Bệnh Creutzfeldt-Jakob biến thể (vCJK)

M. Beekes1
1P24 – Transmissible Spongiforme Enzephalopathien, Robert Koch-Institut, Berlin, Deutschland

Tóm tắt

Bệnh Creutzfeldt-Jakob biến thể (vCJK) là một bệnh prion của con người chưa từng được biết đến, xuất phát từ sự lây truyền zoonotic của các tác nhân gây bệnh bệnh não xốp bò (BSE) từ bò. Bệnh này lần đầu tiên được báo cáo vào năm 1995/96 tại Vương quốc Anh. Đến nay, trên toàn thế giới đã có tổng cộng 219 trường hợp vCJK được báo cáo từ bảy quốc gia châu Âu và bốn quốc gia ngoài châu Âu. Tỷ lệ chính xác của các nhiễm trùng vCJK tiềm ẩn hoặc tiền lâm sàng vẫn chưa rõ ràng. Mặc dù việc kiểm soát BSE đã đạt hiệu quả và các phương thức lây truyền zoonotic đã bị ngắt quãng thành công, hiện vẫn tồn tại một rủi ro lý thuyết về việc lây truyền thứ cấp vCJK giữa con người, chẳng hạn như qua máu và sản phẩm từ máu, cơ quan và mô, hoặc qua các dụng cụ phẫu thuật và sản phẩm y tế bị ô nhiễm. Đến nay, có bốn trường hợp được báo cáo về việc lây truyền vCJK có khả năng qua máu, trong một trường hợp khác, bệnh này có thể đã được truyền qua một chế phẩm huyết tương. Bài viết này cung cấp cái nhìn tổng quan về tình hình dịch tễ học của vCJK và nêu bật việc giám sát, đánh giá rủi ro cũng như quản lý rủi ro của căn bệnh prion mới này liên quan đến BSE ở người.

