visitor firearms permit has to be obtained from the police force in one of the devolved countries. In 2011, 123 licences were granted by the Scottish Police Force for non-EU residents (BASC, pers. Comm., 2012). This includes not only individuals from North America but also Norway and other non-EU countries (BASC, pers. Comm., 2012). The number of hunters arriving without their own rifle and participating in an organised hunting package/holiday is unknown. However, it is likely the total number of hunters from North America is in the low hundreds; the actual number, however, is highlighted as a significant data gap.

 

As well as tourists, British servicemen frequently move between North America and GB. In particular, British Army servicemen use the Canadian Forces base at Suffield, Alberta to take part in extensive training exercises. Suffield is located in the southwest of Alberta and comprises of 2,690km2 of prairie landscape. The eastern boundary is designated as a wildlife management area and is south of a wildlife management area in which CWD was reported as recently as 2011 (www.srd.albert.ca). Consequently, the servicemen have the potential to be in close contact with areas where CWD is present.

 

*** In summary, given the volume of tourists, hunters and servicemen moving between GB and North America, the probability of at least one person travelling to/from a CWD affected area and, in doing so, contaminating their clothing, footwear and/or equipment prior to arriving in GB is greater than negligible. For deer hunters, specifically, the risk is likely to be greater given the increased contact with deer and their environment. However, there is significant uncertainty associated with these estimates.

 

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Probable amount of CWD prions on contaminated boots and equipment

 

Given that a hunter or tourist walks in areas which are contaminated with CWD, it is possible that they will collect soil on their boots and other equipment. This likelihood will increase if the hunter has shot and handled a CWD infected deer resulting in contamination of the hunting equipment (e.g. knives) and their clothing and they subsequently arrive in GB with this equipment, footwear and clothing. Further, the soles of hiking boots tend to retain more soil than those of normal shoes. Wilkinson (2010), for example, removed 0.1 g of soil from hiking boots after returning to GB from a 2-month research visit to Canada. The amount of CWD prion in this amount of soil will depend upon the density of CWD infected animals excreting prions into the environment and the type of soil; CWD prion binds to clay soil, for example. Animal mortality sites could also be hotspots of CWD prion given the highly infectious nervous system matter entering into the environment and soil (Saunders et al., 2010).

 

Importation of deer urine lures

 

It is well established that urine from CWD-infected deer contains CWD infectivity. The urine collected for deer lures has no processing and is immediately refrigerated and bottled (Anon 2015b). There is no inactivation of the CWD agent in the urine and thus all infectivity present at the point of bottling in the USA will still be present at the point of use in the UK. However, under EU rules, (EC Regulation 1069/2009), urine from farmed deer should fall under the definition of “manure” and therefore the import of such a product, if unprocessed, is not allowed, according to Regulation (EC) 142/2011. Nevertheless, the processing required for bottling prior to retail is unlikely to affect the CWD prion and if it did fulfil the requirements in 142/2011, the active ingredient in the urine would no longer be effective. It is therefore presumed that the urine is considered “unprocessed” under EU law. The import and transit of urine from wild deer is not covered by 1069/2009.

 

A survey conducted by the British Deer Society (BDS) in July 2015 suggested small number of hunters in GB were aware of and used deer urine lures (http://www.bds.org.uk/index.php/news-events/135-deer-urine-lure-survey-july-2015 ).

 

For the purpose of the qualitative risk assessment developed here it is necessary to estimate the probability that a 30-ml bottle of lure contains urine from an infected deer. This requires an estimate of the proportion of deer herds in the USA which are infected with CWD together with the within herd prevalence.

 

The distribution map of CWD in US shows it is present mainly in central states (Figure 1). However, Virginia in the east of the country has recorded seven recent cases of CWD (Anon 2015a). Some US manufacturers claim to take steps to prevent urine being taken from infected animals eg by sourcing from farms where the deer are randomly tested for CWD (Anon 2015a). However, if disease is already present and testing is not carried out regularly, captive populations are not necessarily disease free (Strausser 2014). Urine-based deer lures have been known to be collected from domestic white-tailed deer herds and therefore there is a recognised risk. This is reflected by 6 US States which have

 

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banned the use of natural deer urine for lures, as the deer urine may be sourced from CWD-endemic areas in the USA as well as from areas free of CWD. For example, the US State of Virginia is banning the use of urine-based deer lures on July 2015 and Vermont from 2016 due to the risk of spread of CWD. Alaska banned their use in 2012 (Anon 2015a). Pennsylvania Game Commission has banned urine-based deer lures and acknowledged that there is no way to detect their use (Strausser 2014). On the basis of unpublished data (J. Manson, Pers. Comm.) it appears that up to 50% of deer herds can be infected with 80-90% of animals infected within some herds.

 

*** It is therefore assumed that probability that a 30-ml bottle of deer urine lure imported from the USA is sources from an infected deer is medium.

 

Exposure assessment

 

Importation of animal feed

 

Once in GB, the use of animal feed is subject to the TSE Feed Ban and ABP Regulations. The BSE-related feed ban prohibits the feeding of PAP and gelatine from ruminants to ruminants (including farmed deer) or non-ruminant farmed animals. Further, ruminants must not be fed any animal protein or feedstuffs which contain animal protein except for milk, milk-based products and colostrum, eggs and egg products, gelatine from non-ruminants and hydrolysed proteins derived from non-ruminants or from ruminant hides and skins. Therefore, in accordance with the current ban, farmed deer should not be directly exposed to (i.e. feed) imported animal feed containing any PAP. Therefore, assuming this ban is adhered to correctly the risk of farmed deer being exposed to animal feed containing deer protein from North America is considered negligible but with associated uncertainty. ***However, given that non-ruminant feed produced in the USA may contain deer and elk PAP, it is theoretically possible that wild deer may be exposed to deer protein in legally imported non-ruminant feed. For this to occur, wild deer would need to access non-ruminant feed (e.g. pig, fish and chicken feed) on farms near their habitat. Alternatively, wild deer may be exposed to CWD prion in the faeces of pets that have consumed and digested imported, contaminated pet feed. ***The frequency in which these routes may occur is unknown and is considered to be a greater than negligible risk with associated uncertainty.

 

Movement of hunters, other tourists and British servicemen

 

The pathways by which naïve deer can be exposed to CWD contaminated soil and prions on equipment and clothing from people arriving to GB from North America are variable and highly uncertain. In principle, in order to expose a deer to CWD prions, the traveller (hunter, tourist or serviceman) would need to transfer the CWD prion from their clothing and/or equipment to the environment in which deer habit. The latter will depend upon the behaviours of returning GB residents or tourists and the probability of entering into and walking around deer territory. In GB, there are two main deer populations (wild and farmed

 

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or park deer) each of which will have differing risks of exposure given the type and frequency of contact with people. Each population type is considered in turn.

 

Wild deer

 

There are 6 species of wild deer residing in GB including: Red deer (Cervus elaphus), Roe deer (Capreolus capreolus), fallow deer (Dama dama), muntjac (Muntiacus reevesi), sika (Cervus nippon), and Chinese Water deer (Hydropotes inermis). The British Deer Society implemented a survey in 2007 to ascertain the distribution of these deer species across the UK. The survey provides the presence of deer on a standard template of 10km grid squares (www.bds.org.uk ). A further survey was conducted in 2011 of which the results will be published later in 2012. The deer distribution as ascertained from the 2007 survey is summarised in Figure 3.

 

It can be seen from Figure 3 (below) that deer are widely distributed across the UK with Roe deer being the most widespread. Chinese Water deer are the smallest deer population with approximately 700 deer.

 

Deer hunters, particularly, are most likely to be in direct contact with wild deer and their habitat compared to other tourists and returning GB residents. During the stalking and/or hunting of deer, there is opportunity for CWD prion on the hunter’s boots, clothing and/or equipment to be transferred to the environment. The amount transferred will depend upon the measures taken to remove soil etc from the equipment prior to stalking. Assuming that CWD prion is transferred to the environment, there is an uncertain probability that a deer will come into contact with the CWD prion.

 

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Figure 3: Distribution of the six deer species in the UK in 2007 (British Deer Society Deer Distribution Survey, http://www.bds.org.uk)

 

Farmed and park deer

 

Deer farming is a relatively recent enterprise. There are two systems currently used for managing enclosed deer: park and farm deer systems. In the park system, deer are raised in a park type setting and allowed to roam freely and may be provided with some supplemental feed. Farmed deer, in contrast, following conventional agricultural practices and may be housed in the winter and nutritional supplements are provided where necessary. In this farming system, there are several categories including calf rearers, calf finishers, breeder finishers and producer/processors (www.bdfpa.org). In 2011, according to the June Agricultural census, there were approximately 21,000 farmed deer on commercial agricultural holdings in England. The Economic Report on Scottish Agriculture (2011) cited that within Scotland, Wales, England and Northern Ireland there were 30,910 farmed deer. It is less likely that tourists, deer hunters and British servicemen will come into contact with conventionally farmed deer compared to park deer. The total park deer population in GB is unknown. However, in 2005, based on annual population control culling of about 8,000 animals, it was estimated that there were approximately 40,000 park deer. These deer are distributed across several parks (some of which are famous tourist

 

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sites) where wild and/or exotic species of deer can roam and be viewed. These are outlined in Table 2.

 

Table 2: Summary of parks in Great Britain where wild and/or exotic deer roam (The British Deer Society, www.bds.org.uk)

 

Country Park Species

 

England Ashton Court, Bristol Red, Fallow Bolderwood Deer Sanctuary, Minstead, Hampshire Fallow, Bradgate Park, Charnwood Forest, Leicestershire Fallow, Red Bushy Park, Hampton Hill, London Fallow, Red Chatsworth Park, Chatsworth, Derbyshire Fallow, Red Dunham Massey, Altrincham, Cheshire Dyrham Park, Chippenham, Wiltshire Fallow Grimsthorpe Castle Park and Gardens, Bourne, Lincolnshire Helmington Hall, Stowmarket, Suffolk Red Holkham Estate, Wells-next-the-Sea, Norfolk Fallow Knole Park, Sevenoaks, Kent Fallow, Sika Lodge Park & Sherborne Estate, Lyme Park, Disley, Cheshire Red, Fallow New Forest Wildlife Park, Longdown, Hampshire European Bison, Red deer Petworth Park, Petworth, Sussex Fallow Prinknash Deer and Bird Park, Cranham, Gloucester Raby Castle, Staindrop, County Durham Red, Fallow

 

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Richmond Park, Richmond, London Red, Fallow Snettisham Park Farm, King’s Lynn, Norfolk Red South West Deer Rescue and Study Centre, Wayford Red, White Red, Fallow, Axis, Roe, Japanese Sika Tatton Park, Knutsford, Cheshire Wentworth Castle, Stainborough Red, Fallow Wildwood Trust, Hern Bay, Kent Roe, Fallow, Red Woburn Abbey, Woburn Park, Bedfordshire Sika, Axis, Chital, Barasingha, Chinese Water, Rusa, Pere David Wollaton Park, Woolaton, Nottinghamshire Red, Fallow

 

Wales

 

Abergavenny Priory Deer Park, Abergavenny Dinefwr House, Kinefwr Park, Llandeilo, Carmarthenshire Fallow Margam Country Park, Port Talbot Red, Pere David, Chital, Hog, Barasingha, Roe, Muntjac, Chinese Water

 

Scotland

 

Beecraigs Country Park, Linlithgow, West Lothian Red Glengoulandie Deer Park, Aberfeldy, Perthshire Red Highland Wildlife Park, Kingussie, Inverness-shire Red, reindeer Jedforest Deer and Farm Park, Jedburgh The Scottish Deer Centre, Cupar, Fife Nine species of deer

 

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It is evident from Table 2 that there are several locations in GB where tourists and returning residents may come into contact with park deer and, in doing so, potentially expose the deer to CWD on their contaminated clothing and/or footwear. Further, given the volume of tourists and other travellers moving between North America and GB, there are potentially multiple opportunities for CWD prions to be transferred from clothing, boots and/or equipment to the environment. It has been observed that multiple exposures to low levels of CWD prions in the environment and increased infectivity of CWD when prions are bound to the soil are influential factors in transmission (Anger et al., 2009). Given the nature of their management, there is a restricted area (or environment) in which park deer inhabit enabling them to have a potentially higher probability of coming into contact with any CWD transferred to the environment by a tourist or returning GB resident compared to wild deer in a free-ranging environment. ***Therefore, it is considered that farmed and park deer may have a higher probability of exposure to CWD transferred to the environment than wild deer given the restricted habitat range and higher frequency of contact with tourists and returning GB residents.

 

Exposure of UK deer to infected urine

 

For the purpose of the qualitative risk assessment developed here it is necessary to estimate the probability that some of the contents (i.e. 10-ml) of each 30-ml bottle of deer urine lure is ingested by UK deer.

 

The degree of exposure depends on whether deer are likely to lick urine in the woods, and where the urine is placed by the hunter. Some hunters claim it is unlikely deer will lick the urine (Anon 2015a). The main risk reduction factor is the fact that deer roam over a very large area and female deer (those not attracted) would be unlikely to come in contact with it. The risk of exposure to female deer therefore may be relatively low. This raises the question of deer bucks which are attracted to the lure and whether they lick it. The aqueous solvent component of the urine will evaporate or soak into the ground. However, it is well known that TSE infectivity does not leach into the ground but attaches to soil particles, which could be eaten by grazing deer. ***Although the infectivity may be less bioavailable compared to a deer actually imbibing the contents of a 30-ml bottle of deer urine lure the exposure of single bucks attracted to the lure cannot be assumed to be negligible. ***Furthermore it cannot be assumed that the lured deer are shot and the deer lure may continue to attract deer for some time after the hunter/stalker has departed. It is not known how long the lure would work for. CWD infectivity will persist in the environment for long periods of time, and bind to the soil surface. ***Therefore, there is the possibility that the CWD infectivity in deer lure urine may persist for months, such that deer could be exposed at a later date. ***Overall therefore, given the fact that the primary use of a deer lure is to attract bucks, it is assumed here that the probability that some of the contents (i.e. 10-ml) of each 30-ml bottle of deer urine lure is ingested by UK deer is medium.

 

Given that a deer within GB is exposed to CWD bio-available prions in the environment, the probability of becoming infected is dependent upon the infectious dose and the susceptibility of the animal to the prion. The majority of research into CWD has been

 

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conducted in North America where it has been shown that the following species are naturally infected with CWD (Hamir et al., 2008):

 

 Mule deer (Odocoileus hemionus hemionus)

 

 Black-tailed deer (Odocoileus hemionus columbianus)

 

 White-tailed deer (Odocoileus virginianus)

 

 Rocky Mountain elk (Cervus elphus nelsoni)

 

 Shira’s moose (Alces alces shirasi)

 

None of these species are present in GB. However, EFSA (2010) considered that red deer (Cervus elaphus), a species present in GB (see Figure 3), is likely to be susceptible to CWD and was a species specifically targeted in the EU survey for CWD. This stems from the fact that red deer have a close genetic relationship to Rocky Mountain elk. This hypothesis has been supported by recent experimental studies that have demonstrated red deer become infected with CWD after oral inoculation of brain tissue from infected Rocky Mountain elk (Balachandran et al., 2010). Specifically, two of the four 2-month old red deer, showed clinical signs by 585 days p.i. and all deer had CWD prion in the brain, spinal cord and other organs at necropsy (Balachandran et al., 2010). Further, Martin et al., (2009) demonstrated in a similar study of four European red deer, that red deer can become infected upon inoculation of 5g of infected brain homogenate from four CWD elk and hence the species is susceptible to CWD.

 

Hamir et al., (2008) undertook a study to ascertain if fallow deer (Dama dama), another British deer species, could be experimentally infected with CWD brain suspension from infected elk or white-tailed deer. The authors concluded that it is possible to transmit CWD to fallow deer via the intracerebral route but the pathological features of CWD in the deer differs from those observed in white-tailed deer or elk (Hamir et al., 2008). It was further concluded that it might not be possible to transmit CWD via a more natural route or, alternatively, a higher dose of inoculum is required leading to a longer incubation period (Hamir et al., 2008).

 

Initial studies into the PRioN Protein (PRNP) gene variability in European red deer and roe deer suggest that these species have a PRNP genetic background that is compatible with TSE susceptibility, including CWD (EFSA, 2011). It is important to note, however, that no experimental studies on roe deer have been conducted verifying this hypothesis.

 

There are no data on the susceptibility of the other free-ranging deer species present in Britain (muntjac (Muntiacus reevesi), sika (Cervus nippon), Chinese Water deer (Hydropotes inermis)) to CWD. Further experimental studies would be required to investigate the susceptibility of these species to CWD. ***Therefore, on the basis of current scientific understanding, it is likely that given exposure to an infectious dose to CWD, deer in GB could become infected with CWD. Whether the amount of CWD prion that could be transferred from clothing, boots and/or other equipment into the deer’s environment is enough to induce infection given that the infectious dose is extremely small (Saunders et al., 2010) is uncertain. However, given that the amount of soil ingested is likely to be very small, the probability of ingesting an infectious dose via this route is considered negligible

 

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to very low. The probability of ingesting an infectious dose via consumption of non-ruminant feed is likely to be higher and may be very low, with associated uncertainty.

 

The CWD agent is relatively dilute in deer urine compared to brain and spinal cord material with 1 ic LD50 per 10 ml. The LD50 determined by Henderson et al. (2015) is an intracerebral ID50 in cervidized transgenic mice. The oral ID50 in deer would be in a much larger volume of urine, because the oral route may be 100,000-fold less efficient than the intracerebral route in terms of TSE transmission (Gale et al 1998). Thus, in terms of oral LD50, there may be one in a 1,000,000 ml i.e. 1 m3 or 1,000 litre volume of deer urine. Therefore a deer would have to ingest 1,000 litres of urine to have a 50% chance of being infected through the oral route. Of course the CWD agent would be concentrated as the urine evaporated from the tree trunk.

