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Draft High Level Control Strategy for Finding BSE in Sheep or Goats

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DescriptionThe new control strategy will replace the plan that was developed and consulted upon in 2004. The draft control strategy takes into account developments, since 2004, in scientific understanding and of the FSA's strateegy to protect public health.
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Website Publication DateNovember 07, 2008

High Level UK Control Strategy for the Emergence of Naturally Occurring BSE (or other Zoonotic TSEs) in Sheep or Goats

Background

Introduction

1. Regulation (EC) No 999/2001 requires Member States to draw up contingency plans specifying the measures that they would implement where cases of transmissible spongiform encephalopathy (TSE) are confirmed. This control strategy sets out the actions that would be taken in response to finding Bovine Spongiform Encephalopathy (BSE) occurring naturally in sheep or goats.

Aim and Scope

2. This control strategy replaces the plan consulted on and published in June 2004 [1]. It takes into account the developments since 2004 in scientific understanding and of the public health protection strategy that would be employed should BSE be found in sheep or goats.

3. This strategy outlines the science, the process that would be followed to confirm a suspect finding of BSE in sheep or goats, the initial response and the key aspects that would require consideration when developing operational plans to protect the public health and the sheep industry should BSE be found occurring naturally in sheep or goats in the UK. This strategy also provides a basis for considering what action to take should, in the future, other TSEs of sheep and goats be identified as zoonotic (capable of transmitting to humans). To date, BSE is the only zoonotic TSE that has been identified (see paragraph 6).

4. Unlike the previous plan, contingency arrangements are not described in detail. This is because the strategy that has been developed to protect the public health on finding BSE in UK sheep or goats is adaptable to the circumstances at the time and the assessment of the risk. For this reason, this strategy sets out at a high level the key aspects that would need to be considered in developing operational plans to respond to the events at the time should BSE be found in sheep or goats. Since BSE is not a highly infectious disease, like Foot and Mouth Disease for example, there would be time for operational plans to be developed as the situation presents itself at the time. Furthermore, currently definitive confirmation of BSE in sheep or goats may require mouse bioassays that can take two, if not more, years to complete. This high level strategy has also been developed against a background of the very low and diminishing likelihood that BSE will be found occurring naturally in sheep or goats in the UK (see paragraph 12).

5. This high level strategy will be kept under review in light of developments in scientific understanding, in UK Government and European Commission policy and in the capability of the UK to put in place systems to manage the risk should BSE be found in sheep or goats.

Science

6. At present there are three distinct TSEs known to occur in sheep and goats: classical scrapie, atypical scrapie and BSE. Of these diseases, only BSE is known to be zoonotic [2], [3]. However, there is diversity in the properties of the TSE strains that infect sheep, goats and other ruminants2 and TSE strains could potentially change to give rise to new strains with altered properties that cannot be predicted [4]. For these reasons, it is possible that other TSE strains may be identified as zoonotic in the future.

7. BSE in cattle was first recognised in 1986 and was later identified as the causative agent of variant Creutzfeldt-Jakob Disease in humans [5], [6]. In the UK, feed controls were first introduced in 1988, enhanced in 1994 and reinforced in 1996 to prevent exposure of cattle, sheep and goats to the BSE agent [7]. BSE has never been confirmed to be naturally occurring in sheep. However, there remains a possibility that UK sheep and goats may have been infected with BSE in the past and that a low level of infection may have persisted via horizontal transmission (transmission between sheep or goats in the field). This is because: (i) the UK sheep flock and goat herd were exposed in the past to contaminated feedstuffs, similar to those that gave rise to the BSE epidemic in cattle, albeit at a much lower level [8], (ii) sheep can be infected experimentally with BSE by the oral route [9], [10], (iii) BSE has been confirmed in a French goat [11] and was probably present in a UK goat originally diagnosed with classical scrapie in 1990 [12], (iv) BSE has been transmitted between sheep in an experimental setting [13] and (v) the clinical signs of BSE in sheep and goats resemble those of classical scrapie so it could have remained undetected [14] until post mortem tests to distinguish the two diseases became available and their use made mandatory in the European Union (EU) in 2005.