Từ khóa

#Bệnh Creutzfeldt-Jakob #vCJK #bệnh prion #BSE #lây truyền zoonotic #dịch tễ học

Tài liệu tham khảo

Will RG (2001) Portrait der neuen Variante der Creutzfeldt-Jakob-Krankheit (nvCJD). In: Hörnlimann B, Riesner D, Kretzschmar H (Hrsg) Prionen und Prionkrankheiten. De Gruyter, Berlin New York, S 152–157 Will RG, Ironside JW (2006) Portrait of Variant Creutzfeldt-Jakob disease. In: Hörnlimann B, Riesner D, Kretzschmar H (Hrsg) Prions in humans and animals. De Gruyter, Berlin New York, S 204–209 Prusiner SB (1998) Prions. Proc Natl Acad Sci U S A 95:13363–13383 Beekes M, McBride PA (2007) The spread of prions through the body in naturally acquired transmissbile spongiform encephalopathies. FEBS J 264:588–605 The European and allied countries collaborative study group of CJD (2010) Cases of vCJD worldwide (as of February 2010). http://www.eurocjd.ed.ac.uk/vcjdworldeuro.htm Beekes M, Mielke M, Kurth R (2002) Aspekte zur Risikoabschätzung und Prävention nosokomialer Übertragungen der klassischen und varianten CJK. Internist (Berl) 43:738–748 Beekes M, Kurth R (2002) BSE und Creutzfeldt-Jakob-Krankheit – Gesundheitspolitische Bedeutung für die Bundesrepublik Deutschland und Europa. Dtsch Med Wochenschr 127:335–340 Veterinary Laboratories Agency (2010) Cattle TSE surveillance statistics – General statistics. http://www.defra.gov.uk/science/docs/sci_tse_stats_gen.pdf Anderson RM, Donnelly CA, Ferguson NM et al (1996) Transmission dynamics and epidemiology of BSE in British cattle. Nature 382:779–788 MacDiarmid SC, Infanger P, Hörnlimann B (2006) BSE control – internationally recommended approaches. In: Hörnlimann B, Riesner D, Kretzschmar H (eds) Prions in animals and humans. De Gryuter, Berlin New York, pp 620–662920 Peden AH, Head MW, Ritchie DL et al (2004) Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet 364:527–529 Llewelyn CA, Hewitt PE, Knight RS et al (2004) Possible transmission of variant Creutzfeldt-Jakob disease by blood transfusion. Lancet 363:417–421 Wroe SJ, Pal S, Siddique D et al (2006) Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt-Jakob disease associated blood transfusion: a case report. Lancet 368:2061–2067 Health Protection Agency (2007) Health protection report (19 January 2007): Fourth case of transfusion-associated variant-CJD infection. http://www.hpa.org.uk/hpr/archives/2007/news2007/news0307.htm Health Protection Agency (2009) Health protection report (20 February 2009): Post mortem finding of asymptomatic variant Creutzfeldt-Jakob disease abnormal prion protein in a person with haemophilia. http://www.hpa.org.uk/hpr/archives/2009/news0709.htm#vcjd Peden A, McCardle L, Head MW et al (2010) Variant CJD infection in the spleen of a neurologically asymptomatic UK adult patient with haemophilia. Haemophilia 16:296–304 Robert Koch-Institut (2007) Falldefinitionen des Robert Koch-Instituts zur Übermittlung von Erkrankungs- oder Todesfällen und Nachweisen von Krankheitserregern – Ausgabe 2007. http://www.rki.de/cln_178/nn_200532/DE/Content/Infekt/IfSG/Falldefinition/IfSG/Falldefinition,templateId=raw,property=publicationFile.pdf/Falldefinition.pdf The National Creutzfeldt-Jakob Disease Surveillance Unit (2010) Seventeenth annual report 2008. Creutzfeldt-Jakob disease surveillance in the UK. http://www.cjd.ed.ac.uk/report17.pdf Health Protection Agency (2010) Incidence of variant Creutzfeldt-Jakob disease deaths in the UK: January 1994 – December 2009. http://www.cjd.ed.ac.uk/cjdq64.pdf Joint UKBTS/NIBSC Professional Advisory Committee (2009) Postion statement Creutzfeldt-Jakob disease (12 March 2009). http://www.transfusionguidelines.org.uk/docs/pdfs/dl_ps_vcjd_2009–03.pdf The National Creutzfeldt-Jakob Disease Surveillance Unit (2010) Figures for the number of vCJD cases and referrals of suspected cases of CJD to the NCJDSU since 1990. http://www.cjd.ed.ac.uk/figures.htm Collee JG, Bradley R, Liberski PP (2006) Variant CJD (vCJD) and Bovine Spongiform Encephalopathy (BSE): 10 and 20 years on: part 2. Folia