 

The main sources of uncertainty are:-

 

1. The amount of urine ingested by the deer. A bottle of Tink’s “69 Doe-in-Rut Buck” is about 30 ml and boasts 100% natural doe oestrous urine (Anon 2105d). A bottle from an infected doe would thus contain about 3 i.c. LD50 units. The bottles come with an easy to use squirt top, so it seems relatively small amounts are used. There are also gel forms which do not freeze as fast, last longer in the rain, and do not dry out so quickly.

 

2. The magnitude of oral/i.c. barrier

 

Assuming a deer drank 10 ml of urine from each 30 ml bottle, then that deer would ingest 10-5 LD5. It is generally assumed that there is no threshold dose for TSEs (Gale 2006) and the risk of CWD infection in GB deer per 30 ml bottle imported is therefore 0.69 x 10-5 (Gale 1998). This is very low.

 

Table 1: Risk of infection of deer in GB per 30 ml bottle of deer urine lure imported from the USA

 

Step in pathway Risk Uncertainty

 

Entry (probability a 30-ml bottle contains urine from an infected deer) Medium Low

 

Exposure (probability some of 30-ml bottle is ingested by UK deer) Medium High

 

Consequence (probability that deer is infected given exposure) Very low Medium

 

Overall risk Very low High

 

Overall the risk of a deer in GB being infected per 30 ml bottle of urine imported from the USA is very low, albeit with high uncertainty.

 

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The next question is how much deer urine is imported into GB from the USA per year. If there are n 30-ml bottles, then the risk is calculated as:-

 

pn_bottle = 1 - (1 – pone_bottle)n

 

Hundreds of gallons of urine are sold every year just by one company in the USA (Anon 2015b). It is difficult, however, to estimate the volume of deer urine imported into the UK per year from the USA. That some respondents to the BDS survey reported they used upwards of 1litre per year, is consistent with importation of a high number of 30-ml bottles. Assuming that the number, n, of 30-ml bottles imported annually is high, then the risk of CWD infection in at least one deer in the UK per year will be medium (given the probability, of infection of a UK deer from one 30-ml bottle is very low (see Table 1)).

 

***Overall it is concluded that the annual risk of at least one infection of deer in the UK with CWD from deer urine lures imported from the USA is medium. This assumes a high number of 30 ml bottles imported per year from all areas of the USA.

 

Control and risk management options

 

In order to reduce the potential amount of CWD prion entering GB on boots and clothing, it is important to meticulously clean off all adherent material prior to departing from North America. As CWD is a highly resistant agent, where possible, equipment should be soaked in a solution of bleach that has 20,000 parts per million of active chlorine for one hour. However, it is acknowledged it is impractical to soak hunting boots, clothing or firearms, for example, in strong concentrations of bleach.

 

For deer urine lures, the import of unprocessed natural deer urine is not allowed under the EU Animal By-Product legislation. However the import of such commodities including internet sales would still require licenses, but it would be a voluntary action on behalf of the importer to ensure the urine is sourced from safe herds. One option is to ensure the sourcing is from herds in States which are currently registered under the National CWD Herd Certification Programme which has recently been introduced in the USA. Otherwise the use of synthetic of European-produced lures should be promoted by the industry and stakeholder groups.

 

Conclusions

 

There is significant uncertainty associated with estimating the risk of CWD entering the UK via movement of people (tourists, hunters and British servicemen) and importation of animal feed. This partly stems from the lack of data on these two importation routes. Given this uncertainty, the probability of importing CWD into GB from North America and causing infection in British deer is likely to be negligible to very low via movement of deer hunters, other tourists and British servicemen and very low via imported (non-ruminant) animal feed.

 

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***However the risk of natural deer urine lures from the USA containing CWD PrP is considered high (reflected by some US States banning the use) and the probability of such a commodity, if used in significant volumes, leading to CWD infection in GB populations is considered to be medium (lack of susceptibility in certain species and limited use by hunters and stalkers in GB) but with a high level of uncertainty.

 

The consequences of CWD, however, are severe with the minimal possibility of eradicating the disease from a wild cervid population and populations of wild and farmed deer would be under threat.

 

Current research indicates that of the six free-ranging deer species in the UK, red deer are susceptible to CWD. This deer species is concentrated in distinct areas of the country (e.g. North of Scotland) and one of the key species which hunters, in particular, stalk. It is important, therefore, that the risk of this species being exposed to CWD is minimised by taking appropriate precautionary measures.

 

References

 

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Dalgleish, M.P., Martin, S., Steele, P., Finlayson, J., Siso, S., Hamilton, S., Chianini, F., Reid, H.W., Gonzalez, L., & Jeffrey, M. (2008) Experimental transmission of bovine spongiform encephalopathy to European red deer (Cervus elaphus elaphus). BMC Veterinary Research, 4, 17. EFSA (2004) Annex to the EFSA Journal (2004) 70 On the Opinion on a surveillance programme for Chronic Wasting Disease in the EU. EFSA Panel on Biological Hazards. (2011) Joint Scientific Opinion on any possible epidemiological or molecular association between TSEs in animals and humans. EFSA Journal, 9(1), 1945. EFSA Panel on Biological Hazards. (2010). Scientific Opinion on the results of the EU survey for Chronic Wasting Disease (CWD) in cervids. EFSA Journal, 8(10), 1861. Gale, P. (1998) Quantitative BSE risk assessment: relating exposures to risk. Letters in Applied Microbiology, 27, 239-242. Gale, P., Young, C., Stanfield, G. & Oakes, D. (1998) Development of a risk assessment for BSE in the aquatic environment. Journal of Applied Microbiology, 84, 467-477. Gale, P. (2006) The infectivity of transmissible spongiform encephalopathy agent at low doses: the importance of phospholipids. Journal of Applied Microbiology, 101, 261-274. Georgsson, G., Sigurdarson, S., & Brown, P. (2006) Infectious agent of sheep scrapie may persist in the environment for at least 16 years. Journal of General Virology, 87, 3737-3740. Gross, J.E. & Miller, m.W. (2001) Chronic wasting disease in mule deer: disease dynamics and control. Journal of Wildlife Management, 65, 2001. Haley, N.J., Seelig, D.M., Zabel, M.D., Telling, G.C., & Hoover, E.A. (2009) Detection of CWD prions in urine and saliva of deer by transgenic mouse bioassay. PLoS One, 4(3), e4848. Hamir, A.N., Kunkle, R.A., Nicholson, E.M., Miller, J.M., Hall, S.M., Schoenenbruecher, H., Brunelle, B.W., & Richt, J.A. (2008) Preliminary observations on the experimental transmission of chronic wasting disease (CWD) from elk and white-tailed deer to fallow deer. Journal of Comparative Pathology, 138, 121-30. Hamir, A.N., Kunkle, R.A., Miller, J.M., Greenlee, J.J., & Richt, J.A. (2006) Experimental second passage of chronic wasting disease (CWDmule deer) agent to cattle. J. Comp. Path, 134, 67-73. Hartley, M., Voller, F., Murray, T. & Roberts, H. (2012) Qualitative veterinary risk assessment of the role of wild deer in the likelihood of incursion and the impact on effective disease control of selected exotic notifiable diseases in England. Eur J. Wildl Res DOI 10.1.1007/s10344-012-0647-7.

 

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Henderson, D.M., Davenport, K.A., Haley, N.J., Denkers, N.D., Mathiason, C.K. & Hoover, E.A. (2015) Quantitative assessment of prion infectivity in tissues and body fluids by real time quaking-induced conversion. Journal fo General Virology 96: 210-219. Johnson, C.J., Philips, K.E., Schramm, P.T., McKenzie, D., Aiken, J.M., & Pedersen, J.A. (2006) Prions adhere to soil minerals and remain infectious. PLoS Pathogens, 2 (4), e32. Johnson, C.J., Pedersen, J.A., Chappell, R.J., McKenzie, D., & Aiken, J.M. (2007) Oral transmissibility of prion disease is enhanced by binding to soil particles. PLoS Pathogens, 3 (7), e93. Martin, S., Jeffrey, M., Gonzalez, L., Siso, S., Reid, H.W., Steele, P., Dagleish, M.P., Stack, M.J., Chaplin, M.J., & Balachandran, A. (2009) Immunohistochemical and biochemical characteristics of BSE and CWD in experimentally infected European red deer (Cervus elaphus elaphus). BMC Veterinary Research, 5:26. Mathison, C,K., et al., (2006) Infectious prions in the saliva and blood of deer with chronic wasting disease. Science, 314, 133-136. Miller, M.W., Wild, M.A., & Williams, E.S. (1998) Epidemiology of chronic wasting disease in captive rocky mountain elk. Journal of Wildlife Diseases, 34 (3), 532-538. Miller, M.W., Williams, E.S., Hobbs, N.T., & Wolfe, L.L. (2004) Environmental sources of prion transmission in mule deer. Emerging Infectious Diseases, 10(6), 1003-1006. Nichols, T.A., Pulford, B., Wyckoff, A.C., Meyerett, C., Michel, B., Gertig, K., Hoover, E.A., Jewell, J.E., Telling, G.C., & Zabel, M.D. (2009) Detection of protease-resistant cervid protein in water from a CWD-endemic area. Prion, 3, 171-183. OIE, (2004). Handbook on import risk analysis for animals and animal products. World Organisation for Animal Health, 12, rue de Prony, 75017 Paris, France, p.59. Rural & Environmental Science & Analytical Services (2011). Economic Report on Scottish Agriculture 2011 Edition. Scottish Government: Edinburgh. Russo, F., Johnson, C.J., Johnson, C.J., McKenzie, D., Aiken, J.M., & Pedersen, J.A. (2009) Pathogenic prion protein is degraded by manganese oxide material found in soils. Journal of General Virology, 90, 275-280. Safar, J.G., Lessard, P., Tamguney, G., Freyman, V., Deering, C., Letessier, F., DeArmond, S.J., & Prusiner, S.B. (2008) Transmission and detection of prions in feces. Journal of Infectious Diseases, 198, 81-89. Saunders, S.E., Bartz, J.C., Vercauteren, K.C., & Bartelt-Hunt, S.L. (2010) Enzymatic digestion of chronic wasting disease prions bound to soil. Environ. Sci. Technol., 44, 4129-4135.

 

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Seidel, B., Thomzig, A., Buschmann, A., Groschup, M.H., Peters, R., Beekes, M., & Terytze, K., (2007) Scrapie agent (strain 263K) can transmit disease via the oral route after persistence in soil over years. PLoS Pathogens, 5, e435. Sigurdson, C.J. (2008) A prion disease of cervids: Chronic wasting disease. Veterinary Research, 39, 41. Strauser, K. (2014) Detection of Urine-Based Deer Lures to Mitigate CWD Transmission in Pennsylvania Keystone Journal of Undergraduate Research 2(1): 1-7. 2014 Tamguney, G., Miller, M.W., Wolfe, L.L., Sirochman, T.M., Glidden, D.V., Palmer, C., Lemus, A., DeArmond, S.J. & Prusiner, S.B. (2009) Asymptomatic deer excrete infectious prions in faeces. Nature, 461, 529-590. TAMS (2006) Travel activities and motivations of Canadian residents: An overview. Ministry of Tourism. TAMS (2007) Travel activities and motivations of U.S. residents: An overview. Ministry of Tourism. Trifilo, M.J., Ying, G., Teng, C. & Oldstone, M.B.A. (2007) Chronic wasting disease of deer and elk in transgenic mice: oral transmission and pathobiology. Vaccine, 365, 136-143. U.S. Fish & Wildlife Service (2011). Deer hunting in the United States: Demographics and Trends. Addendum to the 2006 National Survey of Fishing, Hunting, and Wildlife-associated recreation. Report 2006–10. Wildlife and Sport Fish Restoration: Arlington, VA. USGS (2016) Chronic Wasting Disease (CWD) http://www.nwhc.usgs.gov/disease_information/chronic_wasting_disease/ Accessed 05/04/2016. Williams, E.S. (2005) Chronic wasting disease. Vet Pathol., 42, 530-549. Williams, E.S., & Miller, M.W. (2003) Transmissible spongiform encephalopathies in non-domestic animals: origin, transmission and risk factors. Rev. Sci. Tech. Off. Int Epiz., 22, 145-156. Wilkinson, D.M. (2010) Have we underestimated the importance of humans in the biogeography of free-living terrestrial microorganisms? Journal of Biogeography, 37, 393-397.

 


 

What is the risk of chronic wasting disease being introduced into Great Britain? An updated Qualitative Risk Assessment March 2016

 


 

 From: Terry S. Singeltary Sr.

 

Sent: Thursday, April 07, 2016 1:02 PM

 

To: psi@nationalarchives.gsi.gov.uk

 

Cc: helen.roberts@apha.gsi.gov.uk ; jan.opgenoorth@efsa.europa.eu ; Press@efsa.europa.eu ; SCER.PublicConsult.55@efsa.europa.eu ; scer@efsa.europa.eu ; publicmeetings@efsa.europa.eu ; Gemma.Smith@wales.gsi.gov.uk

 

BCC: ...

 

Subject: What is the risk of chronic wasting disease being introduced into Great Britain? 6 April 2016

 

Greetings DEFRA MAFF, EFSA, Welsh Government et al,

 

I kindly wish to again the urgent need to address CWD in the USA and risk factors to the world there from.

 

I see ‘What is the risk of chronic wasting disease being introduced into Great Britain?’ was published recently 6 April 2016 and would kindly like to send this additional information.

 

What is the risk of chronic wasting disease being introduced into Great Britain? An updated Qualitative Risk Assessment March 2016

 


 

Since my last correspondence with you, CWD has exploded in new states, with Arkansas topping them all. Texas is reluctant to report in a timely manner, just how bad CWD is in the captive industry, but of course, Texas did the same thing with mad cow disease, covering up the last case for 7 months before it took an act of Congress to finally have that sample retested, which did confirm the mad cow, all this documented on the internet. with that said, I do not have any hope that CWD surveillance and timely reporting to the public will be any different. In two months Arkansas went from a few cases to a total of 56 cases in just two months, from a very small sample survey. also, I have pleaded with the FDA et al to please close the damn mad cow feed loop hole for cervids. FDA has finally readdressed the old Docket, updated it in March 2016, HOWEVER IT’S NON-BINDING, STILL NOTHING BUT INK ON PAPER. things don’t look good here at all. cwd is out of control, and corporate America is and will continue to let cwd spread further. as you all well know, our politicians are too worried about who’s ‘richard’ is bigger than the others, while Rome burns. I am embarrassed about our country and it’s politicians, so I shall apologize to the world for the sheer stupidity of it all. ...

 

I wish to kindly give you an update on this CWD TSE Prion situation here in the USA.

 

Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed Singeltary Submission

 

*** URGENT UPDATE MARCH 2016 OF THIS OLD DOCKET ***

 

however, it’s still non-binding $$$

 

insane!!! imo...

 

Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed Singeltary Submission

 

Greetings again FDA and Mr. Pritchett et al,

 

I would kindly like to comment on ;

 

Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed Singeltary Submission

 

#158

 

Guidance for Industry

 

Use of Material from Deer and Elk in Animal Feed

 

This version of the guidance replaces the version made available September15, 2003.

 

This document has been revised to update the docket number, contact information, and standard disclosures. Submit comments on this guidance at any time.

 

Submit electronic comments to http://www.regulations.gov. Submit written comments to the Division of Dockets Management (HFA-305), Food and Drug Administration, 5630 Fishers Lane, Rm. 1061, Rockville, MD 20852. All comments should be identified with the Docket No. FDA-2003-D-0432 (formerly 03D-0186).

 

For further information regarding this guidance, contact Burt Pritchett, Center for Veterinary Medicine (HFV-222), Food and Drug Administration, 7519 Standish Place, Rockville, MD 20855, 240-402-6276, E-mail: burt.pritchett@fda.hhs.gov.

 

Additional copies of this guidance document may be requested from the Policy and Regulations Staff (HFV-6), Center for Veterinary Medicine, Food and Drug Administration, 7519 Standish Place, Rockville, MD 20855, and may be viewed on the Internet at either http://www.fda.gov/AnimalVeterinary/default.htm or http://www.regulations.gov.

 

U.S. Department of Health and Human Services Food and Drug Administration Center for Veterinary Medicine March 2016

 

Contains Nonbinding Recommendations

 

2

 

Guidance for Industry Use of Material from Deer and Elk in Animal Feed

 

This guidance represents the current thinking of the Food and Drug Administration (FDA or Agency) on this topic. It does not establish any rights for any person and is not binding on FDA or the public. You can use an alternative approach if it satisfies the requirements of the applicable statutes and regulations. To discuss an alternative approach, contact the FDA office responsible for this guidance as listed on the title page.

 

I. Introduction

 

Under FDA’s BSE feed regulation (21 CFR 589.2000) most material from deer and elk is prohibited for use in feed for ruminant animals. This guidance document describes FDA’s recommendations regarding the use in all animal feed of all material from deer and elk that are positive for Chronic Wasting Disease (CWD) or are considered at high risk for CWD. The potential risks from CWD to humans or non-cervid animals such as poultry and swine are not well understood. However, because of recent recognition that CWD is spreading rapidly in white-tailed deer, and because CWD’s route of transmission is poorly understood, FDA is making recommendations regarding the use in animal feed of rendered materials from deer and elk that are CWD-positive or that are at high risk for CWD.

 

In general, FDA’s guidance documents do not establish legally enforceable responsibilities. Instead, guidances describe the Agency’s current thinking on a topic and should be viewed only as recommendations, unless specific regulatory or statutory requirements are cited. The use of the word should in Agency guidances means that something is suggested or recommended, but not required.

 

II. Background

 

CWD is a neurological (brain) disease of farmed and wild deer and elk that belong in the animal family cervidae (cervids). Only deer and elk are known to be susceptible to CWD by natural transmission. The disease has been found in farmed and wild mule deer, white-tailed deer, North American elk, and in farmed black-tailed deer. CWD belongs to a family of animal and human diseases called transmissible spongiform encephalopathies (TSEs). These include bovine spongiform encephalopathy (BSE or “mad cow” disease) in cattle; scrapie in sheep and goats; and classical and variant Creutzfeldt-Jakob diseases (CJD and vCJD) in humans. There is no known treatment for these diseases, and there is no vaccine to prevent them. In addition, although validated postmortem diagnostic tests are available, there are no validated diagnostic tests for CWD that can be used to test for the disease in live animals.

