Bulaşıcı süngerimsi ensefalopatiler: halk sağlığı açısından güncel bir bakış.

Bulașıcı süngerimsi ensefalopatiler veya prion hastalıkları, insanları ve hayvanları etkileyen ilerleyici nörodejeneratif hastalık grubudur. Nörolojik belirtiler, santral sinir sisteminde olușan nöron kaybı, glial aktivasyon ve süngerimsi değișiklikleri içermektedir. Prion hastalıklarının memeliler ve insanlar için ölümcül olması nedeniyle, halk sağlığı açısından acil tedavi ve koruma stratejilerinin geliștirilmesine ihtiyaç duyulmaktadır. Bu yazıda bulașıcı süngerimsi ensefalopatilerin etiyolojisi, patogenezi, klinik belirtileri, epidemiyolojisi ve korunması konusunda güncel bilgiler sunulmuștur.

Transmissible spongiform encephalopathies: a current perpective for public health

Transmissible spongiform encephalopathies or prion diseases are a group of progressive neurodegenerative diseases that affect both humans and animals. The neuropathological features include neuronal loss, glial activation and spongiform changes in the central nervous system. As prion diseases are fatal for both mammalian and human, there is need for urgent therapeutic and prophylactic strategies in relation to public health. Here, current information about etiology, pathogenesis, clinic features, epidemiology and prevention of transmissible spongiform encephalopathy are summarized.

Kaynakça

1. World Health Organization. Food safety and foodborn illness. Fact Sheet, No: 237, http://www.who.int/mediacentre/en.

2. Prusiner SB. Shattuck lecture: Neurodegenerative disease and prions. N Engl J Med 2001; 344: 1516-26.

3. Schneider K, Fangerau H, Michaelsen B, et al. The early history of the transmissible spongiform encephalopaties exemplifi ed by scrapie. Brain Res Bull 2008; 77: 343-55.

4. Prusiner SB. Novel proteinaceous infectious particles cause scrapie. Science 1982; 216: 136-44.

5. Sparkes RS, Simon M, Cohn VH, et al. Assignment of the human and mouse prion protein genes to homologous chromosomes. Proc Natl Acad Sci USA 1986; 83: 7358-62.

6. Kretzschmar HA, Storwing LE, Westaway D, et al. Molecular cloning of a human prion protein cDNA. DNA 1986; 5: 315-24.

7. Cohen F, Prusiner SB. Pathologic conformations of prion proteins. Annu Rev Biochem 1998; 67: 793-819.

8. Sakudo A, İkuta K. Fundamentals of prion diseases and their involvement in the loss of function of cellular prion protein. Protein Peptide Lett 2009;16: 217-29.

9. Kralovicova S, Fontaine SH, Alderton A, et al. The effects of prion protein expression on metal metabolism. Mol Cel Neurosci 2009; 41: 135-47.

10. Giles K, Glidden DV, Beckwith R, et al. Resistance of bovine spongiform encephalopathy (BSE) prions to inactivation. PLoS Pathog 2008; 5: 1-9.

11. Taylor D M. İnactivation of transmissible degeneratif encephalopathy agents: a review. Vet J 2009; 159: 10-7.

12. Taylor DM. Inactivation of the BSE agent. CR Biol 2002; 325: 75-6.

13. Chakraborty C, Nandi S, Jana S. Prion disease: a diedly disease for protein misfolding. Curr Pharml Biotechno 2005; 6: 167-77.

14. Mabbott NA, MacPherson GG. Prions and their lethal journey to the brain. Microbiology 2006; 4: 201-11.

15. Collins SJ, Lawson VA, Masters CL. Transmissible spongiform encephalopathies. Lancet 2004; 363: 51-61.

16. Weels GA, Scott AC, Johnson CT, et al. A novel progressive spongiform encephalopathy in cattle. Vet Rec 1987; 121: 419-20.

17. Anderson RM, Donnelly CA, Ferguson NM. Transmission dinamics and epidemiology of BSE in British Cattle. Nature 1996; 382: 779-87.

18. McGill İS, Weels GA. Neuropathological fi ndings in cattle with clinically suspect but histologically unconfi rmed bovine spongiform encephalopathy (BSE). J Comp Pathol 1993; 108: 241-60.

19. Agerholm JS, Tegtmeier CL, Nielsen TK. Survey of laboratory fi ndings in suspected cases of bovine spongiform encephalopathy in Denmark from 1990-2000. APMIS 2002; 110: 54-60.

20. Miyashita M, Stierstorfer B, Schmahl W. Neuropathological fi ndings in brains of Bavarian cattle clinically suspected of bovine spongiform encephalopathy. J Vet Med 2004; 51: 209-15.

21. İronside JW, Ritchie DL, Head MW. Phenotypic variability in human prion disease. Neuropathol Appl Neurobiol 2005; 31: 565-79.

