Retrospective Neuropathological Review of Prion Disease in UK Haemophilic Patients
Retrospective Neuropathological Review of Prion Disease in UK Haemophilic Patients
复制标题
英国血友病患者朊病毒病的回顾性神经病理学研究
DOI：
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发表时间：
1998
影响因子：
6.7
通讯作者：
J. McLaughlin
中科院分区：
文献类型：
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作者：
Christine A. Lee;J. Ironside;J. Bell;P. Giangrande;C. Ludlam;M. Esiri;J. McLaughlin
1996年，英国爱丁堡的CJD监测单位描述了一种被认为相当于人类牛海绵状脑病(BSE)的nvCJD。在受感染个体的扁桃体中鉴定出普恩蛋白，提出了nvCJD通过血液产品传播的问题。这项研究检查了1962-1995年间接受凝血因子浓缩物治疗的33名患者的身体大脑。凝血因子浓缩物主要来自英国捐赠者。用传统的组织学方法和用单抗KG9和3F4检测了脑组织中的普里恩蛋白。未发现海绵状脑病的证据，所有病例免疫细胞化学均为PrP阴性。结论是，目前没有证据表明nvCJD通过凝血因子浓缩物传播给血友病患者。
Summary In 1996, the CJD surveillance unit in Edinburgh, UK described nvCJD which was thought to be the human equivalent of bovine spongiform encephalopathy (BSE). The identification of prion protein in the tonsil of an affected individual has raised the question of transmission of nvCJD via blood products. This study examines the post mortem brains of 33 patients who were treated with clotting factor concentrate of predominately UK donor source during the years 1962-1995. The brains were examined by conventional histological methods and also for the prion protein using monoclonal antibodies KG9 and 3F4. No evidence of spongiform encephalopathy was found and the immunocytochemistry was negative for PrP in all cases. It is concluded that, at present, there is no evidence for the transmission of nvCJD via clotting factor concentrate to patients with haemophilia.