 

Contains Nonbinding Recommendations

 

III. Use in animal feed of material from CWD-positive deer and elk

 

Material from CWD-positive animals may not be used in any animal feed or feed ingredients. Pursuant to Sec. 402(a)(5) of the Federal Food, Drug, and Cosmetic Act, animal feed and feed ingredients containing material from a CWD-positive animal would be considered adulterated. FDA recommends that any such adulterated feed or feed ingredients be recalled or otherwise removed from the marketplace.

 

IV. Use in animal feed of material from deer and elk considered at high risk for CWD Deer and elk considered at high risk for CWD include: (1) animals from areas declared by State officials to be endemic for CWD and/or to be CWD eradication zones; and (2) deer and elk that at some time during the 60-month period immediately before the time of slaughter were in a captive herd that contained a CWD-positive animal.

 

FDA recommends that materials from deer and elk considered at high risk for CWD no longer be entered into the animal feed system. Under present circumstances, FDA is not recommending that feed made from deer and elk from a non-endemic area be recalled if a State later declares the area endemic for CWD or a CWD eradication zone. In addition, at this time, FDA is not recommending that feed made from deer and elk believed to be from a captive herd that contained no CWD-positive animals be recalled if that herd is subsequently found to contain a CWD-positive animal.

 

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.

 

3

 


 

Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed Singeltary Submission

 

Greetings again FDA and Mr. Pritchett et al,

 

MY comments and source reference of sound science on this very important issue are as follows ;

 

Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed Singeltary Submission

 

I kindly wish to once again submit to Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed.

 

Thank you kindly for allowing me to comment again, ...and again...and again, on a topic so important, why it is ‘NON-BINDING’ is beyond me.

 

this should have been finalized and made ‘BINDING’ or MANDATORY OVER A DECADE AGO.

 

but here lay the problem, once made ‘BINDING’ or ‘MANDATORY’, it is still nothing but ink on paper.

 

we have had a mad cow feed ban in place since August 1997, and since then, literally 100s of millions of pounds BANNED MAD COW FEED has been sent out to commerce and fed out (see reference materials).

 

ENFORCEMENT OF SAID BINDING REGULATIONS HAS FAILED US TOO MANY TIMES.

 

so, in my opinion, any non-binding or voluntary regulations will not work, and to state further, ‘BINDING’ or MANDATORY regulations will not work unless enforced.

 

with that said, we know that Chronic Wasting Disease CWD TSE Prion easily transmits to other cervid through the oral route.

 

the old transmission studies of BSE TSE floored scientist once they figured out what they had, and please don’t forget about those mink that were fed 95%+ dead stock downer cow, that all came down with TME. please see ;

 

*** 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 what the UK GOV warns about this;

 


 

Saturday, April 02, 2016

 

TEXAS TAHC BREAKS IT'S SILENCE WITH TWO MORE CASES CWD CAPTIVE DEER BRINGING TOTAL TO 10 CAPTIVES REPORTED TO DATE

 


 

Friday, February 26, 2016

 

TEXAS Hartley County Mule Deer Tests Positive for Chronic Wasting Disease CWD TSE Prion

 


 

Division of Ag researchers move ahead determining prevalence, distribution of Chronic Wasting Disease in state deer, elk By Ryan McGeeney U of A System Division of Agriculture April 1, 2016

 

Fast Facts:

 

CWD detected in deer in Newton County

 

Experts working to determine prevalence CWD not transmissible between species (732 words)

 

LITTLE ROCK — Researchers with the University of Arkansas System Division of Agriculture said this week that 18 out of 48 white-tailed deer tested in Newton County in March were found to have Chronic Wasting Disease, also known as CWD.

 

Don White, Jr., professor and wildlife ecologist for the Division of Agriculture, said that number was probably not representative of the disease’s actual prevalence in the state’s wildlife herds.

 

“Those samples we had contained what’s known as ‘focal sampling,’” White said. “Those animals were either road-killed or seemed to display the symptoms of CWD, so it’s not random, it’s not representative of the whole.”

 

White said the samples were collected by Arkansas Game and Fish Commission biologists.

 

CWD is a transmissible form of spongiform encephalopathy, which kills members of the deer family by altering proteins known as prions in the animals’ brains. Symptoms include weight loss to the point of emaciation, excessive salivation, loss of coordination and death. White said there is currently no vaccine for the disease, which results in 100 percent fatality.

 

White and he and Game and Fish Commission biologists had collected sample tissue from 265 deer within the Newton County surveillance zone, harvesting tissue post-mortem from the animals’ brain stem and lymph nodes, areas that tend to accumulate the proteins that cause CWD. The samples are being sent in batches to a veterinarian laboratory in Madison, Wis., which takes about 10-14 days to analyze them, White said.

 

Once all of the results from the 265 deer are available, prevalence of CWD in the population of deer within the surveillance zone can be determined. Once prevalence is known, researchers will begin working to determine the distribution of CWD in the general area where it has been detected.

 

By establishing the distribution of the disease, White said, researchers can begin to address areas where the disease is concentrated, which may require looking at some human practices, as well.

 

“Some researchers have seen a relationship between the presence of CWD and the density of the deer herd,” White said. “So the more animals per unit of space, the greater the prevalence of CWD. One approach may be to reduce deer density, and keep it low in those CWD-positive areas.

 

“CWD is transmitted from deer to deer via body fluids such as saliva, urine, and feces. Because deer feeding and baiting concentrate deer into small areas, perhaps we will need to address the issue of feeding and baiting in Arkansas,” he said. “But we really can’t develop a management approach to CWD until we know prevalence rates and the distribution of the disease in the state.”

 

Although various animals, and even humans, host their own specific versions of a transmissible spongiform encephalopathy (in humans, it is known as Creutzfeldt-Jacob Disease), the respective diseases aren’t transmissible between species.

 

Additionally, humans cannot contract CWD by ingesting mean from an infected deer.

 

“People in CWD-positive areas in Colorado, Wyoming, and Wisconsin have undoubtedly consumed millions of tons of venison over the past few decades,” White said. “A human has never contracted a TSE by eating deer or elk meat.”

 

Nevertheless, there are carcass-handling guidelines available for hunters concerned with coming into contact with animals that may be carrying CWD. Becky McPeake, professor and extension wildlife specialist for the Division of Agriculture, is currently finalizing a publication that outlines basic safety precautions, which include:

 

Do not harvest animals exhibiting clinical signs of CWD or any other disease. When processing harvested game, hunters should wear gloved protection, and avoid contact with nervous system tissues. Don’t consume brain or organ meats, especially lymph nodes of the head of cervids. Bone out the meat, and make sure to minimize contact with the brain and spine, as they constitute the bulk of the nervous system where prions tend to concentrate. Dispose of all non-consumable parts securely and in a location where other cervids will not encounter the carcass (such as an approved landfill or buried at a depth of at least 6’). Cleaning processing equipment in a 50% chlorine bleach solution will destroy prions, but is very detrimental to most equipment. Standard cleaning with hot soapy water is typically sufficient, given the limited health risks to humans. Wipe down processing surfaces with the same solution on a clean cloth. For more information on Chronic Wasting Disease or other animal health issues, contact your local Cooperative Extension Service agent or visit www.uaex.edu.

 

The University of Arkansas System Division of Agriculture offers all its Extension and Research programs and services without regard to race, color, sex, gender identity, sexual orientation, national origin, religion, age, disability, marital or veteran status, genetic information, or any other legally protected status, and is an Affirmative Action/Equal Opportunity Employer.

 

# # #

 

Media Contact: Mary Hightower Dir. of Communication Services U of A Division of Agriculture Cooperative Extension Service (501) 671-2126 mhightower@uaex.edu

 


 

Friday, April 01, 2016

 

ARKANSAS CHRONIC WASTING DISEASE CWD TSE PRION CASES EXPLODE BY 27 NEW CASES WITH 50 CASES TOTAL TO DATE

 


 

Friday, April 01, 2016

 

Arkansas confirms six more cases of CWD bringing total to 56 since first reported 2 months ago

 


 

Tuesday, March 29, 2016

 

Maryland Department of Natural Resources Five Deer Test Positive for Chronic Wasting Disease ONE OUTSIDE CWD MANAGEMENT ZONE

 


 

Friday, March 18, 2016

 

Michigan confirms additional CWD-positive free-ranging, white-tailed deer, bringing the total to seven

 


 

Wednesday, March 16, 2016

 

Wisconsin CWD sample survey 2015 confirms 290 cases of Chronic Wasting Disease TSE Prion

 


 

Thursday, March 10, 2016

 

WYOMING RIDE EM COWBOY HELICOPTER WRANGLING RAMBO STYLE DEER BULLDOGGING RODEO FOR CWD VIDEO

 

CHRONIC WASTING DISEASE: The Final Epidemic

 


 

Tuesday, March 08, 2016

 

Oklahoma Chronic Wasting Disease CWD of Deer and Elk Surveillance, Testing, and Preparedness ???

 


 

Wednesday, March 02, 2016

 

Kansas Chronic Wasting Disease CWD TSE Prion 52 cases 2015 updated report 'ALARMING'

 


 

Sunday, March 06, 2016

 

Missouri 2015-2016 CWD Surveillance Summary to Date, with confirmed cases mounting

 


 

Thursday, March 31, 2016

 

*** Chronic Wasting Disease CWD TSE Prion Roundup USA April 1, 2016 ***

 


 

I strenuously once again urge the FDA and its industry constituents, to make it MANDATORY that all ruminant feed be banned to all ruminants, and this should include all cervids, as well as non-ruminants such as cats and dogs as well, as soon as possible for the following reasons...

 

31 Jan 2015 at 20:14 GMT

 

*** Ruminant feed ban for cervids in the United States? ***

 

31 Jan 2015 at 20:14 GMT

 

see Singeltary comment ;

 


 

*** 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

 


 

*** 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

 


 


 

Evidence That Transmissible Mink Encephalopathy Results from Feeding Infected Cattle Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.

 

snip...

 

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

 


 


 


 

Monday, April 04, 2016

 

Limited amplification of chronic wasting disease prions in the peripheral tissues of intracerebrally inoculated cattle

 


 

Saturday, February 6, 2016

 

*** Secretary's Advisory Committee on Animal Health; Meeting [Docket No. APHIS-2016-0007] Singeltary Submission

 


 

RE-What is the risk of chronic wasting disease being introduced into Great Britain? 6 April 2016

 


 

What is the risk of chronic wasting disease being introduced into Great Britain? A Qualitative Risk Assessment October 2012

 


 

I strenuously once again urge the FDA and its industry constituents, to make it MANDATORY that all ruminant feed be banned to all ruminants, and this should include all cervids, as well as non-ruminants such as cats and dogs as well, as soon as possible for the following reasons...

 

31 Jan 2015 at 20:14 GMT

 

*** Ruminant feed ban for cervids in the United States? ***

 

31 Jan 2015 at 20:14 GMT

 

Terry Singeltary Sr. comment ;

 


 

*** 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

 


 

Monday, March 28, 2016

 

National Scrapie Eradication Program February 2016 Monthly Report

 


 

Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed Singeltary Submission

 

Greetings again FDA and Mr. Pritchett et al,

 

I would kindly like to comment on ;

 

Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed Singeltary Submission

 

#158

 

Guidance for Industry

 

Use of Material from Deer and Elk in Animal Feed

 

This version of the guidance replaces the version made available September15, 2003.

 

This document has been revised to update the docket number, contact information, and standard disclosures. Submit comments on this guidance at any time.

 

Submit electronic comments to http://www.regulations.gov. Submit written comments to the Division of Dockets Management (HFA-305), Food and Drug Administration, 5630 Fishers Lane, Rm. 1061, Rockville, MD 20852. All comments should be identified with the Docket No. FDA-2003-D-0432 (formerly 03D-0186).

 

For further information regarding this guidance, contact Burt Pritchett, Center for Veterinary Medicine (HFV-222), Food and Drug Administration, 7519 Standish Place, Rockville, MD 20855, 240-402-6276, E-mail: burt.pritchett@fda.hhs.gov.

 

Additional copies of this guidance document may be requested from the Policy and Regulations Staff (HFV-6), Center for Veterinary Medicine, Food and Drug Administration, 7519 Standish Place, Rockville, MD 20855, and may be viewed on the Internet at either http://www.fda.gov/AnimalVeterinary/default.htm or http://www.regulations.gov.

 

U.S. Department of Health and Human Services Food and Drug Administration Center for Veterinary Medicine March 2016

 

Contains Nonbinding Recommendations

 

2

 

Guidance for Industry Use of Material from Deer and Elk in Animal Feed

 

This guidance represents the current thinking of the Food and Drug Administration (FDA or Agency) on this topic. It does not establish any rights for any person and is not binding on FDA or the public. You can use an alternative approach if it satisfies the requirements of the applicable statutes and regulations. To discuss an alternative approach, contact the FDA office responsible for this guidance as listed on the title page.

 

I. Introduction

 

Under FDA’s BSE feed regulation (21 CFR 589.2000) most material from deer and elk is prohibited for use in feed for ruminant animals. This guidance document describes FDA’s recommendations regarding the use in all animal feed of all material from deer and elk that are positive for Chronic Wasting Disease (CWD) or are considered at high risk for CWD. The potential risks from CWD to humans or non-cervid animals such as poultry and swine are not well understood. However, because of recent recognition that CWD is spreading rapidly in white-tailed deer, and because CWD’s route of transmission is poorly understood, FDA is making recommendations regarding the use in animal feed of rendered materials from deer and elk that are CWD-positive or that are at high risk for CWD.

 

In general, FDA’s guidance documents do not establish legally enforceable responsibilities. Instead, guidances describe the Agency’s current thinking on a topic and should be viewed only as recommendations, unless specific regulatory or statutory requirements are cited. The use of the word should in Agency guidances means that something is suggested or recommended, but not required.

 

II. Background

 

CWD is a neurological (brain) disease of farmed and wild deer and elk that belong in the animal family cervidae (cervids). Only deer and elk are known to be susceptible to CWD by natural transmission. The disease has been found in farmed and wild mule deer, white-tailed deer, North American elk, and in farmed black-tailed deer. CWD belongs to a family of animal and human diseases called transmissible spongiform encephalopathies (TSEs). These include bovine spongiform encephalopathy (BSE or “mad cow” disease) in cattle; scrapie in sheep and goats; and classical and variant Creutzfeldt-Jakob diseases (CJD and vCJD) in humans. There is no known treatment for these diseases, and there is no vaccine to prevent them. In addition, although validated postmortem diagnostic tests are available, there are no validated diagnostic tests for CWD that can be used to test for the disease in live animals.

 

Contains Nonbinding Recommendations

 

III. Use in animal feed of material from CWD-positive deer and elk

 

Material from CWD-positive animals may not be used in any animal feed or feed ingredients. Pursuant to Sec. 402(a)(5) of the Federal Food, Drug, and Cosmetic Act, animal feed and feed ingredients containing material from a CWD-positive animal would be considered adulterated. FDA recommends that any such adulterated feed or feed ingredients be recalled or otherwise removed from the marketplace.

 

IV. Use in animal feed of material from deer and elk considered at high risk for CWD Deer and elk considered at high risk for CWD include: (1) animals from areas declared by State officials to be endemic for CWD and/or to be CWD eradication zones; and (2) deer and elk that at some time during the 60-month period immediately before the time of slaughter were in a captive herd that contained a CWD-positive animal.

 

FDA recommends that materials from deer and elk considered at high risk for CWD no longer be entered into the animal feed system. Under present circumstances, FDA is not recommending that feed made from deer and elk from a non-endemic area be recalled if a State later declares the area endemic for CWD or a CWD eradication zone. In addition, at this time, FDA is not recommending that feed made from deer and elk believed to be from a captive herd that contained no CWD-positive animals be recalled if that herd is subsequently found to contain a CWD-positive animal.

 

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.

 

3

 


 

Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed Singeltary Submission

 

Greetings again FDA and Mr. Pritchett et al,

 

MY comments and source reference of sound science on this very important issue are as follows ;

 

Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed Singeltary Submission

 

I kindly wish to once again submit to Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed.

 

Thank you kindly for allowing me to comment again, ...and again...and again, on a topic so important, why it is ‘NON-BINDING’ is beyond me.

 

this should have been finalized and made ‘BINDING’ or MANDATORY OVER A DECADE AGO.

 

but here lay the problem, once made ‘BINDING’ or ‘MANDATORY’, it is still nothing but ink on paper.

 

we have had a mad cow feed ban in place since August 1997, and since then, literally 100s of millions of pounds BANNED MAD COW FEED has been sent out to commerce and fed out (see reference materials).

 

ENFORCEMENT OF SAID BINDING REGULATIONS HAS FAILED US TOO MANY TIMES.

 

so, in my opinion, any non-binding or voluntary regulations will not work, and to state further, ‘BINDING’ or MANDATORY regulations will not work unless enforced.

 

with that said, we know that Chronic Wasting Disease CWD TSE Prion easily transmits to other cervid through the oral route.

 

the old transmission studies of BSE TSE floored scientist once they figured out what they had, and please don’t forget about those mink that were fed 95%+ dead stock downer cow, that all came down with TME. please see ;

 

It is clear that the designing scientists must also have shared Mr Bradleys surprise at the results because all the dose levels right down to 1 gram triggered infection.

 


 

it is clear that the designing scientists must have also shared Mr Bradleys surprise at the results because all the dose levels right down to 1 gram triggered infection.

 


 

Evidence That Transmissible Mink Encephalopathy Results from Feeding Infected Cattle

 

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

 

snip...

