사람에서 발생하는 프리온 질환들 (Human Prion Diseases)

서지정보

· 발행기관 : 대한의사협회
· 수록지 정보 : 대한의사협회지 / 51권 / 12호 / 1125 ~ 1138페이지
· 저자명 : 김상윤, 정해관, 안성수

초록

Transmissible Spongiform Encephalopathy (TSE) or prion diseases are fatal neurodegenerative
diseases, which are caused by transmissible abnormal prion proteins,
converting the endogenous normal prion in the body to the infectious abnormal prions. The most
common form of human prion diseases is Creutzfeldt - Jakob disease (CJD). Most of CJD are
sporadic with unknown cause. Some familial or iatrogenic CJDs are reported in many countries,
but there have been no formally reported case in Korea. Variant CJD (vCJD) is a new form of
human prion disease, which revealed differentiated clinical presentations and laboratory
diagnostic results. vCJD was thought to be originated from eating the beefs or other parts of
bovine spongiform encephalopathy (BSE) infected cattle. The unpredictable species barriers, the
underestimated distribution of prion infected tissues, the variable clinical courses, and uncertain
disease progressions of many prion diseases, all made the prion related risk assessment very
difficult. Korea needs our own surveillance system for various prion diseases of human and
animals and to make plans for the risk assessment of the various prion disease transmissions for
the minimal spread by maximizing the research capacities.

영어초록

Transmissible Spongiform Encephalopathy (TSE) or prion diseases are fatal neurodegenerative
diseases, which are caused by transmissible abnormal prion proteins,
converting the endogenous normal prion in the body to the infectious abnormal prions. The most
common form of human prion diseases is Creutzfeldt - Jakob disease (CJD). Most of CJD are
sporadic with unknown cause. Some familial or iatrogenic CJDs are reported in many countries,
but there have been no formally reported case in Korea. Variant CJD (vCJD) is a new form of
human prion disease, which revealed differentiated clinical presentations and laboratory
diagnostic results. vCJD was thought to be originated from eating the beefs or other parts of
bovine spongiform encephalopathy (BSE) infected cattle. The unpredictable species barriers, the
underestimated distribution of prion infected tissues, the variable clinical courses, and uncertain
disease progressions of many prion diseases, all made the prion related risk assessment very
difficult. Korea needs our own surveillance system for various prion diseases of human and
animals and to make plans for the risk assessment of the various prion disease transmissions for
the minimal spread by maximizing the research capacities.

참고자료

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