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9 "proteinaceous infectious particles" ( ) (PrP Sc ) (PrP C ) PrP C PrP Sc PrP Sc 9
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17 (1) (2) (3) 17
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25 (1) Creutzfeldt-Jakob - -. Clinical neuroscience2001,19 : (2). 11,
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29 ( ) CJD CJD CJD (1) (2) (3) (4) (5) (6) (7) 3SDS (8) (9) (10) (11) 60 2 (12) 1N 29
30 (13) 3SDS (14) (15) (16) (17) BDA : Advice Sheet A12, Infection Control in Dentistry Report of the working group on decontamination of instruments in dental services in Scotland WHO : WHO infection control guidelines for transmissible spongiform encephalopathies. Report of a WHO consultation. Geneva, Switzerland, March CJD Incidents Panel : Management of possible exposure to CJD through medical procedures. A consulting paper. October CJD Incidents Panel : PUBLIC SUMMARY of the meeting. 17 th April
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46 CJD (1) CJD CJD CJD CJD CJD DNA (2) 2 46
47 CJD CJD CJD CJD CDC (3) CJD CJD CJD CJD CJD CJD CJD 47
48 (4) CJD (1) (2) WHO/HGN/ETH/ (3) (4)
49 (CJD Incidents Panel: Management of possible exposure to CJD through medical procedures. October Department of Health Publications, London ) ACDP Advisory Committee on Dangerous Pathogens 1981 BSE Bovine Spongiform Encephalopathy CDSC Communicable Disease Surveillance Centre CJD Creutzfeldt-Jakob Disease = CWD Chronic Wasting Disease. Cleaning CNS Central Nervous System Contactable Patient CJD CSF Cerebrospinal fluid Decontamination Definite case of CJD ( ) CJD Dose response relationship Dura mater Endoscope 49
50 Familial CJD FFI Florid plaque GSS HGH Kuru Iatrogenic CJD IVH Lymphoreticular system (LRS) CJD (PrP) Fatal Familial Insomnia CJD Gerstmann-Sträussler-Scheinker syndromegss CJD HGH CJD 1900 CJD intravenous hyperalimentation CJD CJD Median infective dose (ID 50 ) 50% Medical device NCJDSU The National CJD Surveillance Unit CJD 1990 CJD Possible case of CJD ( ) CJD Prion Proteinaceous infectious agent CJD TSE Prion protein (PrP) 50
51 (1) PrP Sc, (2) PrP C 2 (1) TSE (PrP Sc )(2) PrP (PrP C ) Probable case of CJD ( ) CJD Prophylactic PrP C PrP Sc PSD Scrapie SEAC Single use device PrP PrP C PrP Periodic synchronized discharge Creutzfeldt-Jakob CJD TSE TSE Spongiform Encephalopathy Advisory Committee Sporadic CJD CJD CJD CJD TME Transmissible mink encephalopathy TSE Transmissible Spongiform Encephalopathy (TSE) CJD BSE Variant CJD CJD 1996 BSE CJD new variant CJD 51
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15 CQ 15 1 15 CQ 15 1 Creutzfeldt Jakob (CJD) CJD, 7.,,,,. 解説 国 病 発症率 人口 100 万人 年間 1 人程度 1,2), 平均年齢 67.9 歳 2). 病 五類感染症 指定, 診断 医師 診断後 7 日以内 保健所 報告 必要. 病 孤発性, 遺伝性, 獲得性 3 種類, 最 多, 古典型孤発性 CJD ( 1). 典型例 古典型
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SRM TMETransmissible Mink Encephalopathy CWDChronic Wasting Disease BSE 1 WB IHC BSE SRM SSOPHACCP BSE AMR 2 BSE BSE 3 4 15 48 24 3 Bovine Spongiform Encephalopathy BSE 17 5 24 1)2) 3) 4) BSE 5) BSE 2003
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23 プリオン病 概要 1. 概要プリオン病は 正常プリオン蛋白が何らかの理由で伝播性を有する異常プリオン蛋白に変化し 主に中枢神経内に蓄積することにより急速に神経細胞変性をおこす稀な致死性疾患である プリオン病の代表的なタイプである孤発性クロイツフェルト ヤコブ病 (CJD) は 1 年間に 100 万人に 1 人程度の割合で発症することが知られている ヒトのプリオン病は病因により 原因不明の特発性
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