 

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

 


 

To further complicate things, we now know that science has shown that plants and vegetables can uptake the TSE Prion, and that the Scrapie agent can still be infectious from soil 16 years later. a frightening thought with the CWD running rampant now in North America (please see source reference materials below).

 

IF we don’t not do this, we have failed, and the TSE Prion agent will continue to spread, as it is doing as we speak.

 

I strenuously once again urge the FDA and its industry constituents, to make it MANDATORY that all ruminant feed be banned to all ruminants, and this should include all cervids, as well as non-ruminants such as cats and dogs as well, as soon as possible for the following reasons...

 

31 Jan 2015 at 20:14 GMT

 

*** Ruminant feed ban for cervids in the United States? ***

 

31 Jan 2015 at 20:14 GMT

 

see Singeltary comment ;

 


 

Friday, December 14, 2012

 

DEFRA U.K. What is the risk of Chronic Wasting Disease CWD being introduced into Great Britain? A Qualitative Risk Assessment October 2012

 

snip...

 

In the USA, under the Food and Drug Administration’s BSE Feed Regulation (21 CFR 589.2000) most material (exceptions include milk, tallow, and gelatin) from deer and elk is prohibited for use in feed for ruminant animals. With regards to feed for non-ruminant animals, under FDA law, CWD positive deer may not be used for any animal feed or feed ingredients. For elk and deer considered at high risk for CWD, the FDA recommends that these animals do not enter the animal feed system. However, this recommendation is guidance and not a requirement by law.

 

Animals considered at high risk for CWD include:

 

1) animals from areas declared to be endemic for CWD and/or to be CWD eradication zones and

 

2) deer and elk that at some time during the 60-month period prior to slaughter were in a captive herd that contained a CWD-positive animal.

 

Therefore, in the USA, materials from cervids other than CWD positive animals may be used in animal feed and feed ingredients for non-ruminants.

 

The amount of animal PAP that is of deer and/or elk origin imported from the USA to GB can not be determined, however, as it is not specified in TRACES. It may constitute a small percentage of the 8412 kilos of non-fish origin processed animal proteins that were imported from US into GB in 2011.

 

Overall, therefore, it is considered there is a __greater than negligible risk___ that (nonruminant) animal feed and pet food containing deer and/or elk protein is imported into GB.

 

There is uncertainty associated with this estimate given the lack of data on the amount of deer and/or elk protein possibly being imported in these products.

 

snip...

 

36% in 2007 (Almberg et al., 2011). In such areas, population declines of deer of up to 30 to 50% have been observed (Almberg et al., 2011). In areas of Colorado, the prevalence can be as high as 30% (EFSA, 2011). The clinical signs of CWD in affected adults are weight loss and behavioural changes that can span weeks or months (Williams, 2005). In addition, signs might include excessive salivation, behavioural alterations including a fixed stare and changes in interaction with other animals in the herd, and an altered stance (Williams, 2005). These signs are indistinguishable from cervids experimentally infected with bovine spongiform encephalopathy (BSE). Given this, if CWD was to be introduced into countries with BSE such as GB, for example, infected deer populations would need to be tested to differentiate if they were infected with CWD or BSE to minimise the risk of BSE entering the human food-chain via affected venison.

 

snip...

 

The rate of transmission of CWD has been reported to be as high as 30% and can approach 100% among captive animals in endemic areas (Safar et al., 2008).

 

snip...

 

In summary, in endemic areas, there is a medium probability that the soil and surrounding environment is contaminated with CWD prions and in a bioavailable form. In rural areas where CWD has not been reported and deer are present, there is a greater than negligible risk the soil is contaminated with CWD prion.

 

snip...

 

In summary, given the volume of tourists, hunters and servicemen moving between GB and North America, the probability of at least one person travelling to/from a CWD affected area and, in doing so, contaminating their clothing, footwear and/or equipment prior to arriving in GB is greater than negligible. For deer hunters, specifically, the risk is likely to be greater given the increased contact with deer and their environment. However, there is significant uncertainty associated with these estimates.

 

snip...

 

Therefore, it is considered that farmed and park deer may have a higher probability of exposure to CWD transferred to the environment than wild deer given the restricted habitat range and higher frequency of contact with tourists and returning GB residents.

 

snip...

 


 

please see further ;

 

REFERENCE MATERIALS

 

*** Docket No. APHIS-2007-0127 Scrapie in Sheep and Goats Terry Singeltary Sr. Submission ***

 

Monday, November 16, 2015

 

*** Docket No. APHIS-2007-0127 Scrapie in Sheep and Goats Terry Singeltary Sr. Submission ***

 


 


 

Friday, March 18, 2016 CFSAN

 

Constituent Update: FDA Announces Final Rule on Bovine Spongiform Encephalopathy BSE MAD COW TSE PRION Center for Food Safety and Applied Nutrition - Constituent Update

 


 

Tuesday, March 15, 2016

 

Docket No. FDA-2016-N-0321 Risk Assessment of Foodborne Illness Associated with Pathogens from Produce Grown in Fields Amended with Untreated Biological Soil Amendments of Animal Origin; Request for Comments, Scientific Data, and Information Singeltary Submission

 


 

Saturday, February 6, 2016

 

Secretary's Advisory Committee on Animal Health; Meeting [Docket No. APHIS-2016-0007] Singeltary Submission

 


 

Friday, August 14, 2015

 

*** Susceptibility of cattle to the agent of chronic wasting disease from elk after intracranial inoculation

 


 

PRION 2015 CONFERENCE FT. COLLINS CWD RISK FACTORS TO HUMANS

 

*** LATE-BREAKING ABSTRACTS PRION 2015 CONFERENCE ***

 

O18

 

Zoonotic Potential of CWD Prions

 

Liuting Qing1, Ignazio Cali1,2, Jue Yuan1, Shenghai Huang3, Diane Kofskey1, Pierluigi Gambetti1, Wenquan Zou1, Qingzhong Kong1 1Case Western Reserve University, Cleveland, Ohio, USA, 2Second University of Naples, Naples, Italy, 3Encore Health Resources, Houston, Texas, USA

 

*** These results indicate that the CWD prion has the potential to infect human CNS and peripheral lymphoid tissues and that there might be asymptomatic human carriers of CWD infection.

 

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

 

***These results indicate that the CWD prion has the potential to infect human CNS and peripheral lymphoid tissues and that there might be asymptomatic human carriers of CWD infection.***

 

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

 

P.105: RT-QuIC models trans-species prion transmission

 

Kristen Davenport, Davin Henderson, Candace Mathiason, and Edward Hoover Prion Research Center; Colorado State University; Fort Collins, CO USA

 

Conversely, FSE maintained sufficient BSE characteristics to more efficiently convert bovine rPrP than feline rPrP. Additionally, human rPrP was competent for conversion by CWD and fCWD.

 

***This insinuates that, at the level of protein:protein interactions, the barrier preventing transmission of CWD to humans is less robust than previously estimated.

 

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

 

***This insinuates that, at the level of protein:protein interactions, the barrier preventing transmission of CWD to humans is less robust than previously estimated.***

 

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

 


 

*** PRICE OF CWD TSE PRION POKER GOES UP 2014 ***

 

Transmissible Spongiform Encephalopathy TSE PRION update January 2, 2014

 

*** chronic wasting disease, there was no absolute barrier to conversion of the human prion protein.

 

*** Furthermore, the form of human PrPres produced in this in vitro assay when seeded with CWD, resembles that found in the most common human prion disease, namely sCJD of the MM1 subtype.

 


 


 

*** 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).***

 


 

*** 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.

 


 

now, let’s see what the authors said about this casual link, personal communications years ago. 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

 

To: rr26k@nih.gov; rrace@niaid.nih.gov; ebb8@CDC.GOV

 

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 ;

 


 

CJD is so rare in people under age 30, one case in a billion (leaving out medical mishaps), that four cases under 30 is "very high," says Colorado neurologist Bosque. "Then, if you add these other two from Wisconsin [cases in the newspaper], six cases of CJD in people associated with venison is very, very high." Only now, with Mary Riley, there are at least seven, and possibly eight, with Steve, her dining companion. "It's not critical mass that matters," however, Belay says. "One case would do it for me." The chance that two people who know each other would both contact CJD, like the two Wisconsin sportsmen, is so unlikely, experts say, it would happen only once in 140 years.

 

Given the incubation period for TSEs in humans, it may require another generation to write the final chapter on CWD in Wisconsin. "Does chronic wasting disease pass into humans? We'll be able to answer that in 2022," says Race. Meanwhile, the state has become part of an immense experiment.

 


 

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 ***

 


 

Envt.07:

 

Pathological Prion Protein (PrPTSE) in Skeletal Muscles of Farmed and Free Ranging White-Tailed Deer Infected with Chronic Wasting Disease

 

***The presence and seeding activity of PrPTSE in skeletal muscle from CWD-infected cervids suggests prevention of such tissue in the human diet as a precautionary measure for food safety, pending on further clarification of whether CWD may be transmissible to humans.

 


 

Prions in Skeletal Muscles of Deer with Chronic Wasting Disease Rachel C. Angers1,*, Shawn R. Browning1,*,†, Tanya S. Seward2, Christina J. Sigurdson4,‡, Michael W. Miller5, Edward A. Hoover4, Glenn C. Telling1,2,3,§ snip...

 

Abstract The emergence of chronic wasting disease (CWD) in deer and elk in an increasingly wide geographic area, as well as the interspecies transmission of bovine spongiform encephalopathy to humans in the form of variant Creutzfeldt Jakob disease, have raised concerns about the zoonotic potential of CWD. Because meat consumption is the most likely means of exposure, it is important to determine whether skeletal muscle of diseased cervids contains prion infectivity. 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.

 


 

***********CJD REPORT 1994 increased risk for consumption of veal and venison and lamb***********

 

CREUTZFELDT JAKOB DISEASE SURVEILLANCE IN THE UNITED KINGDOM THIRD ANNUAL REPORT AUGUST 1994

 

Consumption of venison and veal was much less widespread among both cases and controls. For both of these meats there was evidence of a trend with increasing frequency of consumption being associated with increasing risk of CJD. (not nvCJD, but sporadic CJD...tss)

 

These associations were largely unchanged when attention was restricted to pairs with data obtained from relatives. ...

 

Table 9 presents the results of an analysis of these data.

 

There is STRONG evidence of an association between ‘’regular’’ veal eating and risk of CJD (p = .0.01).

 

Individuals reported to eat veal on average at least once a year appear to be at 13 TIMES THE RISK of individuals who have never eaten veal.

 

There is, however, a very wide confidence interval around this estimate. There is no strong evidence that eating veal less than once per year is associated with increased risk of CJD (p = 0.51).

 

The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).

 

There is some evidence that risk of CJD INCREASES WITH INCREASING FREQUENCY OF LAMB EATING (p = 0.02).

 

The evidence for such an association between beef eating and CJD is weaker (p = 0.14). When only controls for whom a relative was interviewed are included, this evidence becomes a little STRONGER (p = 0.08).

 

snip...

 

It was found that when veal was included in the model with another exposure, the association between veal and CJD remained statistically significant (p = < 0.05 for all exposures), while the other exposures ceased to be statistically significant (p = > 0.05).

 

snip...

 

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. ...

 

snip...

 

In the study in the USA, a range of foodstuffs were associated with an increased risk of CJD, including liver consumption which was associated with an apparent SIX-FOLD INCREASE IN THE RISK OF CJD. By comparing the data from 3 studies in relation to this particular dietary factor, the risk of liver consumption became non-significant with an odds ratio of 1.2 (PERSONAL COMMUNICATION, PROFESSOR A. HOFMAN. ERASMUS UNIVERSITY, ROTTERDAM). (???...TSS)

 

snip...see full report ;

 


 

CJD9/10022

 

October 1994

 

Mr R.N. Elmhirst Chairman British Deer Farmers Association Holly Lodge Spencers Lane BerksWell Coventry CV7 7BZ

 

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 a completely independant 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.

 

The Chief Medical Officer has undertaken to keep the public fully informed of the results of any research in respect of CJD. This report was entirely the work of the unit and was produced completely independantly of the the Department.

 

The statistical results reqarding 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 once by the media ie. in the News at one television proqramme.

 

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.

 


 

*** 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).***

 


 

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***

 

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

 

***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.

 

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

 


 

Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES

 

Title: Transmission of scrapie prions to primate after an extended silent incubation period

 

Authors

 

item Comoy, Emmanuel - item Mikol, Jacqueline - item Luccantoni-Freire, Sophie - item Correia, Evelyne - item Lescoutra-Etchegaray, Nathalie - item Durand, Valérie - item Dehen, Capucine - item Andreoletti, Olivier - item Casalone, Cristina - item Richt, Juergen item Greenlee, Justin item Baron, Thierry - item Benestad, Sylvie - item Hills, Bob - item Brown, Paul - item Deslys, Jean-Philippe -

 

Submitted to: Scientific Reports Publication Type: Peer Reviewed Journal Publication Acceptance Date: May 28, 2015 Publication Date: June 30, 2015 Citation: Comoy, E.E., Mikol, J., Luccantoni-Freire, S., Correia, E., Lescoutra-Etchegaray, N., Durand, V., Dehen, C., Andreoletti, O., Casalone, C., Richt, J.A., Greenlee, J.J., Baron, T., Benestad, S., Brown, P., Deslys, J. 2015. Transmission of scrapie prions to primate after an extended silent incubation period. Scientific Reports. 5:11573.

 

Interpretive Summary: The transmissible spongiform encephalopathies (also called prion diseases) are fatal neurodegenerative diseases that affect animals and humans. The agent of prion diseases is a misfolded form of the prion protein that is resistant to breakdown by the host cells. Since all mammals express prion protein on the surface of various cells such as neurons, all mammals are, in theory, capable of replicating prion diseases. One example of a prion disease, bovine spongiform encephalopathy (BSE; also called mad cow disease), has been shown to infect cattle, sheep, exotic undulates, cats, non-human primates, and humans when the new host is exposed to feeds or foods contaminated with the disease agent. The purpose of this study was to test whether non-human primates (cynomologous macaque) are susceptible to the agent of sheep scrapie. After an incubation period of approximately 10 years a macaque developed progressive clinical signs suggestive of neurologic disease. Upon postmortem examination and microscopic examination of tissues, there was a widespread distribution of lesions consistent with a transmissible spongiform encephalopathy. This information will have a scientific impact since it is the first study that demonstrates the transmission of scrapie to a non-human primate with a close genetic relationship to humans. This information is especially useful to regulatory officials and those involved with risk assessment of the potential transmission of animal prion diseases to humans. Technical Abstract: Classical bovine spongiform encephalopathy (c-BSE) is an animal prion disease that also causes variant Creutzfeldt-Jakob disease in humans. Over the past decades, c-BSE's zoonotic potential has been the driving force in establishing extensive protective measures for animal and human health.

 

*** 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.

 


 

***This information will have a scientific impact since it is the first study that demonstrates the transmission of scrapie to a non-human primate with a close genetic relationship to humans. This information is especially useful to regulatory officials and those involved with risk assessment of the potential transmission of animal prion diseases to humans.

 

***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.

 


 

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

 


 

”The occurrence of CWD must be viewed against the contest of the locations in which it occurred. It was an incidental and unwelcome complication of the respective wildlife research programmes. Despite it’s subsequent recognition as a new disease of cervids, therefore justifying direct investigation, no specific research funding was forthcoming. The USDA veiwed it as a wildlife problem and consequently not their province!” page 26.

 


 

In Confidence - Perceptions of unconventional slow virus diseases of animals in the USA - APRIL-MAY 1989 - G A H Wells

 

3. Prof. A. Robertson gave a brief account of BSE. The US approach was to accord it a very low profile indeed. Dr. A Thiermann showed the picture in the ''Independent'' with cattle being incinerated and thought this was a fanatical incident to be avoided in the US at all costs. ...

 


 

MAD COW DISEASE HAS BEEN IN THE USA FOR DECADES, AND I BELIEVE IT WAS IN THE USA FIRST, PLEASE SEE ;

 

Evidence That Transmissible Mink Encephalopathy Results from Feeding Infected Cattle Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.

 

snip...

 

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

 


 


 


 

PL1

 

Using in vitro prion replication for high sensitive detection of prions and prionlike proteins and for understanding mechanisms of transmission.

 

Claudio Soto

 

Mitchell Center for Alzheimer's diseases and related Brain disorders, Department of Neurology, University of Texas Medical School at Houston.

 

Prion and prion-like proteins are misfolded protein aggregates with the ability to selfpropagate to spread disease between cells, organs and in some cases across individuals. I n T r a n s m i s s i b l e s p o n g i f o r m encephalopathies (TSEs), prions are mostly composed by a misfolded form of the prion protein (PrPSc), which propagates by transmitting its misfolding to the normal prion protein (PrPC). The availability of a procedure to replicate prions in the laboratory may be important to study the mechanism of prion and prion-like spreading and to develop high sensitive detection of small quantities of misfolded proteins in biological fluids, tissues and environmental samples. Protein Misfolding Cyclic Amplification (PMCA) is a simple, fast and efficient methodology to mimic prion replication in the test tube. PMCA is a platform technology that may enable amplification of any prion-like misfolded protein aggregating through a seeding/nucleation process. In TSEs, PMCA is able to detect the equivalent of one single molecule of infectious PrPSc and propagate prions that maintain high infectivity, strain properties and species specificity. Using PMCA we have been able to detect PrPSc in blood and urine of experimentally infected animals and humans affected by vCJD with high sensitivity and specificity. Recently, we have expanded the principles of PMCA to amplify amyloid-beta (Aβ) and alphasynuclein (α-syn) aggregates implicated in Alzheimer's and Parkinson's diseases, respectively. Experiments are ongoing to study the utility of this technology to detect Aβ and α-syn aggregates in samples of CSF and blood from patients affected by these diseases.