8. Unlike the BSE agent in cattle, the BSE agent in sheep is widely distributed through the body within the lymphoid system in a similar manner to the classical scrapie agent in sheep [15]. Data on the distribution of the BSE agent in goats are lacking. However, should the BSE agent behave similarly to the classical scrapie agent in goats [16], it may also be widely distributed. For this reason, the removal of specified risk material (SRM) (tissues that may carry the BSE agent) from sheep and goats as a public health protection measure that is as effective as SRM removal from cattle would be much more difficult to achieve. If the BSE agent in sheep behaves in a similar manner to the classical scrapie agent, the BSE agent may also transfer to sheep milk [17], and this may also be true for goats' milk.

9. TSE agents accumulate slowly in the body. Therefore, the public health risk from animals entering the food supply that may be infected, but not showing signs of infection, would generally increase with the age of the animals [18]. For this reason, restrictions on the age of animals entering the food supply can also be used to reduce the risk to public health.

10. There are appreciable differences in the genetic susceptibility of sheep to TSEs. There are forms of the prion protein gene that confer a high resistance (the ARR homozygote genotype) or semi-resistance (ARR heterozygote genotypes) to BSE [19]. This genetic resistance provides the opportunity for public health protection strategies that allow animals of the resistant genotype or semi-resistant genotypes into the food chain. The genetic susceptibility of goats to TSEs is not sufficiently well understood to provide a basis for similar strategies for goats. There may be differences in the relative susceptibilities of sheep and goats to BSE, irrespective of genotype, but these are also not well understood.

11. An extensive survey of samples of UK sheep and goat TSE cases collected from 1998 onwards, using tests that are able to distinguish BSE from classical scrapie, has not found any cases of BSE in sheep and goats. The EU currently requires that 10 000 sheep over the age of 18 months are slaughtered for human consumption and 10 000 fallen sheep and 500 fallen goats over the age of 18 months are tested for the presence of TSEs in the UK. All sheep or goat TSE cases are tested for the possible presence of BSE.

12. The Spongiform Encephalopathy Advisory Committee (SEAC) concluded in 2007 that estimates based on the survey of sheep TSE cases suggest that the prevalence of BSE in sheep may be zero and in the worse case no more than 10 flocks would be affected [20]. The European Food Safety Authority (EFSA) also concluded in 2007 that the prevalence of BSE in sheep may be zero or, if present, very low, from analysis of pan-European data [21]. Thus, the likelihood that BSE is found to be occurring naturally in sheep or goats currently is very low and this likelihood is diminishing the longer no cases of BSE in sheep and goats are found.

13. Although finding BSE in the UK sheep flock or goat herd is highly unlikely, it cannot be ruled out. For this reason, this high level control strategy has been developed in order to facilitate operational planning should BSE in sheep or goats be found. Although what follows is directly applicable to BSE in sheep or goats, it could also be used as a basis for the development of a response should other zoonotic TSEs of sheep and goats emerge in the future.

Scenarios for finding BSE in sheep or goats

14. BSE in sheep or goats, if it is present currently, could be found during the course of:

  • active or passive surveillance of sheep and goats in the UK
  • surveillance conducted by other countries on their own sheep flocks or goat herds

Cases identified during the course of UK surveillance

15. The action required upon the identification by active or passive surveillance of a suspect TSE in sheep or goats is specified in Regulation (EC) No 999/2001 and amendments. The presence, or not, of a TSE would first be confirmed by the Veterinary Laboratories Agency (VLA) using an approved confirmatory test. If the presence of a TSE is confirmed, discriminatory testing is undertaken by the VLA to identify the TSE and to investigate whether the TSE is BSE with the results available within days. If, from the results of this testing, BSE cannot be excluded, the Community Reference Laboratory (CRL), which is the VLA, would circulate samples to members of the CRL Strain Typing Expert Group (STEG) for further testing using a range of discriminatory tests (a ring trial). The results of the ring trial would be interpreted by the CRL in consultation with STEG with the conclusions available within weeks of circulating the samples. The UK Government and European Commission would be informed of the outcome. If BSE cannot be excluded following the ring trial tests, samples would be tested by mouse bioassays to allow a definitive diagnosis to be reached, which could take two, if not more, years to complete.

