Risk of transmission of Creutzfeldt-Jakob disease by transfusion of blood,plasma, and plasma derivatives

Studies in experimental animals and case-reports of transmission of Creutzfeldt-Jakob Disease (CJD) by blood transfusion or by albumin products have raised the possibility that CJD may be transmitted by transfusion. The risk of transmission of CJD by transfusion remains theoretical, since no confirmed case of CJD has ever been causally attributed to the receipt of a blood transfusion, no confirmed case of CJD has developed in recipients of clotting factor concentrates, and no cluster of CJD cases has been reported following the administration of a pooled plasma derivative to which a donor who subsequently developed CJD had contributed. However, based on a review of the hitherto available data, it is impossible to conclude at this time that CJD is not transmitted by blood or plasma transfusion or by the administration of pooled plasma derivatives. This review discusses the findings of the animal experiments and the human studies that investigated the potential for transmission of CJD among humans by transfusion, and explains the statistical difficulties associated with proving the negative hypothesis that CJD is not transmitted by transfusion. J. Clin. Apheresis 14:135–143, 1999. © 1999 Wiley-Liss, Inc.     

头颅磁共振成像在克雅氏病监测中的特点分析

目的 了解头颅磁共振成像(magnetic resonance imaging,MRI)扫描在克雅氏病(Creutzfeldt-Jakob disease, CJD)监测及诊断中的重要作用。方法 对2013年以来我国CJD监测网络中的MRI数据进行分析。结果 通过数据分析发现,95.93%(377/393)的散发型克雅氏病(sporadic Creutzfeldt-Jakob disease,sCJD)临床诊断病例进行过头颅MRI扫描。弥散加权像(diffusion-weight imaging,DWI)显示对称或不对称性缎带征是sCJD临床诊断中最常见的MRI特征性改变,而质子密度相双侧丘脑后结节部高信号的病例数相对较少。结论 头颅MRI扫描检测对sCJD的临床诊断具有重要的作用。MRI扫描结果纳入到CJD监测网络后,将进一步完善我国CJD监测体系,增加对sCJD临床诊断的准确性。     

2018年中国克–雅病监测网络病例特征分析

  目的  了解2018年中国克–雅病监测网络中12个省份的发病情况、流行病学和临床特征。  方法  对我国克–雅病监测网络获得的可疑克–雅病病例的临床及流行病学资料进行分析,收集患者脑脊液及血液样品,采用Western blot检测脑脊液中14-3-3蛋白,提取全血基因组DNA并利用聚合酶链式反应及测序方法对PRNP基因进行129位和219位氨基酸多态性及基因突变的分析。  结果  2018年共监测克–雅病病例537例,其中散发型确诊诊断病例2例（0.37%）,临床诊断病例212例（39.48%）,疑似诊断病例13例（2.42%）,遗传型病例16例（2.98%）,致死性家族型失眠症5 例（0.93%）,格斯特曼综合征2例（0.37%）。 病例报告无季节聚集性,长久居住地呈散在分布,职业分布广泛。 确诊诊断和临床诊断病例年龄中位数为64（34～87）岁,男女性别比为1.19∶1;疑似诊断病例年龄中位数为63（39～76）岁,男女性别比为0.86∶1。 快速进行性痴呆为最常见的首发症状。 517份血液样品PRNP基因的检测结果显示,其中507例129位氨基酸为M/M纯合子,510例219位氨基酸为E/E纯合子。  结论  2018年我国监测到的克–雅病病例的报告时间、长久居住地、职业、性别比例以及年龄分布均符合散发型克–雅病的发病特点。     

2020年中国克–雅病监测网络病例特征分析

  目的   了解2020年中国克–雅病（CJD）的发病情况、流行病学及临床特征。  方法  对2020年我国CJD监测网络获得的可疑CJD病例的临床及流行病学资料进行分析,并收集患者的血液及脑脊液,提取全血基因组DNA并利用聚合酶链式反应及测序方法对朊蛋白基因（PRNP）129位及219位氨基酸多态性进行分析,Western Blot检测脑脊液中14-3-3蛋白。   结果   2020年共监测病例458例,其中散发型CJD临床诊断病例179例（39.08%）,疑似诊断病例12例（2.62%）,遗传型CJD病例18例（3.93%）,吉斯特曼–施特劳斯综合征（GSS）1例（0.22%）。 病例报告无季节聚集性,长久居住地呈散在分布,职业分布无聚集性。临床诊断病例年龄中位数为63.14（43,87）岁,男女性别比为 0.97∶1;疑似诊断病例年龄中位数为68.08（54,77）岁,男女性别比为1∶1。 快速进行性痴呆为最常见的首发症状。 脑脊液14-3-3蛋白、脑电图以及头颅磁共振成像3项检测结果中,出现阳性结果越多的病例典型症状也较多。 对439份血液样品进行PRNP检测,其中129位氨基酸为M/M 纯合子的434例,M/V杂合子的3例,V/V纯合子的2例;219位氨基酸为E/E纯合子的435 例,E/K纯合子的4例。  结论   2020年我国监测到的CJD病例的报告时间、长久居住地分布、职业、性别比例以及年龄分布均符合CJD的发病特点。     