 

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

 

***Recently, we have been using PMCA to study the role of environmental prion contamination on the horizontal spreading of TSEs. These experiments have focused on the study of the interaction of prions with plants and environmentally relevant surfaces. Our results show that plants (both leaves and roots) bind tightly to prions present in brain extracts and excreta (urine and feces) and retain even small quantities of PrPSc for long periods of time. Strikingly, ingestion of prioncontaminated leaves and roots produced disease with a 100% attack rate and an incubation period not substantially longer than feeding animals directly with scrapie brain homogenate. Furthermore, plants can uptake prions from contaminated soil and transport them to different parts of the plant tissue (stem and leaves). Similarly, prions bind tightly to a variety of environmentally relevant surfaces, including stones, wood, metals, plastic, glass, cement, etc. Prion contaminated surfaces efficiently transmit prion disease when these materials were directly injected into the brain of animals and strikingly when the contaminated surfaces were just placed in the animal cage. These findings demonstrate that environmental materials can efficiently bind infectious prions and act as carriers of infectivity, suggesting that they may play an important role in the horizontal transmission of the disease.

 

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

 

Since its invention 13 years ago, PMCA has helped to answer fundamental questions of prion propagation and has broad applications in research areas including the food industry, blood bank safety and human and veterinary disease diagnosis.

 


 

see ;

 


 


 


 


 


 

Wednesday, December 16, 2015

 

Objects in contact with classical scrapie sheep act as a reservoir for scrapie transmission

 

Objects in contact with classical scrapie sheep act as a reservoir for scrapie transmission

 

Timm Konold1*, Stephen A. C. Hawkins2, Lisa C. Thurston3, Ben C. Maddison4, Kevin C. Gough5, Anthony Duarte1 and Hugh A. Simmons1

 

1 Animal Sciences Unit, Animal and Plant Health Agency Weybridge, Addlestone, UK, 2 Pathology Department, Animal and Plant Health Agency Weybridge, Addlestone, UK, 3 Surveillance and Laboratory Services, Animal and Plant Health Agency Penrith, Penrith, UK, 4 ADAS UK, School of Veterinary Medicine and Science, University of Nottingham, Sutton Bonington, UK, 5 School of Veterinary Medicine and Science, University of Nottingham, Sutton Bonington, UK

 

Classical scrapie is an environmentally transmissible prion disease of sheep and goats. Prions can persist and remain potentially infectious in the environment for many years and thus pose a risk of infecting animals after re-stocking. In vitro studies using serial protein misfolding cyclic amplification (sPMCA) have suggested that objects on a scrapie affected sheep farm could contribute to disease transmission. This in vivo study aimed to determine the role of field furniture (water troughs, feeding troughs, fencing, and other objects that sheep may rub against) used by a scrapie-infected sheep flock as a vector for disease transmission to scrapie-free lambs with the prion protein genotype VRQ/VRQ, which is associated with high susceptibility to classical scrapie. When the field furniture was placed in clean accommodation, sheep became infected when exposed to either a water trough (four out of five) or to objects used for rubbing (four out of seven). This field furniture had been used by the scrapie-infected flock 8 weeks earlier and had previously been shown to harbor scrapie prions by sPMCA. Sheep also became infected (20 out of 23) through exposure to contaminated field furniture placed within pasture not used by scrapie-infected sheep for 40 months, even though swabs from this furniture tested negative by PMCA. This infection rate decreased (1 out of 12) on the same paddock after replacement with clean field furniture. Twelve grazing sheep exposed to field furniture not in contact with scrapie-infected sheep for 18 months remained scrapie free. The findings of this study highlight the role of field furniture used by scrapie-infected sheep to act as a reservoir for disease re-introduction although infectivity declines considerably if the field furniture has not been in contact with scrapie-infected sheep for several months. PMCA may not be as sensitive as VRQ/VRQ sheep to test for environmental contamination.

 

snip...

 

Discussion

 

Classical scrapie is an environmentally transmissible disease because it has been reported in naïve, supposedly previously unexposed sheep placed in pastures formerly occupied by scrapie-infected sheep (4, 19, 20). Although the vector for disease transmission is not known, soil is likely to be an important reservoir for prions (2) where – based on studies in rodents – prions can adhere to minerals as a biologically active form (21) and remain infectious for more than 2 years (22). Similarly, chronic wasting disease (CWD) has re-occurred in mule deer housed in paddocks used by infected deer 2 years earlier, which was assumed to be through foraging and soil consumption (23).

 

Our study suggested that the risk of acquiring scrapie infection was greater through exposure to contaminated wooden, plastic, and metal surfaces via water or food troughs, fencing, and hurdles than through grazing. Drinking from a water trough used by the scrapie flock was sufficient to cause infection in sheep in a clean building. Exposure to fences and other objects used for rubbing also led to infection, which supported the hypothesis that skin may be a vector for disease transmission (9). The risk of these objects to cause infection was further demonstrated when 87% of 23 sheep presented with PrPSc in lymphoid tissue after grazing on one of the paddocks, which contained metal hurdles, a metal lamb creep and a water trough in contact with the scrapie flock up to 8 weeks earlier, whereas no infection had been demonstrated previously in sheep grazing on this paddock, when equipped with new fencing and field furniture. When the contaminated furniture and fencing were removed, the infection rate dropped significantly to 8% of 12 sheep, with soil of the paddock as the most likely source of infection caused by shedding of prions from the scrapie-infected sheep in this paddock up to a week earlier.

 

This study also indicated that the level of contamination of field furniture sufficient to cause infection was dependent on two factors: stage of incubation period and time of last use by scrapie-infected sheep. Drinking from a water trough that had been used by scrapie sheep in the predominantly pre-clinical phase did not appear to cause infection, whereas infection was shown in sheep drinking from the water trough used by scrapie sheep in the later stage of the disease. It is possible that contamination occurred through shedding of prions in saliva, which may have contaminated the surface of the water trough and subsequently the water when it was refilled. Contamination appeared to be sufficient to cause infection only if the trough was in contact with sheep that included clinical cases. Indeed, there is an increased risk of bodily fluid infectivity with disease progression in scrapie (24) and CWD (25) based on PrPSc detection by sPMCA. Although ultraviolet light and heat under natural conditions do not inactivate prions (26), furniture in contact with the scrapie flock, which was assumed to be sufficiently contaminated to cause infection, did not act as vector for disease if not used for 18 months, which suggest that the weathering process alone was sufficient to inactivate prions.

 

PrPSc detection by sPMCA is increasingly used as a surrogate for infectivity measurements by bioassay in sheep or mice. In this reported study, however, the levels of PrPSc present in the environment were below the limit of detection of the sPMCA method, yet were still sufficient to cause infection of in-contact animals. In the present study, the outdoor objects were removed from the infected flock 8 weeks prior to sampling and were positive by sPMCA at very low levels (2 out of 37 reactions). As this sPMCA assay also yielded 2 positive reactions out of 139 in samples from the scrapie-free farm, the sPMCA assay could not detect PrPSc on any of the objects above the background of the assay. False positive reactions with sPMCA at a low frequency associated with de novo formation of infectious prions have been reported (27, 28). This is in contrast to our previous study where we demonstrated that outdoor objects that had been in contact with the scrapie-infected flock up to 20 days prior to sampling harbored PrPSc that was detectable by sPMCA analysis [4 out of 15 reactions (12)] and was significantly more positive by the assay compared to analogous samples from the scrapie-free farm. This discrepancy could be due to the use of a different sPMCA substrate between the studies that may alter the efficiency of amplification of the environmental PrPSc. In addition, the present study had a longer timeframe between the objects being in contact with the infected flock and sampling, which may affect the levels of extractable PrPSc. Alternatively, there may be potentially patchy contamination of this furniture with PrPSc, which may have been missed by swabbing. The failure of sPMCA to detect CWD-associated PrP in saliva from clinically affected deer despite confirmation of infectivity in saliva-inoculated transgenic mice was associated with as yet unidentified inhibitors in saliva (29), and it is possible that the sensitivity of sPMCA is affected by other substances in the tested material. In addition, sampling of amplifiable PrPSc and subsequent detection by sPMCA may be more difficult from furniture exposed to weather, which is supported by the observation that PrPSc was detected by sPMCA more frequently in indoor than outdoor furniture (12). A recent experimental study has demonstrated that repeated cycles of drying and wetting of prion-contaminated soil, equivalent to what is expected under natural weathering conditions, could reduce PMCA amplification efficiency and extend the incubation period in hamsters inoculated with soil samples (30). This seems to apply also to this study even though the reduction in infectivity was more dramatic in the sPMCA assays than in the sheep model. Sheep were not kept until clinical end-point, which would have enabled us to compare incubation periods, but the lack of infection in sheep exposed to furniture that had not been in contact with scrapie sheep for a longer time period supports the hypothesis that prion degradation and subsequent loss of infectivity occurs even under natural conditions.

 

In conclusion, the results in the current study indicate that removal of furniture that had been in contact with scrapie-infected animals should be recommended, particularly since cleaning and decontamination may not effectively remove scrapie infectivity (31), even though infectivity declines considerably if the pasture and the field furniture have not been in contact with scrapie-infected sheep for several months. As sPMCA failed to detect PrPSc in furniture that was subjected to weathering, even though exposure led to infection in sheep, this method may not always be reliable in predicting the risk of scrapie infection through environmental contamination. These results suggest that the VRQ/VRQ sheep model may be more sensitive than sPMCA for the detection of environmentally associated scrapie, and suggest that extremely low levels of scrapie contamination are able to cause infection in susceptible sheep genotypes.

 

Keywords: classical scrapie, prion, transmissible spongiform encephalopathy, sheep, field furniture, reservoir, serial protein misfolding cyclic amplification

 


 

Wednesday, December 16, 2015

 

*** Objects in contact with classical scrapie sheep act as a reservoir for scrapie transmission ***

 


 

Circulation of prions within dust on a scrapie affected farm

 

Kevin C Gough1, Claire A Baker2, Hugh A Simmons3, Steve A Hawkins3 and Ben C Maddison2*

 

Abstract

 

Prion diseases are fatal neurological disorders that affect humans and animals. Scrapie of sheep/goats and Chronic Wasting Disease (CWD) of deer/elk are contagious prion diseases where environmental reservoirs have a direct link to the transmission of disease. Using protein misfolding cyclic amplification we demonstrate that scrapie PrPSc can be detected within circulating dusts that are present on a farm that is naturally contaminated with sheep scrapie. The presence of infectious scrapie within airborne dusts may represent a possible route of infection and illustrates the difficulties that may be associated with the effective decontamination of such scrapie affected premises.

 

snip...

 

Discussion

 

We present biochemical data illustrating the airborne movement of scrapie containing material within a contaminated farm environment. We were able to detect scrapie PrPSc within extracts from dusts collected over a 70 day period, in the absence of any sheep activity. We were also able to detect scrapie PrPSc within dusts collected within pasture at 30 m but not at 60 m distance away from the scrapie contaminated buildings, suggesting that the chance of contamination of pasture by scrapie contaminated dusts decreases with distance from contaminated farm buildings. PrPSc amplification by sPMCA has been shown to correlate with infectivity and amplified products have been shown to be infectious [14,15]. These experiments illustrate the potential for low dose scrapie infectivity to be present within such samples. We estimate low ng levels of scrapie positive brain equivalent were deposited per m2 over 70 days, in a barn previously occupied by sheep affected with scrapie. This movement of dusts and the accumulation of low levels of scrapie infectivity within this environment may in part explain previous observations where despite stringent pen decontamination regimens healthy lambs still became scrapie infected after apparent exposure from their environment alone [16]. The presence of sPMCA seeding activity and by inference, infectious prions within dusts, and their potential for airborne dissemination is highly novel and may have implications for the spread of scrapie within infected premises. The low level circulation and accumulation of scrapie prion containing dust material within the farm environment will likely impede the efficient decontamination of such scrapie contaminated buildings unless all possible reservoirs of dust are removed. Scrapie containing dusts could possibly infect animals during feeding and drinking, and respiratory and conjunctival routes may also be involved. It has been demonstrated that scrapie can be efficiently transmitted via the nasal route in sheep [17], as is also the case for CWD in both murine models and in white tailed deer [18-20].

 

The sources of dust borne prions are unknown but it seems reasonable to assume that faecal, urine, skin, parturient material and saliva-derived prions may contribute to this mobile environmental reservoir of infectivity. This work highlights a possible transmission route for scrapie within the farm environment, and this is likely to be paralleled in CWD which shows strong similarities with scrapie in terms of prion dissemination and disease transmission. The data indicate that the presence of scrapie prions in dust is likely to make the control of these diseases a considerable challenge.

 


 

Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES

 

Title: Scrapie transmits to white-tailed deer by the oral route and has a molecular profile similar to chronic wasting disease

 

Authors

 

item Greenlee, Justin item Moore, S - item Smith, Jodi - item Kunkle, Robert item West Greenlee, M -

 

Submitted to: American College of Veterinary Pathologists Meeting Publication Type: Abstract Only Publication Acceptance Date: August 12, 2015 Publication Date: N/A Technical Abstract: The purpose of this work was to determine susceptibility of white-tailed deer (WTD) to the agent of sheep scrapie and to compare the resultant PrPSc to that of the original inoculum and chronic wasting disease (CWD). We inoculated WTD by a natural route of exposure (concurrent oral and intranasal (IN); n=5) with a US scrapie isolate. All scrapie-inoculated deer had evidence of PrPSc accumulation. PrPSc was detected in lymphoid tissues at preclinical time points, and deer necropsied after 28 months post-inoculation had clinical signs, spongiform encephalopathy, and widespread distribution of PrPSc in neural and lymphoid tissues. Western blotting (WB) revealed PrPSc with 2 distinct molecular profiles. WB on cerebral cortex had a profile similar to the original scrapie inoculum, whereas WB of brainstem, cerebellum, or lymph nodes revealed PrPSc with a higher profile resembling CWD. Homogenates with the 2 distinct profiles from WTD with clinical scrapie were further passaged to mice expressing cervid prion protein and intranasally to sheep and WTD. In cervidized mice, the two inocula have distinct incubation times. Sheep inoculated intranasally with WTD derived scrapie developed disease, but only after inoculation with the inoculum that had a scrapie-like profile. The WTD study is ongoing, but deer in both inoculation groups are positive for PrPSc by rectal mucosal biopsy. In summary, this work demonstrates that WTD are susceptible to the agent of scrapie, two distinct molecular profiles of PrPSc are present in the tissues of affected deer, and inoculum of either profile readily passes to deer.

 


 


 

White-tailed Deer are Susceptible to Scrapie by Natural Route of Infection

 

Jodi D. Smith, Justin J. Greenlee, and Robert A. Kunkle; Virus and Prion Research Unit, National Animal Disease Center, USDA-ARS

 

Interspecies transmission studies afford the opportunity to better understand the potential host range and origins of prion diseases. Previous experiments demonstrated that white-tailed deer are susceptible to sheep-derived scrapie by intracranial inoculation. The purpose of this study was to determine susceptibility of white-tailed deer to scrapie after a natural route of exposure. Deer (n=5) were inoculated by concurrent oral (30 ml) and intranasal (1 ml) instillation of a 10% (wt/vol) brain homogenate derived from a sheep clinically affected with scrapie. Non-inoculated deer were maintained as negative controls. All deer were observed daily for clinical signs. Deer were euthanized and necropsied when neurologic disease was evident, and tissues were examined for abnormal prion protein (PrPSc) by immunohistochemistry (IHC) and western blot (WB). One animal was euthanized 15 months post-inoculation (MPI) due to an injury. At that time, examination of obex and lymphoid tissues by IHC was positive, but WB of obex and colliculus were negative. Remaining deer developed clinical signs of wasting and mental depression and were necropsied from 28 to 33 MPI. Tissues from these deer were positive for scrapie by IHC and WB. Tissues with PrPSc immunoreactivity included brain, tonsil, retropharyngeal and mesenteric lymph nodes, hemal node, Peyer’s patches, and spleen. This work demonstrates for the first time that white-tailed deer are susceptible to sheep scrapie by potential natural routes of inoculation. In-depth analysis of tissues will be done to determine similarities between scrapie in deer after intracranial and oral/intranasal inoculation and chronic wasting disease resulting from similar routes of inoculation.

 

see full text ;

 


 

PO-039: A comparison of scrapie and chronic wasting disease in white-tailed deer

 

Justin Greenlee, Jodi Smith, Eric Nicholson US Dept. Agriculture; Agricultural Research Service, National Animal Disease Center; Ames, IA USA

 


 

White-tailed deer are susceptible to the agent of sheep scrapie by intracerebral inoculation

 

snip...

 

It is unlikely that CWD will be eradicated from free-ranging cervids, and the disease is likely to continue to spread geographically [10]. However, the potential that white-tailed deer may be susceptible to sheep scrapie by a natural route presents an additional confounding factor to halting the spread of CWD. This leads to the additional speculations that

 

1) infected deer could serve as a reservoir to infect sheep with scrapie offering challenges to scrapie eradication efforts and

 

2) CWD spread need not remain geographically confined to current endemic areas, but could occur anywhere that sheep with scrapie and susceptible cervids cohabitate.

 

This work demonstrates for the first time that white-tailed deer are susceptible to sheep scrapie by intracerebral inoculation with a high attack rate and that the disease that results has similarities to CWD. These experiments will be repeated with a more natural route of inoculation to determine the likelihood of the potential transmission of sheep scrapie to white-tailed deer. If scrapie were to occur in white-tailed deer, results of this study indicate that it would be detected as a TSE, but may be difficult to differentiate from CWD without in-depth biochemical analysis.

 


 


 

2012

 

PO-039: A comparison of scrapie and chronic wasting disease in white-tailed deer

 

Justin Greenlee, Jodi Smith, Eric Nicholson US Dept. Agriculture; Agricultural Research Service, National Animal Disease Center; Ames, IA USA

 

snip...

 

The results of this study suggest that there are many similarities in the manifestation of CWD and scrapie in WTD after IC inoculation including early and widespread presence of PrPSc in lymphoid tissues, clinical signs of depression and weight loss progressing to wasting, and an incubation time of 21-23 months. Moreover, western blots (WB) done on brain material from the obex region have a molecular profile similar to CWD and distinct from tissues of the cerebrum or the scrapie inoculum. However, results of microscopic and IHC examination indicate that there are differences between the lesions expected in CWD and those that occur in deer with scrapie: amyloid plaques were not noted in any sections of brain examined from these deer and the pattern of immunoreactivity by IHC was diffuse rather than plaque-like.