16. In circumstances where the CRL and STEG consider that the presence of BSE can be confirmed, SEAC would be convened as soon as practicable to assess the risk at the UK level. SEAC would take into account the circumstances of the case (e.g. age, flock of origin, movements, feeding and husbandry practices) and the results of investigations of the possible origins and spread of the infection (see section 'Action on the sheep flock / goat herd of origin'). SEAC advice on the risk may also be sought under circumstances where BSE cannot be excluded by the CRL and STEG.

17. On the basis of the assessment of risk, the Food Standards Agency (FSA) would advise the UK Government on what action, if any, is needed to manage the risk to public health and / or clarify the level of risk. Decisions on what measures to take would be made by Ministers. Should Ministers decide to implement the measures recommended by the FSA, the UK Government, Devolved Administrations and other relevant organisations would respond by developing detailed operational plans to implement the measures (see the 'Development of operational plans' section below).

18. Should a finding of BSE in sheep or goats be confirmed, it is likely that the Commission would also ask EFSA to assess the risk at the EU level to formulate an EU-wide response.

Finding BSE in sheep or goats outside the UK

19. A finding of BSE in sheep or goats in another country would not trigger any immediate action to protect public health in the UK, although changes to, for example, surveillance may be required by the Commission at a later stage following an assessment of the risk by EFSA. An investigation would be carried out by Defra and the other UK Rural Affairs Departments to determine, if possible, whether any UK sheep flocks or goat herds were linked to the sheep flock(s) or goat herd(s) infected with BSE in the other country. If links were established, the advice of SEAC would be sought as soon as practicable on the potential risk of BSE for those flocks or herds. This advice would inform actions, if any, by the UK Government to manage the risk. In addition, depending on FSA / EFSA advice, action may be taken to remove sheep or goat meat or products from the food supply, if any had been imported from the country where BSE had been confirmed.

Testing archived UK samples

20. There is also a possibility that BSE could be found through the testing of archived samples of sheep or goats in the UK (or elsewhere). However, the number of archived samples, at least in the UK, is likely to be very low and therefore, this scenario would appear to be highly unlikely.

21. The significance of a suspect or confirmed case of BSE identified from testing archived UK sheep or goat samples would be dependent on the date of birth and the date of slaughter of the animal. This information would provide the context of the feed controls in force during the lifetime of the case and give an indication of the timing of BSE exposure. The CRL is likely to involve STEG in ring trial testing and the diagnosis of such cases. In circumstances where BSE is confirmed or cannot be excluded, SEAC would be convened as soon as practicable to assess the significance of these results for the likelihood that BSE may be present in sheep or goats currently. SEAC would also assess the risk at the UK level taking into account any interpretation of the results by CRL and STEG and the circumstances of the case. These assessments would inform decisions by the FSA and the UK Government on what further action, if any, may be necessary, and could draw upon the actions set out in this plan.

22. Should confirmed cases of BSE be found from testing archived samples of sheep or goats elsewhere, the Commission is likely to ask EFSA for an assessment of the risk to inform what action, if any, is necessary at the EU level.

Action on the sheep flock or goat herd of origin

23. EU Regulation (EC) No 999/2001 and amendments sets out the action required to be taken on the sheep flock or goat herd of origin if a TSE is suspected.

24. Firstly, the holding on which the infected animal is present is placed under official control by Animal Health and the movement onto and from the holding of all animals susceptible to TSEs is restricted.

25. Secondly, an epidemiological investigation would be conducted by Animal Health to identify, where possible, the parents, and in the case of an infected female, the embryos, ova and the last progeny of the case. The investigation would also try to identify the possible origins of the infection including other holdings that may have been the source of the infection. It would also try to establish the movements of feed that may have been contaminated or any other possible routes of transmission of the infection both to, and from, the holding. Depending on the epidemiological information available, other holdings could be placed under official control by Animal Health should they be considered to have potentially been the source of the infection or be at risk from spread of the infection. Similar restrictions of movements and investigations would be undertaken by the Department of Agriculture and Rural Development Veterinary Service for Northern Ireland.

26. If BSE cannot be excluded by the CRL and STEG following the results of the ring trial, Animal Health would kill and destroy all the TSE susceptible animals and material from those animals identified by the epidemiological investigation.

Development of operational plans

27. Should Ministers decide to implement the public health protection measures recommend by the FSA following a confirmed finding of BSE in sheep or goats, operational planning to implement those measures would begin.