22. Will RG, İronside JW, Zeidler M. A new variant of Creutzfeldt- Jakob disease in the UK. Lancet 1996; 347: 921-5.

23. Peden AH, Head MW, Ritchie DL, et al. Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet 2004; 364: 527-9.

24. Health Protection Agency. New case of transfusion-associated variant - CJD. CDR Weekly 2006;16 (6).

25. Hewitt PE, Liewelyn CA, Mackenzie J, et al. Creutzfeldt-Jakob disease and blood transfusion: Results of the UK transfusion medicine epidemiological review study. Vax Sang 2006; 91: 221-30.

26. Boelle P, Thomas G, Valleron AJ, et al. Modelling the epidemic of variant Creutzfeldt-Jakob disease in the UK based on age characteristics: Updated, detailled analysis. Stat Methods Med Res 2003; 12: 221-33.

27. Zeidler M, Stewart G, Cousens SN. Codon 129 genotype and variant CJD. Lancet 1997; 350: 668.

28. Hill AF, Butterworth RJ, Joiner S, et al. İnvestigation of variant Creutzfeldt-Jakob disease and other human prion diseases with tonsil biopsy samples. Lancet 1999; 353: 183-9.

29. Centers for Disease Control and prevention. vCJD Fact Sheet. (November 2006). Last Access: 20.02.2011.

30. Sakudo A, Nakamura İ, İkuta K, et al. Recent developments in prion disease research: Diagnostic tools and in vitro cell culture models. J Vet Med Sci 2007; 69: 329-37.

31. Saa P, Castilla J, Soto C. Presymptomatic detection of prions in blood. Science 2006; 3: 92-4.

32. Castilla J, Saa P, Soto C. Detection of prions in blood. Nat Med 2005;11: 982-5.

33. Sim VL, Caughey B. Recent advances in prion chemotherapeutics. Infectious Disorders -Curr Drug Targets 2009; 9: 81-91.

34. Sakaguchi S. Prospects for preventative vaccines against prion disease. Protein Peptide Lett 2009; 16: 260-70.

35. MAFF. Bovine spongiform encephalopathy in Great Britain: A progress report, 1997. http://archive.defra.gov.uk/foodfarm/farmanimal/diseases/ atoz/bse/publications/ Last Access: 21.02.2011.

36. Boschert K, Gill B. Germany’s agri-biotechnology policy: Precaution for choice and alternatives. SPP 2005; 32: 285-92.

37. World Health Organization. WHO Tables on tissue infectivity distribution in transmissible spongiform encephalopathies. Updated 2010. http://www.who.int/bloodproducts/tablestissueinfectivity.pdf

38. Ludlam CA, Turner ML. Managing the risk of transmission of variant Creutzfeldt- Jakob disease by blood products. BJH 2005; 132: 13-24.

39. FDA. Guidance for Industry: Revised Preventive Measures to Reduce the Possible Risk of Transmission of Creutzfeldt- Jakob Disease (CJD) and New Variant Creutzfeldt-Jakob Disease (nvCJD) by Blood and Blood Products. May 2002. http://www.fda.gov/BiologicsBloodVaccines/ GuidanceComplianceRegulatoryInformation/Guidances/ Blood/ucm074089.htm Last access 20.02.2011.

40. T.C. Sağlık Bakanlığı. C02. Yeni varyant Creutzfeldt-Jakob Hastalığı. http://www.saglik.gov.tr/TR/belge/1-6542/genel-bilgiler.html Last access: 20.02.2011.

41. T.C. Tarım ve Köyişleri Bakanlığı. Sığır eti ithalatında kontrol belgesi alınabilmesi için aranacak şartlar hakkında tebliğde değişiklik yapılmasına dair tebliğ (no:2010/56). http://www.resmi-gazete.org/tarih/20110114-1.htm Last access: 20.02.2011.

42. T.C. Tarım ve Köyişleri Bakanlığı. Sığır cinsi hayvanların tanımlanması, tescili ve izlenmesi Yönetmenliği. Resmi gazete, sayı: 24829, 28.07.2002. http://mevzuat.basbakanlik.gov.tr/Metin.Aspx Last access: 20.02.2011.

43. The European and Allied Countries Colloborative Study Group of CJD (EUROCJD) plus the Extended Collaborative Study Group of CJD (NEUROCJD) http://www.eurocjd.ed.ac.uk/ Last access 20.07.2011.

44. Bons N, Mestre-Frances N, Belli P, et al. Natural and experimental oral infection of non-human primates by bovine spongiform encephalopathy agents. Proc Natl Acad Sci USA 1999; 96: 4046-51.

45. Beghi E, Gandolfo C, Ferrarese C, et al. Bovine spongiform encephalopathy and Creutzfeldt-Jakob disease: facts and uncertainties underlying the causal link between animal and human disease. Neurol Sci 2004; 25: 122-9.

46. Coste J, Prowse C, Elgin R, et al. A report on transmissible spongiform encephalopaties and transfusion safety. Vox Sang 2009; 96: 284-91.

Kaynak Göster