 

*** After a natural route of exposure, 100% of WTD were susceptible to scrapie.

 

Deer developed clinical signs of wasting and mental depression and were necropsied from 28 to 33 months PI. Tissues from these deer were positive for PrPSc by IHC and WB. Similar to IC inoculated deer, samples from these deer exhibited two different molecular profiles: samples from obex resembled CWD whereas those from cerebrum were similar to the original scrapie inoculum. On further examination by WB using a panel of antibodies, the tissues from deer with scrapie exhibit properties differing from tissues either from sheep with scrapie or WTD with CWD. Samples from WTD with CWD or sheep with scrapie are strongly immunoreactive when probed with mAb P4, however, samples from WTD with scrapie are only weakly immunoreactive. In contrast, when probed with mAb’s 6H4 or SAF 84, samples from sheep with scrapie and WTD with CWD are weakly immunoreactive and samples from WTD with scrapie are strongly positive. This work demonstrates that WTD are highly susceptible to sheep scrapie, but on first passage, scrapie in WTD is differentiable from CWD.

 


 

2011

 

*** After a natural route of exposure, 100% of white-tailed deer were susceptible to scrapie.

 


 

White-tailed Deer are Susceptible to Scrapie by Natural Route of Infection

 

Jodi D. Smith, Justin J. Greenlee, and Robert A. Kunkle; Virus and Prion Research Unit, National Animal Disease Center, USDA-ARS

 

Interspecies transmission studies afford the opportunity to better understand the potential host range and origins of prion diseases. Previous experiments demonstrated that white-tailed deer are susceptible to sheep-derived scrapie by intracranial inoculation. The purpose of this study was to determine susceptibility of white-tailed deer to scrapie after a natural route of exposure. Deer (n=5) were inoculated by concurrent oral (30 ml) and intranasal (1 ml) instillation of a 10% (wt/vol) brain homogenate derived from a sheep clinically affected with scrapie. Non-inoculated deer were maintained as negative controls. All deer were observed daily for clinical signs. Deer were euthanized and necropsied when neurologic disease was evident, and tissues were examined for abnormal prion protein (PrPSc) by immunohistochemistry (IHC) and western blot (WB). One animal was euthanized 15 months post-inoculation (MPI) due to an injury. At that time, examination of obex and lymphoid tissues by IHC was positive, but WB of obex and colliculus were negative. Remaining deer developed clinical signs of wasting and mental depression and were necropsied from 28 to 33 MPI. Tissues from these deer were positive for scrapie by IHC and WB. Tissues with PrPSc immunoreactivity included brain, tonsil, retropharyngeal and mesenteric lymph nodes, hemal node, Peyer’s patches, and spleen. This work demonstrates for the first time that white-tailed deer are susceptible to sheep scrapie by potential natural routes of inoculation. In-depth analysis of tissues will be done to determine similarities between scrapie in deer after intracranial and oral/intranasal inoculation and chronic wasting disease resulting from similar routes of inoculation.

 

see full text ;

 


 

Monday, November 3, 2014

 

Persistence of ovine scrapie infectivity in a farm environment following cleaning and decontamination

 


 

PPo3-22:

 

Detection of Environmentally Associated PrPSc on a Farm with Endemic Scrapie

 

Ben C. Maddison,1 Claire A. Baker,1 Helen C. Rees,1 Linda A. Terry,2 Leigh Thorne,2 Susan J. Belworthy2 and Kevin C. Gough3 1ADAS-UK LTD; Department of Biology; University of Leicester; Leicester, UK; 2Veterinary Laboratories Agency; Surry, KT UK; 3Department of Veterinary Medicine and Science; University of Nottingham; Sutton Bonington, Loughborough UK

 

Key words: scrapie, evironmental persistence, sPMCA

 

Ovine scrapie shows considerable horizontal transmission, yet the routes of transmission and specifically the role of fomites in transmission remain poorly defined. Here we present biochemical data demonstrating that on a scrapie-affected sheep farm, scrapie prion contamination is widespread. It was anticipated at the outset that if prions contaminate the environment that they would be there at extremely low levels, as such the most sensitive method available for the detection of PrPSc, serial Protein Misfolding Cyclic Amplification (sPMCA), was used in this study. We investigated the distribution of environmental scrapie prions by applying ovine sPMCA to samples taken from a range of surfaces that were accessible to animals and could be collected by use of a wetted foam swab. Prion was amplified by sPMCA from a number of these environmental swab samples including those taken from metal, plastic and wooden surfaces, both in the indoor and outdoor environment. At the time of sampling there had been no sheep contact with these areas for at least 20 days prior to sampling indicating that prions persist for at least this duration in the environment. These data implicate inanimate objects as environmental reservoirs of prion infectivity which are likely to contribute to disease transmission.

 


 

Veterinary Pathology Onlinevet.sagepub.com Published online before print February 27, 2014, doi: 10.1177/0300985814524798 Veterinary Pathology February 27, 2014 0300985814524798

 

Lesion Profiling and Subcellular Prion Localization of Cervid Chronic Wasting Disease in Domestic Cats

 

D. M. Seelig1⇑ A. V. Nalls1 M. Flasik2 V. Frank1 S. Eaton2 C. K. Mathiason1 E. A. Hoover1 1Department of Microbiology, Immunology, and Pathology, Colorado State University, Fort Collins, CO, USA 2Department of Biomedical Sciences, Colorado State University, Fort Collins, CO, USA D. M. Seelig, University of Minnesota, Department of Veterinary Clinical Sciences, Room 339 VetMedCtrS, 6192A (Campus Delivery Code), 1352 Boyd Ave, St Paul, MN 55108, USA. Email address: dseelig@umn.edu

 

Abstract

 

Chronic wasting disease (CWD) is an efficiently transmitted, fatal, and progressive prion disease of cervids with an as yet to be fully clarified host range. While outbred domestic cats (Felis catus) have recently been shown to be susceptible to experimental CWD infection, the neuropathologic features of the infection are lacking. Such information is vital to provide diagnostic power in the event of natural interspecies transmission and insights into host and strain interactions in interspecies prion infection. Using light microscopy and immunohistochemistry, we detail the topographic pattern of neural spongiosis (the “lesion profile”) and the distribution of misfolded prion protein in the primary and secondary passage of feline CWD (FelCWD). We also evaluated cellular and subcellular associations between misfolded prion protein (PrPD) and central nervous system neurons and glial cell populations. From these studies, we (1) describe the novel neuropathologic profile of FelCWD, which is distinct from either cervid CWD or feline spongiform encephalopathy (FSE), and (2) provide evidence of serial passage-associated interspecies prion adaptation. In addition, we demonstrate through confocal analysis the successful co-localization of PrPD with neurons, astrocytes, microglia, lysosomes, and synaptophysin, which, in part, implicates each of these in the neuropathology of FelCWD. In conclusion, this work illustrates the simultaneous role of both host and strain in the development of a unique FelCWD neuropathologic profile and that such a profile can be used to discriminate between FelCWD and FSE.

 

prion chronic wasting disease immunohistochemistry interspecies cat feline spongiform encephalopathy transmissible spongiform encephalopathy adaptation species barrier

 


 

Monday, August 8, 2011 Susceptibility of Domestic Cats to CWD Infection

 

Oral.29: Susceptibility of Domestic Cats to CWD Infection

 

Amy Nalls, Nicholas J. Haley, Jeanette Hayes-Klug, Kelly Anderson, Davis M. Seelig, Dan S. Bucy, Susan L. Kraft, Edward A. Hoover and Candace K. Mathiason†

 

Colorado State University; Fort Collins, CO USA†Presenting author; Email: ckm@lamar.colostate.edu

 

Domestic and non-domestic cats have been shown to be susceptible to one prion disease, feline spongiform encephalopathy (FSE), thought to be transmitted through consumption of bovine spongiform encephalopathy (BSE) contaminated meat. Because domestic and free ranging felids scavenge cervid carcasses, including those in CWD affected areas, we evaluated the susceptibility of domestic cats to CWD infection experimentally. Groups of n = 5 cats each were inoculated either intracerebrally (IC) or orally (PO) with CWD deer brain homogenate. Between 40–43 months following IC inoculation, two cats developed mild but progressive symptoms including weight loss, anorexia, polydipsia, patterned motor behaviors and ataxia—ultimately mandating euthanasia. Magnetic resonance imaging (MRI) on the brain of one of these animals (vs. two age-matched controls) performed just before euthanasia revealed increased ventricular system volume, more prominent sulci, and T2 hyperintensity deep in the white matter of the frontal hemisphere and in cortical grey distributed through the brain, likely representing inflammation or gliosis. PrPRES and widely distributed peri-neuronal vacuoles were demonstrated in the brains of both animals by immunodetection assays. No clinical signs of TSE have been detected in the remaining primary passage cats after 80 months pi. Feline-adapted CWD was sub-passaged into groups (n=4 or 5) of cats by IC, PO, and IP/SQ routes. Currently, at 22 months pi, all five IC inoculated cats are demonstrating abnormal behavior including increasing aggressiveness, pacing, and hyper responsiveness.

 

*** Two of these cats have developed rear limb ataxia. Although the limited data from this ongoing study must be considered preliminary, they raise the potential for cervid-to-feline transmission in nature.

 


 


 

AD.63:

 

Susceptibility of domestic cats to chronic wasting disease

 

Amy V.Nalls,1 Candace Mathiason,1 Davis Seelig,2 Susan Kraft,1 Kevin Carnes,1 Kelly Anderson,1 Jeanette Hayes-Klug1 and Edward A. Hoover1 1Colorado State University; Fort Collins, CO USA; 2University of Minnesota; Saint Paul, MN USA

 

Domestic and nondomestic cats have been shown to be susceptible to feline spongiform encephalopathy (FSE), almost certainly caused by consumption of bovine spongiform encephalopathy (BSE)-contaminated meat. Because domestic and free-ranging nondomestic felids scavenge cervid carcasses, including those in areas affected by chronic wasting disease (CWD), we evaluated the susceptibility of the domestic cat (Felis catus) to CWD infection experimentally. Cohorts of 5 cats each were inoculated either intracerebrally (IC) or orally (PO) with CWD-infected deer brain. At 40 and 42 mo post-inoculation, two IC-inoculated cats developed signs consistent with prion disease, including a stilted gait, weight loss, anorexia, polydipsia, patterned motor behaviors, head and tail tremors, and ataxia, and progressed to terminal disease within 5 mo. Brains from these two cats were pooled and inoculated into cohorts of cats by IC, PO, and intraperitoneal and subcutaneous (IP/SC) routes. Upon subpassage, feline-adapted CWD (FelCWD) was transmitted to all IC-inoculated cats with a decreased incubation period of 23 to 27 mo. FelCWD was detected in the brains of all the symptomatic cats by western blotting and immunohistochemistry and abnormalities were seen in magnetic resonance imaging, including multifocal T2 fluid attenuated inversion recovery (FLAIR) signal hyper-intensities, ventricular size increases, prominent sulci, and white matter tract cavitation. Currently, 3 of 4 IP/SQ and 2 of 4 PO inoculared cats have developed abnormal behavior patterns consistent with the early stage of feline CWD.

 

*** These results demonstrate that CWD can be transmitted and adapted to the domestic cat, thus raising the issue of potential cervid-to- feline transmission in nature.

 


 

www.landesbioscience.com

 

PO-081: Chronic wasting disease in the cat— Similarities to feline spongiform encephalopathy (FSE)

 


 


 

FELINE SPONGIFORM ENCEPHALOPATHY FSE

 


 


 

Wednesday, October 17, 2012

 

Prion Remains Infectious after Passage through Digestive System of American Crows (Corvus brachyrhynchos)

 


 

Chronic Wasting Disease Susceptibility of Four North American Rodents

 

Chad J. Johnson1*, Jay R. Schneider2, Christopher J. Johnson2, Natalie A. Mickelsen2, Julia A. Langenberg3, Philip N. Bochsler4, Delwyn P. Keane4, Daniel J. Barr4, and Dennis M. Heisey2 1University of Wisconsin School of Veterinary Medicine, Department of Comparative Biosciences, 1656 Linden Drive, Madison WI 53706, USA 2US Geological Survey, National Wildlife Health Center, 6006 Schroeder Road, Madison WI 53711, USA 3Wisconsin Department of Natural Resources, 101 South Webster Street, Madison WI 53703, USA 4Wisconsin Veterinary Diagnostic Lab, 445 Easterday Lane, Madison WI 53706, USA *Corresponding author email: cjohnson@svm.vetmed.wisc.edu

 

We intracerebrally challenged four species of native North American rodents that inhabit locations undergoing cervid chronic wasting disease (CWD) epidemics. The species were: deer mice (Peromyscus maniculatus), white-footed mice (P. leucopus), meadow voles (Microtus pennsylvanicus), and red-backed voles (Myodes gapperi). The inocula were prepared from the brains of hunter-harvested white-tailed deer from Wisconsin that tested positive for CWD. Meadow voles proved to be most susceptible, with a median incubation period of 272 days. Immunoblotting and immunohistochemistry confirmed the presence of PrPd in the brains of all challenged meadow voles. Subsequent passages in meadow voles lead to a significant reduction in incubation period. The disease progression in red-backed voles, which are very closely related to the European bank vole (M. glareolus) which have been demonstrated to be sensitive to a number of TSEs, was slower than in meadow voles with a median incubation period of 351 days. We sequenced the meadow vole and red-backed vole Prnp genes and found three amino acid (AA) differences outside of the signal and GPI anchor sequences. Of these differences (T56-, G90S, S170N; read-backed vole:meadow vole), S170N is particularly intriguing due its postulated involvement in "rigid loop" structure and CWD susceptibility. Deer mice did not exhibit disease signs until nearly 1.5 years post-inoculation, but appear to be exhibiting a high degree of disease penetrance. White-footed mice have an even longer incubation period but are also showing high penetrance. Second passage experiments show significant shortening of incubation periods. Meadow voles in particular appear to be interesting lab models for CWD. These rodents scavenge carrion, and are an important food source for many predator species. Furthermore, these rodents enter human and domestic livestock food chains by accidental inclusion in grain and forage. Further investigation of these species as potential hosts, bridge species, and reservoirs of CWD is required.

 

please see ;

 


 


 

Monday, April 04, 2016

 

*** Limited amplification of chronic wasting disease prions in the peripheral tissues of intracerebrally inoculated cattle

 


 

 

I7

 

Early Trafficking and Dissemination of CWD Prions in Deer

 

Edward A. Hoover1*, Clare E. Hoover1, Davin M. Henderson1, Nathaniel D. Denkers1, Kristin A. Davenport1, Shannon Bartelt-Hunt2, Alan M. Elder1, Anthony E. Kincaid3, 4, Jason C. Bartz3, Mark D. Zabel1, Candace K. Mathiason1 1Prion Research Center, Department of Microbiology, Immunology, and Pathology, Colorado State University, Fort Collins, Colorado, USA 2Department of Civil Engineering, University of Nebraska-Lincoln, Prion2015 Program Guide 10 invited speakers

 

PRION 2015

 

Efficient horizontal infection is a hallmark of chronic wasting disease (CWD) in freeranging cervids. The mechanisms and pathways that enable this remarkable process, however, remain incompletely understood--in particular the facile transmucosal entry, exit, and environmental persistence of CWD prions. We have focused on trans-mucosal CWD infection in white-tailed deer, specifically on early prion tissue tropism and later stage prion shedding and association with environmental constituents using modifications of real-time quaking-induced conversion combined with amplified immunohistochemistry. We have documented very early trans-mucosal prion passage (within hours), followed by uptake and amplification in upper digestive tract lymphoid t i s s u e s ( 4 w e e k s ) , and dissemination to more distant lymphoid and non-lymphoid tissue sites (8-12 weeks). We have used quantitative approaches to realtime conversion to estimate the relatively low (i.e. vs. tissues) prion concentrations in body fluids and excreta; i.e. >100 (cervidized mouse) LD50 are shed daily in the urine of one CWD infected deer. Using similar methods, we have also demonstrated and quantified the impressive affinity of CWD prions for both silty clay loam (a major environmental soil constituent) and metal surfaces. Mucosal uptake, excretion, and environmental interactions are central to this most transmissible of prion diseases.

 


 

PMCA Detection of CWD Infection in Cervid and Non-Cervid Species

 

Hoover, Edward Arthur Colorado State University-Fort Collins, Fort Collins, CO, United States

 

ABSTRACT Chronic wasting disease (CWD) of deer and elk is an emerging highly transmissible prion disease now recognized in 18 States, 2 Canadian provinces, and Korea.

 

***We have shown that Infected deer harbor and shed high levels of infectious prions in saliva, blood, urine, and feces, and in the tissues generating those body fluids and excreta, thereby leading to facile transmission by direct contact and environmental contamination.

 

***We have also shown that CWD can infect some non-cervid species, thus the potential risk CWD represents to domestic animal species and to humans remains unknown.

 

Whether prions borne in blood, saliva, nasal fluids, milk, or excreta are generated or modified in the proximate peripheral tissue sites, may differ in subtle ways from those generated in brain, or may be adapted for mucosal infection remain open questions.

 

The increasing parallels in the pathogenesis between prion diseases and human neurodegenerative conditions, such as Alzheimer's and Parkinson's diseases, add relevance to CWD as a transmissible protein misfolding disease.

 

The overall goal of this work is to elucidate the process of CWD prion transmission from mucosal secretory and excretory tissue sites by addressing these questions:

snip...