28. Development of operational plans to implement Ministerial decisions would be the responsibility of a BSE in Sheep/Goats Project Board. This Project Board would be convened by Defra and consist of representatives of the relevant parties involved in communicating, coordinating and planning the implementation and operation of the measures agreed by Ministers. A scheme of the process from identification of a suspect case of BSE in sheep or goats to operational planning is given at Annex A.

29. The Project Board would include the Chief Veterinary Officer (CVO), Deputy CVO, Chief Scientific Advisor, Director of the Food Chain and Endemic Diseases Portfolio, Deputy Director of the Livestock Hub and the Director of Communications from Defra, and representatives from Animal Health, the FSA, the Meat Hygiene Service, the Department of Health, UKREP (United Kingdom Permanent Representation to the European Union) and the Cabinet Office Civil Contingencies Secretariat. The Project Board would also include the CVOs and other representatives from the Scottish Government, Welsh Assembly Government and the Department for Agriculture and Rural Development Northern Ireland. The Project Board would be chaired by the Chief Veterinary Officer for the UK (CVO UK). Annex B sets out the roles and responsibilities of the relevant authorities. The Project Board would be advised by a core external stakeholder group that would be established by the Project Board, which would include representatives from principal stakeholders from the agricultural, veterinary and rural sectors.

30. Key considerations for the Project Board when developing operational plans are set out below. These are set in the context of the FSA's current policy of a graduated response to finding BSE in sheep. This includes a range of public health protection measures that could be applied depending on an assessment of the level of risk.

BSE in sheep

31. The current FSA policy [22] should BSE be confirmed in sheep is to recommend enhancement of surveillance to improve estimates of the prevalence of BSE in sheep. The FSA could also recommend a range of measures to protect public health depending on an assessment of the risk that would be influenced by the number of confirmed cases found. Should multiple cases of BSE be found and considered, on the basis of epidemiological investigations, to be indicative of horizontal transmission, this would increase the level of concern that BSE in sheep is much more widespread than considered currently to be likely (see paragraph 12).

32. The FSA has identified a range of risk reduction measures of increasing effectiveness in reducing the risk to public health, but at increasing cost22. The first level of protection, which the FSA could recommend, depending on the risk assessment made at the time, would be provided by an extension of the list of tissues designated as SRM to include all thoracic viscera, excluding the heart, all abdominal viscera, stomach contents, pre-crural fat and all accessible lymph nodes on to the current list of SRM [23]. The FSA could, in addition to extending the SRM controls, recommend more stringent measures depending on the risk assessment and the level of concern about the number of sheep with BSE. These could include exclusion of sheep aged over 12 months from the food supply or only allowing sheep that are most genetically resistant to BSE or semi-resistant sheep aged under 12 months into the food supply. The FSA would also consider the level of risk posed by sheep milk and milk products.

33. On the basis that initial action is most likely to involve the implementation of extended SRM controls, given the very low estimated prevalence of BSE in sheep (see paragraph 12), the Project Board, in consultation with the core external stakeholder group, would need to consider some or all of the following issues in drawing up operational plans:

  • design and implementation of an enhanced TSE surveillance programme to provide better estimates of the prevalence of BSE in sheep and / or goats and of the epidemiological investigations to determine the likelihood of horizontal transmission of BSE. The Project Board would convene an expert group to provide epidemiological advice on how best to enhance and target surveillance to obtain reliable estimates of the prevalence of BSE in sheep and / or goats as quickly as practicable.
  • liaison with the Commission and EFSA (see section on 'Role of the Commission').
  • disposal of an increased volume of SRM.
  • identification of, and communication with, sheep and / or goat keepers and communications with veterinarians, the agricultural community, the wider industry and the public (see section on 'Communications').
  • the effect on the industry of a possible reduction in public confidence in UK sheep meat and products both in the UK and internationally.
  • reconsideration of breeding strategies to increase the resistance of the UK sheep flock to BSE and certification of suitably resistant sheep. Advice would be sought from the National Standing Committee on Farm Animal Genetic Resources in relation to conservation of breeds.

34. The FSA would lead on:

  • advice to consumers on the safety of sheep and goat meat and food products and, if necessary, the coordination with Local Authorities of the possible withdrawal of sheep and/or goat products already within the food supply.
  • the regulatory impact of the new measures.
  • legislative changes to implement and enforce new SRM controls and consultation on the legislative changes.
  • changes to the practices at cutting plants to ensure effective removal of all the SRM specified on the extended list, including training and supervision of staff.
  • monitoring compliance with the new SRM controls.
  • the need for safety checks of food or other products destined for export.