Neuropathol 44:102–110 Kaski D, Mead S, Hyare H et al (2009) Variant CJD in an individual heterozygous for PRNP codon 129. Lancet 374:2128 Hilton DA, Ghani AC, Conyers L et al (2004) Prevalence of lymphoreticular prion protein accumulation in UK tissue samples. J Pathol 203:733–739 Ironside JW, Bishop MT, Connolly K et al (2006) Variant Creutzfeldt-Jakob disease: prion protein genotype analysis of positive appendix tissue samples from a retrospective prevalence study. BMJ 332:1186–1188 World Health Organization (2006) WHO guidelines on tissue infectivity distribution in transmissible spongiform encephalopathies. http://www.who.int/bloodproducts/cs/TSEPUBLISHEDREPORT.pdf Notari S, Moleres FJ, Hunter SB et al (2010) Multiorgan detection and characterization of protease-resistant prion protein in a case of variant CJD examined in the United States. PLoS One 5:e8765 Valleron AJ, Boelle PY, Will R, Cesbron JY (2001) Estimation of epidemic size and incubation time based on age characteristics of vCJD in the United Kingdom. Science 294:1726–1728 Boelle PY, Cesbron JY, Valleron AJ (2004) Epidemiological evidence of higher susceptibility to vCJD in the young. BMC Infect Dis 4:26 Hilton DA, Fathers E, Edwards P et al (1998) Prion immunoreactivity in appendix before clinical onset of variant Creutzfeldt-Jakob disease. Lancet 352:703–704 Hilton DA, Ghani AC, Conyers L et al (2002) Accumulation of prion protein in tonsil and appendix: review of tissue samples. BMJ 325:633–634 SEAC (Spongiform Encephalopathies Advisory Committee) (2008) Position statement „Prevalence of subclinical variant Creutzfeldt-Jakob disease infections“. http://www.seac.gov.uk/statements/state-cjd-infections.pdf Clewley JP, Kelly CM, Andrews N et al (2009) Prevalence of disease related prion protein in anonymous tonsil specimens in Britain: cross sectional opportunistic survey. BMJ 338:b1442 Department of Health (2009) Mapping out the consequences of screening blood donations for PrPSc. http://www.dh.gov.uk/prod_consum_dh/groups/dh_digitalassets/documents/digitalasset/dh_094803.pdf Clarke P, Will RG, Ghani AC (2007) Is there the potential for an epidemic of variant Creutzfeldt-Jakob disease via blood transfusion in the UK? J R Soc Interface 4:675–684 Dietz K, Raddatz G, Wallis J et al (2007) Blood transfusion and spread of variant Creutzfeldt-Jakob disease. Emerg Infect Dis 13:89–96 Garske T, Hester JT, Clarke P et al (2006) Factors determining the potential for onward transmission of variant Creutzfeldt-Jakob disease via surgical instruments. J R Soc Interface 3:757–766 Hörnlimann B, Pauli G, Lemmer K et al (2006) Prevention of transmission of prion diseases in healthcare settings. In: Hörnlimann B, Riesner D, Kretzschmar H (Hrsg) Prions in humans and animals. De Gruyter, Berlin New York, pp 546–560 Arbeitskreis Krankenhaus- und Praxishygiene der AWMF (2007) Prophylaxe der Creutzfeldt-Jakob-Erkrankung in Krankenhaus und Praxis. Hyg Med 7/8:301–306 Beekes M, Mielke M, Pauli G et al (2004) Aspects of risk assessment and risk management of nosocomial transmission of classical and variant Creutzfeldt-Jakob disease with special attention to German regulations. Contrib Microbiol 11:117–135 Bertram J, Mielke M, Beekes M et al (2004) Inaktivierung und Entfernung von Prionen bei der Aufbereitung von Medizinprodukten. Bundesgesundheitsbl Gesundheitsforsch Gesundheitsschutz 47:36–40 Department of Health (2010) Guidance from the ACDP TSE working group. http://www.dh.gov.uk/ab/ACDP/TSEguidance/DH_098253 National Institute for Health and Clinical Excellence (NICE) (2006) Patient safety and reduction of risk of transmission of Creutzfeldt-Jakob disease (CJD) via interventional procedures. http://www.nice.org.uk/IPG196 Department of Health (2006) The decontamination of surgical instruments with special attention to the removal of proteins: and inactivation of any contaminating human prions – 2006 report. http://www.dh.gov.uk/en/Publicationsandstatistics/Publications/PublicationsPolicyAndGuidance/DH_072443 