 

Public Health Relevance Chronic wasting disease (CWD) of deer and elk is an emergent highly transmissible prion disease now recognized throughout the USA as well as in Canada and Korea. We have shown that infected deer harbor and shed high levels of infectious prions in saliva, blood, urine, and feces thereby leading to transmission by direct contact and environmental contamination. In that our studies have also shown that CWD can infect some non-cervid species, the potential risk CWD may represents to domestic animal species and humans remains unknown. The increasing parallels in the development of major human neurodegenerative conditions, such as Alzheimer's and Parkinson's diseases, and prion diseases add relevance to CWD as a model of a transmissible protein misfolding disease. Understanding how infectious misfolded proteins (prions) are generated and transmitted will aid in interrupting, treating, and managing the risks associated with these agents and the diseases they cause.

 


 

 

Prion Amplification and Hierarchical Bayesian Modeling Refine Detection of Prion Infection

 

Infected animals shed prions into the environment through saliva, feces, urine and even antler velvet15,20,21,22,23,24. Studies have successfully transmitted PrPCWD through a single dose of urine or feces from animals displaying signs of CWD, indicating that at the time of clinical disease sufficient prions are shed to result in an infectious dose24,25. However, at what stage(s) of disease animals shed prions into the environment remains unclear. If shedding occurs early in disease, a sub-clinical animal may not only shed prions into the environment, increasing the infectious reservoir, but may also transmit CWD horizontally to their associates.

 


 

Keywords: RT-QuIC, chronic wasting disease, diagnosis, feces, prion, surveillance, urine

 

Authors: Theodore R. John, Hermann M. Schätzl and Sabine Gilch

 

Theodore R. John Department of Veterinary Sciences; University of Wyoming; Laramie, WY USA

 

Hermann M. Schätzl Department of Veterinary Sciences; University of Wyoming; Laramie, WY USA; Department of Molecular Biology; University of Wyoming; Laramie, WY USA; Faculty of Veterinary Medicine; Department of Comparative Biology and Experimental Medicine; University of Calgary; Calgary, AB Canada

 

Sabine Gilch Corresponding author: sgilch@ucalgary.ca Department of Veterinary Sciences; University of Wyoming; Laramie, WY USA; Faculty of Veterinary Medicine; Department of Ecosystem and Public Health; University of Calgary; Calgary, AB Canada

 

Abstract:

 

Chronic wasting disease (CWD) is a prion disease of captive and free-ranging deer (Odocoileus spp), elk (Cervus elaphus nelsonii) and moose (Alces alces shirasi). Unlike in most other prion diseases, in CWD prions are shed in urine and feces, which most likely contributes to the horizontal transmission within and between cervid species. To date, CWD ante-mortem diagnosis is only possible by immunohistochemical detection of protease resistant prion protein (PrPSc) in tonsil or recto-anal mucosa-associated lymphoid tissue (RAMALT) biopsies, which requires anesthesia of animals. We report on detection of CWD prions in urine collected from pre-symptomatic deer and in fecal extracts by using real time quaking-induced conversion (RT-QuIC). This assay can be useful for non-invasive pre-symptomatic diagnosis and surveillance of CWD.

 

Received: February 7, 2013; Accepted: March 24, 2013; Published Online: April 10, 2013

 

snip...

 

Introduction

 

Chronic wasting disease (CWD) is to date the most contagious prion disease and affects captive and free-ranging elk, deer and moose in North America.1,2 The disease is caused by the accumulation of an abnormally folded isoform of the cellular prion protein PrPC, denominated PrPSc.3,4 CWD is the cervid equivalent of bovine spongiform encephalopathy (BSE), scrapie in sheep and goat5 or Creutzfeldt-Jakob disease (CJD) in humans.6 Although transmission studies of CWD prions to humanized transgenic mice or non-human primates suggest a strong species barrier,7-9 recent in vitro studies have demonstrated that human PrP can be converted by CWD prions into PrPSc upon adaptation.10 Therefore, a potential for zoonotic transmission, as exemplified by BSE,11 cannot be completely excluded.

 

A huge body of evidence suggests that CWD can be efficiently transmitted horizontally within and between cervid species,12 which may be the reason for geographical spread and increase in case numbers. Horizontal transmission is explained by the rather unusual peripheral distribution of prions in CWD affected animals and the high susceptibility to the disease by oral infection.13,14 Unlike in most other prion diseases, CWD prions can be found in a wide variety of tissues, such as skeletal and cardiac muscle15,16 or kidney,17 in addition to the lymphoreticular system and blood.18 Furthermore, they are shed in significant amounts in saliva,18,19 urine19 or feces,20 which enables oral infection of animals by foraging on contaminated pastures.

 

In addition, it has been demonstrated that prions can persist in soil21 and that water in endemic areas can contain CWDassociated PrPSc.22

 

snip...

 

We demonstrate that CWD prions are detectable in urine of orally infected deer prior to the onset of clinical symptoms. Furthermore, we show that fecal extracts can be used as a seed in RT-QuIC assays. Thereby, we were able to detect CWD prions in fecal extracts collected at later stages of the disease. This study provides the first evidence that RT-QuIC can be successfully used for the preclinical diagnosis of CWD in specimens that are available by non-invasive methods.

 

snip...

 

In summary, we demonstrate that CWD prions can be detected by RT-QuIC in urine of orally infected white-tailed deer and mule deer at a pre-symptomatic stage of the disease.

 

snip...

 


 

2008

 

Excretion of Transmissible Spongiform Encephalopathy Infectivity in Urine

 

Luisa Gregori, Gabor G. Kovacs, Irina Alexeeva, Herbert Budka, and Robert G. Rohwer

 

To the extent that results from the hamster model can be generalized to other TSE infections (and it has so far proven highly predictive), then even the very low concentrations of infectivity measured here could result in substantial environmental contamination. Several liters of urine and several thousand doses of TSE infectivity may be excreted daily over the course of the illness; even higher titers might be excreted by an animal with nephritis. The high stability of TSE infectivity would account for its persistence in pasture years after infected animals are removed (31). Recent studies have shown that infectivity that is adsorbed and immobilized by soil minerals (32) can still infect hamsters by oral exposure 29 months later (33). Our study also warns of a possible risk from TSE contamination to fertility hormones and other medicinal products extracted from human urine.

 


 

Sunday, November 10, 2013

 

LARGE CJD TSE PRION POTENTIAL CASE STUDY AMONG HUMANS WHO TAKE DEER ANTLER VELVET WILL BE ONGOING FOR YEARS IF NOT DECADES, but who's cares $

 


 

Saturday, February 6, 2016

 

Secretary's Advisory Committee on Animal Health; Meeting [Docket No. APHIS-2016-0007] Singeltary Submission

 


 

Friday, March 18, 2016 CFSAN

 

Constituent Update: FDA Announces Final Rule on Bovine Spongiform Encephalopathy BSE MAD COW TSE PRION Center for Food Safety and Applied Nutrition - Constituent Update

 


 

Tuesday, March 15, 2016

 

*** Docket No. FDA-2016-N-0321 Risk Assessment of Foodborne Illness Associated with Pathogens from Produce Grown in Fields Amended with Untreated Biological Soil Amendments of Animal Origin; Request for Comments, Scientific Data, and Information Singeltary Submission ***

 


 

Monday, March 28, 2016

 

National Scrapie Eradication Program February 2016 Monthly Report

 


 

*** Docket No. APHIS-2007-0127 Scrapie in Sheep and Goats Terry Singeltary Sr. Submission ***

 

Monday, November 16, 2015

 

*** Docket No. APHIS-2007-0127 Scrapie in Sheep and Goats Terry Singeltary Sr. Submission ***

 


 


 

”The occurrence of CWD must be viewed against the contest of the locations in which it occurred. It was an incidental and unwelcome complication of the respective wildlife research programmes. Despite it’s subsequent recognition as a new disease of cervids, therefore justifying direct investigation, no specific research funding was forthcoming. The USDA veiwed it as a wildlife problem and consequently not their province!” page 26.

 


 

In Confidence - Perceptions of unconventional slow virus diseases of animals in the USA - APRIL-MAY 1989 - G A H Wells

 

3. Prof. A. Robertson gave a brief account of BSE. The US approach was to accord it a very low profile indeed. Dr. A Thiermann showed the picture in the ''Independent'' with cattle being incinerated and thought this was a fanatical incident to be avoided in the US at all costs. ...

 


 

MAD COW DISEASE HAS BEEN IN THE USA FOR DECADES, AND I BELIEVE IT WAS IN THE USA FIRST, PLEASE SEE ;

 

Evidence That Transmissible Mink Encephalopathy Results from Feeding Infected Cattle Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.

 

snip...

 

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

 


 


 


 

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

 

From: Gemma.Smith@wales.gsi.gov.uk

 

Sent: Wednesday, February 17, 2016 3:41 AM

 

To: flounder9@verizon.net

 

Subject: RE: Welsh Government and Food Standards Agency Wales Joint Public Consultation on the Proposed Transmissible Spongiform Encephalopathies (Wales) Regulations 2013

 

Dear Mr Singeltary,

 

Thank you for your recent email update on Transmissible Spongiform Encephalopathies (TSEs) and in particular the Chronic Wasting Disease (CWD) prion. The prevention of all TSEs are a priority for the Welsh Government when protecting human and animal health, and the development of cases will continue to be monitored and acted on accordingly.

 

Many thanks and kindest regards,

 

Gemma

 

Gemma Smith Office of the Chief Veterinary Officer / Swyddfa'r Prif Swyddog Milfeddygol Welsh Government / Llywodraeth Cymru

 

From: Terry S. Singeltary Sr. [mailto:flounder9@verizon.net]

 

Sent: 05 February 2016 19:52

 

To: Endemics

 

Cc: WAG-EN; Jones, Carwyn (Ministerial); Correspondence.Alun.Davies@Wales.gsi.gov.uk; Correspondence Mail - CS; Correspondence Mail - EH; Correspondence Mail - HL; Correspondence Mail - JH; Correspondence.John.Griffiths@Wales.gsi.gov.uk; Correspondence Mail - LG; Correspondence Mail - MD; Correspondence.Jeff.Cuthbert@Wales.gsi.gov.uk; Correspondence Mail - VG; Correspondence Mail - KS; Correspondence.Gwenda.Thomas@Wales.gsi.gov.uk

 

Subject: Re: Welsh Government and Food Standards Agency Wales Joint Public Consultation on the Proposed Transmissible Spongiform Encephalopathies (Wales) Regulations 2013

 

update for you all, it’s not pretty. that bse mrr policy will come back to haunt everyone...kindest regards, terry

 

Subject: TEXAS NEW CHRONIC WASTING DISEASE CWD CASE DISCOVERD AT CAPTIVE DEER RELEASE SITE

 

snip...end...tss

 

From: Terry S. Singeltary Sr.

 

Sent: Thursday, February 18, 2016 11:52 AM

 

To: URL Bernhard

 

Cc: Press@efsa.europa.eu ; SCER.PublicConsult.55@efsa.europa.eu ; scer@efsa.europa.eu ; publicmeetings@efsa.europa.eu

 

Subject: re-Revisiting EFSA@EXPO: emerging issues in animal and plant health

 

re-Revisiting EFSA@EXPO: emerging issues in animal and plant health

 


 

Greetings Dr. Url and EFSA EXPO Revisiting EFSA@EXPO: emerging issues in animal and plant health et al,

 

with the mad cow follies seemingly never to end since the BSE MRR policy i.e. the legal trading of the TSE PRION was adopted around the globe, I wish to kindly submit the following, and I urge all of you to finally put to bed once and for all, bury it, the infamous UKBSEnvCJD only theory. this theory, myth, corporate BSE, call it what you like, has helped fuel the spread of the TSE prion disease, of which, North America is now awash in. I urge you to take heed to your own demise of which you went through during the BSE debacle the first go around. what we are seeing in North America is the same thing, except our governments are just better at covering it up with junk science from the USDA, CFIA, and OIE. with CWD TSE Prion in cervid spreading, maybe on plants too, and the continued cover up of BSE in cattle in North America, I would be very concerned if I were the EFSA et al imo. ...

 

with kindest regards, terry

 

Report of the Committee on Wildlife Diseases FY2015 CWD TSE PRION Detections in Farmed Cervids and Wild

 

snip...end...tss

 

From: Terry S. Singeltary Sr.

 

Sent: Sunday, October 25, 2015 5:19 PM

 

To: EFSA Press ; URL Bernhard

 

Cc: SCER ; EFSA Press ; SCER.PublicConsult.55

 

Subject: Re: re-Wasting disease is threat to the entire UK deer population URGENT UPDATE USA TEXAS ON CWD

 

Greetings again Dr. Bernhard Url, EFSA et al,

 

thought you all might want to see this. I do not advertise on the blogs, or make money doing this. the blogs are for educational use on the tse prion disease.

 

CWD TSE prion update update for your files, as follows ;

 

snip...end...tss

 

From: Terry S. Singeltary Sr.

 

Sent: Tuesday, August 18, 2015 11:46 AM

 

To: sp.info@scottish.parliament.uk

 

Cc: enquiries@swt.org.uk ; admin@jmt.org ; info@johnmuiraward.org ; nicky.mcclure@jmt.org ; broadcasting@scottish.parliament.uk ; racce.committee@scottish.parliament.uk ; Rob.Gibson.msp@scottish.parliament.uk ; Graeme.Dey.msp@scottish.parliament.uk ; tom.edwards@scottish.parliament.uk ; wendy.kenyon@scottish.parliament.uk ; SPICe@scottish.parliament.uk ; racce.committee@scottish.parliament.uk

 

Subject: re-Wasting disease is threat to the entire UK deer population URGENT UPDATE USA TEXAS ON CWD

 

Chronic Wasting Disease CWD TSE Prion is threat to the entire UK deer population URGENT UPDATE USA TEXAS ON CWD August 2015.

 

Greetings Honorable Scottish Parliament et al,

 

I wish to kindly update you on the mad deer disease debacle in Texas. we have lost Texas to CWD, and TAHC et al are making it worse by catering to the industry. same as they did with the mad cow they tried to cover up for 7 months in Texas.

 

I am just trying to warn you again, please take heed. this is an explosive situation, that Texas has taken very lightly, which I believe could potentially have global ramifications, to your wild herds as well ;

 


 

Angus MacDonald (Falkirk East) (Scottish National Party): To ask the Scottish Government what action it is taking to prevent Chronic Wasting Disease (CWD) in wild deer from entering the country and what discussions it has had with the EU on the possible impact of CWD on (a) country sport tourism and (b) sales of venison.

 

snip...end...tss

 

From: EFSA Press

 

Sent: Wednesday, August 19, 2015 5:34 AM

 

To: 'Terry S. Singeltary Sr.' ; URL Bernhard

 

Cc: SCER ; EFSA Press ; SCER.PublicConsult.55

 

Subject: RE: re-Wasting disease is threat to the entire UK deer population URGENT UPDATE USA TEXAS ON CWD

 

Dear Mr Singeltary,

 

Dr. Bernhard Url would like to thank you for your message and asked me to reply on his behalf.

 

Going through your email, we have noticed that you are also running a blog and therefore we thought it would be best if Media Relations acknowledges receipt of your mail.

 

I will take care that your mail is distributed to the appropriate scientific Unit(s) within EFSA. If further questions arise these scientific Units will get in touch with you.

 

Kind regards,

 

Jan

 

Description: cid:image003.png@01D0C89D.0E1DA970

 

Jan Op Gen Oorth Team Leader Media Relations Communications & External Relations Department

 

European Food Safety Authority Via Carlo Magno 1A 43126 Parma - Italy Direct: +39 0521 036 474 Email: jan.opgenoorth@efsa.europa.eu

 

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

 

Description: Description: cid:image001.png@01CFCC2F.614429A0

 

Dear Mr Singeltary

 

Thank you for your email, which was received by Public Information at the Scottish Parliament. We provide information about the Scottish Parliament, its membership, business and procedures. As an impartial information service, we are unable to take matters forward on your behalf, but we hope that the following may be of use.

 

You may be interested in this page on the Scottish Government’s website about chronic wasting disease in deer. As you may know, the Scottish Parliament and Scottish Government are separate organisations. The Scottish Parliament is the law-making body for a wide range of devolved matters such as rural affairs and the environment, and its role is to scrutinise the work of the Scottish Government. The Scottish Government is the government in Scotland for devolved matters, and it is responsible for defining and implementing policy in these areas.

 

The webpage above mentions Scottish Natural Heritage, which is funded through the Scottish Government’s Environment and Forestry Directorate of. Scottish Natural Heritage has information about this disease on their website. Scottish Natural Heritage may therefore be the best people for you to contact about this issue. They have a good contact details section on their website, which includes contacts for senior staff.

 

I hope this information is useful. Please contact us again if you have any questions about the Scottish Parliament.

 

Yours sincerely

 

George Clark Public Information and Publications The Scottish Parliament

 

Our contact details

 

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

 

Description: Description: Description: Description: Description: Description: Description: Description: Description: Description: Corp ID for Signatures

 

Dear Mr Singeltary,

 

Thank you for your email, which was received by Public Information at the Scottish Parliament. We provide impartial information about the Scottish Parliament, its membership, business and procedures.

 

The Scottish Parliament’s Rural Affairs, Climate Change and Environment Committee has been looking into deer management, as you can see from the following press release, and your email has been forwarded to the committee for information:

 


 

You may be interested in following the committee’s work. Papers for committee meetings and transcripts of past meetings (called Official Reports) appear on the following webpage:

 


 

I hope this information is of use to you. Please contact us again if you have any questions about the Scottish Parliament.

 

Yours sincerely,

 

George Clark Public Information and Publications The Scottish Parliament

 

Our contact details

 

Description: Description: TwitterButtonJuly2012

 

Please note that any links to external websites are suggestions only. The Scottish Parliament is not responsible for the content of external websites.

 

From: Stuart MacDiarmid (Stuart)

 

Sent: Monday, May 12, 2014 5:12 PM

 

To: Terry S. Singeltary Sr. Cc: Stuart MacDiarmid (Stuart) ; Alex Thiermann

 

Subject: RE: Member Country’s detailed justification for listing of chronic wasting disease of cervids (CWD) against the criteria of Article 1.2.2., the Code Commission _recommended_ this disease be reconsidered for listing.