35. Other departments would lead on the formulation and dissemination of advice to the users of, and on the possible withdrawal of, sheep or goat products in other supply chains: consumer products (Department for Business, Enterprise and Regulatory Reform), medicinal / medical products (UK Health Departments and the Medicines and Healthcare products Regulatory Agency) and veterinary medicines / medical products (Veterinary Medicines Directorate). The Health and Safety Executive would lead on any occupational health issues that might arise.

36. The Project Board would also consider whether detailed contingency plans should be developed to respond to circumstances where the level of risk to public health is judged to have increased such that the more stringent public health protection measures that the FSA could recommend may need to be implemented. These would be more complicated to implement and have a greater impact on the industry.

BSE in goats

37. In the UK, there are considerable differences in the number [24] and in the management of sheep and goats. Given these differences and also the possible differences in the susceptibility of sheep and goats to BSE, the implications of a confirmed finding of BSE in a goat for the presence of BSE in the UK sheep flock or goat herd would need to be assessed. On confirmation of finding BSE in a UK goat by the CRL and STEG, SEAC would be convened as soon as practicable to conduct a risk assessment. This would consider the circumstances of the case (e.g. age, flock of origin, movements, feeding and husbandry practices) and the results of the epidemiological investigations of the possible origins and spread of the infection. The advice of SEAC would inform consideration of what further work might be required, if any, to understand the risk better. The advice of SEAC would also inform considerations by the FSA on what further action, if any, may be necessary to protect public health. Should further action be recommended by the FSA, the operational planning set out above could be used as a basis for the UK Government's response.

Role of the European Commission

38. Should BSE be confirmed in sheep or goats in any Member State, the Commission is likely to seek the advice of EFSA on the level of risk posed. The advice of EFSA would inform the Commission's management of the risk at the EU level. The Project Board would work closely with the Commission and other Member States to provide information to facilitate the EFSA assessment and decision making on what action should be taken. As ultimately the Commission will decide upon the action that should be taken by Member States, operational planning in the UK would be heavily influenced by Commission decisions.

Communications

39. A communications team would be formed by the Defra Director of Communications to develop and manage the communications strategy including stakeholder liaison and communications, press office, e-communications, Defra Helpline and internal communications functions. This would be coordinated with communications colleagues from other Project Board members as appropriate.

40. There would be a number of stages at which external communications would be likely to be required, in particular:

  • on confirmation of the presence of BSE in sheep or goats by the CRL and STEG
  • at the time SEAC issues advice on the risk
  • at the time FSA issues recommendations to Government
  • on Ministerial decisions being made on what action to take
  • during the life span of the BSE in sheep/goats Project Board

41. The communications team would keep interested parties informed of developments. The precise nature of communications will depend on the circumstances at the time, and on what Ministers decide, taking into account scientific uncertainties. The external stakeholder group would be kept informed of developments and would be a channel of communication between key stakeholders and the Project Board. Wider stakeholders would be kept informed via updates on the Defra, Devolved Administrations, Animal Health and FSA websites, messages conveyed through the media, and other routes as appropriate.

Arrangements for future review of the plan

Future review of this high level strategy needs to consider the implications of any changes in scientific understanding and the FSA's public health protection strategy. It also needs to consider the impact of any alterations to the systems that are in place currently that may alter the capability of the UK to respond should BSE be found in UK sheep or goats. It is envisaged that this strategy will be kept under review internally. Comments on the strategy can be sent at any time to Ian Murdoch at ian.murdoch2@scotland.gsi.gov.uk or The Scottish Government, Animal Health & Welfare Division, Pentland House, 47 Robb's Loan, Edinburgh, EH14 1TY. Should the strategy be revised radically, comments would be sought from external stakeholders.

[1]Department for Environment, Food and Rural Affiars (2004) The UK contigency plan for the emergence of naturally occurring BSE in sheep.