Task Force vCJK (2002) Die Variante der Creutzfeldt-Jakob-Krankheit (vCJK). Epidemiologie, Erkennung, Diagnostik und Prävention unter besonderer Berücksichtigung der Risikominimierung einer iatrogenen Übertragung durch Medizinprodukte, insbesondere chirurgische Instrumente – Abschlussbericht der Task Force vCJK zu diesem Thema. Bundesgesundheitsbl Gesundheitsforsch Gesundheitsschutz 45:376–394 (o A) (1996) Desinfektion und Sterilisation von chirurgischen Instrumenten bei Verdacht auf Creutzfeldt-Jakob-Erkrankungen. Bundesgesundheitsblatt 39:282–283 Simon D, Pauli G (1998) Krankenversorgung und Instrumentensterilisation bei CJK-Patienten und CJK-Verdachtsfällen. Bundesgesundheitsblatt 41:279–285 Kommission für Krankenhaushygiene und Infektionsprävention und Bundesinstitut für Arzneimittel und Medizinprodukte (2001) Gemeinsame Empfehlung der Kommission für Krankenhaushygiene und Infektionsprävention und des Bundesinstitutes für Arzneimittel und Medizinprodukte – Anforderungen an die Hygiene bei der Aufbereitung von Medizinprodukten. Bundesgesundheitsbl Gesundheitsforsch Gesundheitsschutz 44:1115–1126 Lehmann S, Rauwel GPM, Rogez-Kreuz C et al (2009) New hospital disinfection processes for both conventional and prion infectious agents compatible with thermosensitive equipment. J Hosp Infect 72:342–350 Beekes M, Lemmer K, Thomzig A et al (2010) Fast, broad-range disinfection of bacteria, fungi, viruses and prions. J Gen Virol 91:580–589 Department of Health (2000) Guidance on the microbiological safety of human organs, tissues and cells used in transplantation. http://www.dh.gov.uk/en/Publicationsandstatistics/Publications/PublicationsPolicyAndGuidance/DH_4005526 Human Tissue Authority (2006) HTA Directions 001/2006. http://www.hta.gov.uk/legislationpoliciesandcodesofpractice/htalegaldirections.cfm Advisory Committee on the Microbiological Safety of Blood and Tissues for Transplantation (MBST) (2005) Summary of meeting of advisory committee on the microbiological safety of blood and tissues for transplantation (MSBT). http://www.advisorybodies.doh.gov.uk/acmsbtt/msbtsum200105.pdf Department of Health (2005) Exclusion of femoral heads from living donors – vCJD precautions. http://www.dh.gov.uk/en/Publicationsandstatistics/Lettersandcirculars/Professionalletters/Chiefmedicalofficerletters/DH_4114116 Arbeitskreis Blut des Bundesministeriums für Gesundheit und Soziale Sicherung (2005) Variante Creutzfeldt-Jakob-Krankheit – Stellungnahme des Arbeitskreises Blut des Bundesministeriums für Gesundheit und soziale Sicherung. Bundesgesundheitsbl Gesundheitsforsch Gesundheitsschutz 48:1082–1090 Arbeitsgruppe „Gesamtstrategie Blutversorgung angesichts vCJK“ (2006) Bericht der Arbeitsgruppe „Gesamtstrategie Blutversorgung angesichts vCJK“. http://www.rki.de/cln_151/nn_206134/DE/Content/Infekt/Blut/AK__Blut/Links/Bericht__PEI,templateId=raw,property=publicationFile.pdf/Bericht_PEI.pdf Arbeitskreis Blut des Bundesministeriums für Gesundheit (2006) Vorgehensweise bei variante Creutzfeldt-Jakob-Krankheit (vCJK) im Zusammenhang mit Blut, Plasma und Blutprodukten. Bundesgesundheitsbl Gesundheitsforsch Gesundheitsschutz 49:396–398 Turner ML, Ludlam CA (2009) An update on the assessment and management of the risk of transmission of variant Creutzfeldt-Jakob disease by blood and plasma products. Br J Haematol 144:14–23 Arbeitskreis Blut des Bundesministeriums für Gesundheit (2009) Stellungnahme zum Risiko der Übertragung von vCJK durch Plasmaderivate aus humanem Plasma. Bundesgesundheitsbl Gesundheitsforsch Gesundheitsschutz 52:648–649 Arbeitsgruppe „Gesamtstrategie Blutversorgung angesichts vCJK“ (2008) Stellungnahme zur Entwicklung und Einführung von Testsystemen zum Screening von Blutspendern auf vCJK. http://www.rki.de/cln_151/nn_206142/DE/Content/Infekt/Blut/AK__Blut/Links/StellungnahmeTestsvCJK,templateId=raw,property=publicationFile.pdf/StellungnahmeTestsvCJK.pdf Watts JC, Balachandran A, Westaway D (2006) The expanding universe of prion diseases. PLoS Pathog 2:e26