 

Dear Mr Singeltary, I apologise for the lengthy delay in replying to your email; I have been unwell. I assume that you are writing to me in my capacity as an elected member of the OIE's Terrestrial Animal Health Standards Commission. On more than one occasion our Commission has received a request from a Member Country to list CWD as a disease notifiable to the OIE. However, it is not our practice to specify which Member Countries make specific requests to us. All countries which submit national comments to us at our February and September meetings are listed in the reports of our meetings. However, the country names are not linked to specific comments or requests. The most recent submissions on CWD, in February 2013 and September 2013, were in response to the work of an ad hoc group which met in 2012 to examine all currently listed diseases against the recently-adopted criteria for listing published in the Terrestrial Animal Health Code. The report of that ad hoc group and its recommendations were circulated for Member Country comments in the report of the Terrestrial Animal Health Standards Commission. The ad hoc group had recommended the delisting of certain diseases because they do not meet the criteria for listing. However, in commenting on the recommendations, a Member Country made a submission proposing the listing of CWD. That was in February 2013. The same country made the same proposal to the September meeting of the Terrestrial Animal Health Standards Commission (TAHSC). The TAHSC passed the recommendation to the Director General of the OIE with a request that next time an ad hoc expert group is convened to consider issues of listing or delisting they may also evaluate CWD against the OIE's criteria. That is where the situation stands at present. Next time an ad hoc group is convened to consider issues of listing and delisting, CWD will be evaluated. I have no idea of time frames. Because I am off work I do not have access to my usual resources, otherwise I could have given you URLs to the various reports and also the Terrestrial Animal Health Code chapter which gives the criteria for listing a disease as notifiable to the OIE. However, I think if you search the OIE web site you should be able to find everything. Regards, Stuart C MacDiarmid Terrestrial Animal Health Standards Commission

 

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

 

From: Terry S. Singeltary Sr. [flounder9@verizon.net]

 

Sent: Thursday, 8 May 2014 10:07 a.m.

 

To: Stuart MacDiarmid (Stuart)

 

Subject: Member Country’s detailed justification for listing of chronic wasting disease of cervids (CWD) against the criteria of Article 1.2.2., the Code Commission _recommended_ this disease be reconsidered for listing.

 

Subject: Member Country’s detailed justification for listing of chronic wasting disease of cervids (CWD) against the criteria of Article 1.2.2., the Code Commission _recommended_ this disease be reconsidered for listing.

 

> In response to a _Member Country’s_ detailed justification for listing of chronic wasting disease of cervids (CWD) against the criteria of Article 1.2.2., the Code Commission _recommended_ this disease be reconsidered for listing.

 


 

Annual report of the Scientific Network on BSE-TSE EFSA, Question No EFSA-Q-2013-01004, approved on 11 December 2013 *** Further, it was addressed that recently discussions have being held at OIE level on Chronic Wasting Disease of cervids. page 6; http://www.efsa.europa.eu/en/supporting/doc/532e.pdf

 

Greetings Prof. Stuart MacDiarmid,

 

I kindly wish to ask a question please. as you know, I have been very concerned since 1997, of the TSE prion disease, since the death of my mother to the hvCJD. I have watched keenly the spread of cwd in North America and Korea. In the links above, it states that there was a possibility that CWD Chronic Wasting Disease of cervids, was to be brought to the table for possible listing by OIE, and that some Country had brought this to the table for reasons I am not sure about. wisely so though.

 

could you please tell me which Country brought this to the table and for what reasons ?

 

and since the code commission recommended this, what happened since then ?

 

was it finally listed ?

 

if not, what were the reasons for not listing it ?

 

Thank You Kindly,

 

terry

 


 

Dear Mr Singeltary,

 

Further to Professor MacDiarmid’s reply, and in acknowledgement of the same email sent to a variety of OIE recipients, including Dr Vallat, the urls to the Terrestrial Animal Health Standards Commission reports that Professor MacDiarmid refers to are:

 

February 2013 (Item 5 Criteria for listing diseases):

 


 

September 2013 (Item 5 Criteria for listing diseases):

 


 

Thank you for your interest and support of OIE’s work.

 

Yours sincerely

 

OIE International Trade Department

 

Thank you for the links, and Thank you for reconsidering CWD as a listed disease.

 

I am writing up a report on cwd TSE prion disease, in North America and the USA, risk factors there from, and game farms, I will be submitting it to you at a later date for consideration or just for your files later...thanks again, terry

 

snip...end...tss

 

From: Terry S. Singeltary Sr. [mailto:flounder9@verizon.net]

 

Sent: 23 November 2013 03:39

 

To: OfficialReport

 

Cc: Public Information

 

Subject: Fw: Wasting disease is threat to the entire UK deer population

 

snip...end...tss

 

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

 

From: Terry S. Singeltary Sr.

 

Sent: Sunday, July 21, 2013 1:44 PM

 

To: endemics@wales.gsi.gov.uk

 

Cc: wag-en@mailuk.custhelp.com ; Carwyn.jones@wales.gsi.gov.uk ; Correspondence.Alun.Davies@Wales.gsi.gov.uk ; Correspondence.Carl.Sargeant@Wales.gsi.gov.uk ; Correspondence.Edwina.Hart@Wales.gsi.gov.uk ; Correspondence.Huw.Lewis@Wales.gsi.gov.uk ; Correspondence.Jane.Hutt@Wales.gsi.gov.uk ; Correspondence.John.Griffiths@Wales.gsi.gov.uk ; Correspondence.Lesley.Griffiths@Wales.gsi.gov.uk ; Correspondence.Mark.Drakeford@Wales.gsi.gov.uk ; Correspondence.Jeff.Cuthbert@Wales.gsi.gov.uk ; Correspondence.Vaughan.Gething@wales.gsi.gov.uk ; Correspondence.Ken.Skates@wales.gsi.gov.uk ; Correspondence.Gwenda.Thomas@Wales.gsi.gov.uk

 

Subject: Welsh Government and Food Standards Agency Wales Joint Public Consultation on the Proposed Transmissible Spongiform Encephalopathies (Wales) Regulations 2013

 

July 21, 2013

 

Subject: Welsh Government and Food Standards Agency Wales Joint Public Consultation on the Proposed Transmissible Spongiform Encephalopathies (Wales) Regulations 2013

 

endemics@wales.gsi.gov.uk; fenris@caramail.com;

 

Greetings Welsh Government and Food Standards Agency Wales,

 

With great urgency, I would kindly like to comment on ;

 

Number: WG18417

 

Joint Public Consultation on the Proposed Transmissible Spongiform Encephalopathies (Wales) Regulations 2013

 

The European Food Safety Authority (EFSA) and the European Centre for Disease Prevention and Control jointly advised in 2011 that BSE is the only animal TSE that has been shown to be a risk to human health and that there is no epidemiological evidence to suggest that classical scrapie is a risk to human health.

 

and

 

What are the main issues under consideration?

 

2.1 The main issues under consideration relate to changes in BSE testing requirements; more proportionate measures for controlling classical scrapie in sheep flocks and goat herds in which classical scrapie is confirmed; and more proportionate controls on animal feed. The Welsh Government also wishes to consult on proposed amendments to the BSE cattle compensation system in light of identified anomalies in the current system, in addition to a variety of other proposed technical and procedural amendments to the 2008 Regulations.

 

2.2 The key specific amendments are summarised and then considered in more detail below. Other proposed technical amendments, which are considered to have a negligible impact or have already been implemented administratively, are listed at Annex A. What are the main issues under consideration?

 

2.1

 

* The main issues under consideration relate to changes in BSE testing requirements;

 

* more proportionate measures for controlling classical scrapie in sheep flocks

 

* and goat herds in which classical scrapie is confirmed; and

 

* more proportionate controls on animal feed.

 

Greetings again Welsh Government and Food Standards Agency Wales, with another TSE prion medical blunder happening just this past week ;

 

Friday, July 19, 2013

 

Beaumont Hospital in Dublin assessing patients for CJD

 


 

I STRENUOUSLY urge you take my submission with the greatest urgency. My submission and concerns as follows, and in part mixed in and throughout the WG18417 Proposed Transmissible Spongiform Encephalopathies (Wales) Regulations 2013. First and foremost, I think it is very important for the Welsh Government and Food Standards Agency Wales, to take a full inventory of your imports from North America, and other Countries, especially your pet and fish foods, for reasons and scientific facts I have supplied below. I think the attempt by Governments around the globe to do away with the Transmissible Spongiform Encephalopathy TSE prion disease, via weakening of the BSE TSE prion surveillance, lowering of age limits in testing, weakening the feed bans, caving in to industry, ignoring all the science of the past 3 decades, ignoring these different atypical TSE prion disease breaking out, making up new names for these TSE prion disease, and by the way, what ever happened to the IBNC BSE, and the pathology there, and what about testing there from?

 

The USDA, CFIA, with the help of the OIE, have made it there goal to extinguish all BSE TSE prion trade barriers, before all the science on the TSE prion disease is in, and are working hard to exempt all TSE prion in all species from any trade barrier, and the OIE is working right along with them. This happened December 2003, when the USA lost it’s OIE BSE Gold Card, when BSE was first documented in the USA, after a long hard fought battle trying to cover mad cow disease up. This proven time and time again by the OIG and the GAO of the USA. I will supply url links of submissions I have made to the USDA et al over the years, since the death of my Mother to the Heidenhain Variant of Creutzfeldt Jakob Disease i.e. hvCJD ‘confirmed’ 12/14/97. just another happenstance of bad luck they tell me, that it only happens in the UK, and that it’s a UK disease, this mad cow disease, that no other animal species TSE prion disease in the world, at no other location, can transmit a TSE prion disease to a human, and then basing all trading protocol from country to country, based on this junk science, destroys the past 3 decades of trying to eradicate the damn disease, and the OIE, USDA, CFIA, and all the other countries that know better, that just goes along with this due to trade purposes, just because it’s a long incubating disease, just because the science is still in it’s infancy, does not mean we should start ignoring what early science has taught us, and start weakening any safety protocols there from.

 

North America has the most documented TSE prion disease in the wild and in farmed livestock than any other country in the world, excluding the TSE prion disease documented in zoo animals. Chronic Wasting Disease CWD in cervids is running rampant, the USA and Canada can’t stop it, while Mexico has not a clue of any TSE prion disease. The shooting pens, and CWD there from in the USA, is a real risk factor, one the game farms refuse to admit they are a big problem with the spreading of the CWD TSE prion disease, fighting tooth and nail completely ignoring the evolving science on the CWD TSE prion disease, and how it’s spread, and these antler mills are multiplying from state to state in big numbers. There is ample evidence of CWD transmission to humans, to warrant a warning to the world, of the IATROGENIC potential for this TSE prion disease in cervids, via the multitude of potential routes of infection, via the medical, dental, surgical, blood, tissue. CWD has now mutated to multiple strains. The science is there to warrant this very real concern, it’s just the same as with what happened in the U.K., the industry and USDA inc., are stopping these concerns to be made public, with the same watered down junk science used in the beginning of the BSE blunder. you should all be very aware of this, if you come abroad to North America. DEFRA has put out a warning on CWD TSE prion disease in the USA and have put out a document I supplied with additional risk factors from North America, this is supplied below as well.

 

Another concern is with your assumptions that typical classical scrapie is not a risk factor for humans, when there _is_ evidence to show otherwise, that indeed typical scrapie is a risk factor for humans, as with atypical Scrapie. The OIE, USDA inc, and the CFIA, have come to the conclusion that neither typical scrapie nor the atypical Nor-98 scrapie are neither a risk factor for humans, they have urged the OIE to conclude that atypical Nor-98 to be exempt from any trading protocols, and indeed have made the Nor-98 atypical scrapie EXEMPT, and made it legal to trade, and they are also in the works to make typical scrapie exempt.

 

typical scrapie consist of many different strains of scrapie, not just one. and the atypical Nor-98 has very similar features with human Gerstmann-Sträussler-Scheinker Disease GSS and Variably Protease-Sensitive Prionopathy VPSPr. I have not seen anywhere in the Bible, or the scrolls of the Dead Sea, where it was stipulated that indeed typical c-BSE is the only zoonosis Transmissible Spongiform Encephalopathy TSE prion disease. This is ludicrous in 2013 to still believe this junk science.

 

With the science to date of the 1st 10 nvCJD victims of Dr. Ironside et al, and the diagnostic criteria then to diagnose the nvCJD, compared to what Dr. Gambetti et al diagnosed in their 1st 10, of which young victims are being diagnosed, but yet changed the name to VPSPr type CJD human TSE, is not scientific in my opinion. I believe that the UKBSEnvCJD only theory is bogus, it is not scientific, and should be put to bed once and for all. you cannot have your cake and eat it too. either Ironside was wrong, or Gambetti is wrong. to continue this UKBSEnvCJD only myth, will only help continue spread the TSE prion agent long and far.

 

typical Scrapie has been studied for decades and decades, and has proven to be transmitted to non-human primates by their NON-FORCED oral consumption (Gibbs et al). when you write in absolute terms as this is fact that ‘’BSE is the only animal TSE that has been shown to be a risk to human health and that there is no epidemiological evidence to suggest that classical scrapie is a risk to human health’’ may be true in terms of documentation, but in terms of science to date, I think you are wishing. I kindly wish to submit the following in good faith ;

 

snip...end...tss

 

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

 

Friday, November 22, 2013

 

Wasting disease is threat to the entire UK deer population

 

Wasting disease is threat to the entire UK deer population CWD TSE PRION disease in cervids

 

***SINGELTARY SUBMISSION

 

The Scottish Parliament’s Rural Affairs, Climate Change and Environment Committee has been looking into deer management, as you can see from the following press release,

 

***and your email has been forwarded to the committee for information:

 


 

Sunday, July 21, 2013

 

Welsh Government and Food Standards Agency Wales Joint Public Consultation on the Proposed Transmissible Spongiform Encephalopathies (Wales) Regulations 2013

 

*** Singeltary Submission WG18417

 


 

PREVIOUS ASSESSMENT 2012

 

This assessment has been provided as an update to a previous version carried out in 2012, due to new evidence about the import of lures for deer hunting which contain deer urine. The previous assessment is available at:

 


 

Monday, May 05, 2014

 

Member Country details for listing OIE CWD 2013 against the criteria of Article 1.2.2., the Code Commission recommends consideration for listing

 


 

Tuesday, July 17, 2012

 

O.I.E. BSE, CWD, SCRAPIE, TSE PRION DISEASE Final Report of the 80th General Session, 20 - 25 May 2012

 


 

Monday, January 4, 2016

 

Long live the OIE, or time to close the doors on a failed entity?

 


 

*** Needless conflict ***

 

Nature 485, 279–280 (17 May 2012) doi:10.1038/485279b

 

Published online 16 May 2012

 

Terry S. Singeltary Sr. said:

 

I kindly wish to submit the following please ;

 


 

"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.

 


 

Thursday, October 22, 2015

 

*** Former Ag Secretary Ann Veneman talks women in agriculture and we talk mad cow disease USDA and what really happened ***

 


 

Thursday, January 14, 2016

 

*** EMERGING ANIMAL DISEASES Actions Needed to Better Position USDA to Address Future Risks Report to the Chairman, Committee on Energy and Commerce, House of Representatives December 2015 GAO-16-132

 

GAO

 


 

Thursday, March 24, 2016

 

FRANCE CONFIRMS BOVINE SPONGIFORM ENCEPHALOPATHY BSE MAD COW (ESB) chez une vache dans les Ardennes

 


 

Monday, March 28, 2016

 

National Scrapie Eradication Program February 2016 Monthly Report

 


 

Thursday, February 25, 2016

 

U.S. Food & Drug Administration (FDA) FDA/CFSAN Cosmetics Update: Cosmetics Program; Import and Domestic and Transmissible Spongiform Encephalopathy TSE Prion Disease Risk Factors

 

***WARNING TO ALL CONSUMERS AND COUNTRIES AROUND THE WORLD***

 

***Note: FDA labs do not conduct BSE analysis and thus no sampling guidance is issued for BSE. ***

 


 

Friday, February 05, 2016

 

Report of the Committee on Wildlife Diseases FY2015 CWD TSE PRION Detections in Farmed Cervids and Wild

 


 

Saturday, February 6, 2016

 

*** Secretary's Advisory Committee on Animal Health; Meeting [Docket No. APHIS-2016-0007] Singeltary Submission ***

 


 

Tuesday, March 15, 2016

 

Docket No. FDA-2016-N-0321 Risk Assessment of Foodborne Illness Associated with Pathogens from Produce Grown in Fields Amended with Untreated Biological Soil Amendments of Animal Origin; Request for Comments, Scientific Data, and Information Singeltary Submission

 


 

Friday, March 18, 2016

 

CFSAN Constituent Update: FDA Announces Final Rule on Bovine Spongiform Encephalopathy BSE MAD COW TSE PRION

 

Center for Food Safety and Applied Nutrition - Constituent Update

 


 

PLEASE REMEMBER, IN 55 YEARS AND OLDER, THE RATE OF DOCUMENTED CJD JUMPS TO ONE IN 9,000. but officials don’t tell you that either. carry on...

 

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.

 


 

26 March 2003

 

Terry S. Singeltary, retired (medically) CJD WATCH

 

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?

 


 

2 January 2000

 

British Medical Journal

 

U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well

 


 

15 November 1999

 

British Medical Journal

 

vCJD in the USA * BSE in U.S.

 


 

The Lancet Infectious Diseases, Volume 3, Issue 8, Page 463, August 2003 doi:10.1016/S1473-3099(03)00715-1Cite or Link Using DOI

 

Tracking spongiform encephalopathies in North America

 

Original

 

Xavier Bosch

 

“My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem.” 49-year—old Singeltary is one of a number of people who have remained largely unsatisfied after being told that a close relative died from a rapidly progressive dementia compatible with spontaneous Creutzfeldt—Jakob ...

 


 
 
Terry S. Singeltary Sr. Bacliff, Texas USA 77518 flounder9@verizon.net

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