[2]Opinion of the Scientific Panel on biological hazards (BIOHAZ) on certain aspects related to the risk of Transmissible Spongiform Encephalopathies (TSEs) in ovine and caprine animals. (2007) The EFSA Journal. (2007) 466, 1-10.http://www.efsa.europa.eu/cs/BlobServer/Scientific_Opinion/biohaz_op_ej466_tse_ovine_caprine_en.pdf?ssbinary=true

[3]SEAC (2008) Statement on the potential human health risk from changes to classical scrapie controls. http://www.seac.gov.uk/statements/scrapiestatement080207.pdf

[4]SEAC (2008) Statement on options relating to modification of the total feed ban.http://www.seac.gov.uk/statements/feedban-oct08.pdf

[5]Will et al. (1996) A new variant of Creutzfeldt-Jakob disease in the UK. Lancet. 347, 921-925.

[6]Bruce et al. (1997) Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent. Nature. 389, 498-501.

[7]Feed ban controls. http://www.defra.gov.uk/animalh/bse/controls-eradication/feed-ban.html

[8]Schreuder & Sommerville (2003) Bovine spongiform encephalopathy in sheep. Rev.Sci. Tech. Off. Int. Epiz. 22, 103-120.

[9]Foster et al. (2001) Distribution of the prion protein in sheep terminally affected with BSE following experimental oral transmission. J. Gen. Virol. 82, 2319-2326.

[10]Bellworthy et al. (2008) Oral transmission of BSE to VRQ/VRQ sheep in an experimental flock. Vet. Rec. 130-131.

[11]Eloit et al. (2005) BSE agent signatures in a goat. Vet Rec. 156, 523-524.

[12]Summary Report of the TSE Community Reference Laboratory Expert Group on Strains of 30 April 2008.http://www.defra.gov.uk/vla/science/docs/sci_tse_rl_steg.pdf

[13]Bellworthy et al. (2005) Natural transmission of BSE between sheep within an experimental flock. Vet Rec. 157, 206.

[14]Office International des Epizooties. (2002) Report of the ad hoc group on the hypothetical presence of bovine spongiform encephalopathy in sheep and goats 14-16 January, Paris. OIE, Paris.

[15]Van Keulen et al. (2008) Pathogenesis of bovine spongiform encephalopathy in sheep. Arch. Virol. 153, 445-453.

[16]TSE risk assessment from carcasses of ovine and caprine animals below 6 months of age from TSE infected flocks intended for human consumption. The EFSA Journal. (2008) 719, 1-27. http://www.efsa.europa.eu/cs/BlobServer/Scientific_Opinion/biohaz_op_ej719_tse_carcasses_smru_en,3.pdf?ssbinary=true



[17]Spongiform Encephalopathy Advisory Committee Sheep Subgroup (2007) Report of the 2007 meeting. Paragraphs 34-39.http://www.seac.gov.uk/committee/sheepsubgroupreport07.pdf

[18]Opinion on the TSE risk assessment from carcasses of ovine and caprine animals below 6

months of age from TSE infected flocks intended for human consumption. The EFSA Journal. (2008) 719, 1-27. http://www.efsa.europa.eu/cs/BlobServer/Scientific_Opinion/biohaz_op_ej719_tse_carcasses_smru_en,3.pdf?ssbinary=true

[19]Baylis. (2002) The BSE-susceptible proportion of UK sheep. Vet. Rec. 151, 481-482.

[20]Spongiform Encephalopathy Advisory Committee (2007) SEAC Sheep Subgroup report of 2007 meeting.http://www.seac.gov.uk/committee/sheepsubgroupreport07.pdf

[21]Opinion on the quantitative risk assessment on the residual BSE risk in sheep meat and meat products. The EFSA Journal. (2007) 442, 1-44.http://www.efsa.europa.eu/cs/BlobServer/Scientific_Opinion/biohaz_op_ej442_qra_sheep_en.pdf?ssbinary=true

[22]FSA Board Paper 06/06/04 and 15 June 2006 minutes.

http://www.food.gov.uk/multimedia/pdfs/fsa060604.pdf

http://www.food.gov.uk/aboutus/ourboard/boardmeetings/boardmeetings2006/boardmeeting60612/boardmins15jun06

[23]The current list of SRM includes the skull, including brain, eyes and tonsils, and the spinal cord of animals over 12 months and the spleen and ileum of animals of all ages.

[24]In the UK, there are around 35 million sheep and 90 thousand goats.

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