Lack of evidence for the heterosexual transmission of hepatitis C

The importance of sexual transmission in the epidemiology of hepatitis C virus (HCV) infection is still controversial. To assess the risk of heterosexual HCV transmission, we examined eighty patients with chronic HCV-associated liver disease and their spouses in a cross-sectional clinical and serological cohort study. Serum samples from index patients and their spouses were assayed for HCV antibodies and HCV RNA. In the couples positive for both, further HCV genotyping was done. A questionnaire addressing points such as additional risk factors for HCV infection, sexual behaviour or duration of partnership was completed by all couples. HCV antibodies were detected in four (5%) spouses, of whom three (4%) were also positive for HCV-RNA. HCV genotyping revealed concordance (genotype 1) in two couples, indicating a risk of interspousal HCV transmission of 2.5%. Spouses of patients with HCV viraemia and chronic liver disease have a low risk for acquiring HCV. Even long-term spouses seem not to be at increased risk. We therefore suggest that the risk of HCV transmission between monogamous sex partners does not depend on the duration of sexual exposure.     

Transmission of sporadic Creutzfeldt-Jakob disease by blood transfusion: risk factor or possible biases

BACKGROUND: The occurrence of transfusion transmissions of variant Creutzfeldt‐Jakob disease (CJD) cases has reawakened attention to the possible similar risk posed by other forms of CJD. STUDY DESIGN AND METHODS: CJD with a definite or probable diagnosis (sporadic CJD, n = 741; genetic CJD, n = 175) and no‐CJD patients with definite alternative diagnosis (n = 482) with available blood transfusion history were included in the study. The risk of exposure to blood transfusion occurring more than 10 years before disease onset and for some possible confounding factors was evaluated by calculating crude odds ratios (ORs). Variables with significant ORs in univariate analyses were included in multivariate logistic regression analyses. RESULTS: In the univariate model, blood transfusion occurring more than 10 years before clinical onset is 4.1‐fold more frequent in sporadic CJD than in other neurologic disorders. This significance is lost when the 10‐year lag time was not considered. Multivariate analyses show that the risk of developing sporadic CJD after transfusion increases (OR, 5.05) after adjusting for possible confounding factors. Analysis conducted on patients with genetic CJD did not reveal any significant risk factor associated with transfusion. CONCLUSION: This is the first case‐control study showing a significant risk of transfusion occurring more than 10 years before clinical onset in sporadic CJD patients. It remains questionable whether the significance of these data is biologically plausible or the consequence of biases in the design of the study, but they counterbalance previous epidemiologic negative reports that might have overestimated the assessment of blood safety in sporadic CJD.     

Clinical evidence for rapid transmission of Lyme disease following a tickbite

Lyme disease transmission to humans by Ixodes ticks is thought to require at least 36-48 h of tick attachment. We describe 3 cases in which transmission of Borrelia burgdorferi, the spirochetal agent of Lyme disease, appears to have occurred in less than 24 h based on the degree of tick engorgement, clinical signs of acute infection, and immunologic evidence of acute Lyme disease. Health care providers and individuals exposed to ticks should be aware that transmission of Lyme disease may occur more rapidly than animal models suggest. A diagnosis of Lyme disease should not be ruled out based on a short tick attachment time in a subject with clinical evidence of B. burgdorferi infection.     

Creutzfeldt-Jakob disease guidelines: a regional approach

Creutzfeldt-Jakob Disease (CJD) is an infectious, progressive, degenerative neurological disorder. Unique CJD Precautions must be adhered to as the infectious agent is difficult to destroy. A regional group in Hamilton-Burlington Ontario developed CJD guidelines based on critical review of the current evidence of transmission in a Canadian healthcare environment, current published standards of practice in North America, the United Kingdom and Australia; and principles of laboratory and patient care safety as well as expert opinion.     

Estimated risk of transmission of the West Nile virus through blood transfusion in the US, 2002

BACKGROUND: The West Nile virus (WNV) epidemic in 2002 in the US saw over 3300 reported human cases of WNV disease, with over 2300 reported cases of WNV encephalitis and meningitis. The first documented cases of transfusion transmission of WNV through voluntary blood donation also occurred. STUDY DESIGN AND METHODS: Case onset dates from the 2002 WNV epidemic in the US were used to estimate the risk of transfusion-associated transmission with statistical resampling. An easily computed approximating formula for the mean risk was derived. Estimates were computed for six high-incidence states and metropolitan areas. RESULTS: Mean and maximum risk of transfusion-associated WNV transmission (per 10,000 donations) during the epidemic period for the selected states ranged from 2.12 to 4.76 and from 4.34 to 10.46, respectively; for the selected metropolitan areas, they ranged from 1.46 to 12.33 and from 3.02 to 21.32, respectively. CONCLUSIONS: Estimates of the mean risk of WNV transmission by transfusion ranged from 1.46 to 12.33 per 10,000 donations for six high-incidence metropolitan areas during the 2002 epidemic. Because the risk was highly geographically and temporally variable, computation of geographically localized estimates is recommended. The derived approximating formula for the mean risk performed well for the estimates given.     

克-亚二氏综合征的MR表现

目的描述克-亚二氏综合征的脑部MRI表现,探讨MRI对该病的诊断价值。方法经病理证实的克-亚二氏综合征4例,回顾性分析其MRI和扩散加权像(DWI)表现,并复习相关临床及文献资料。结果1例表现为双侧脑室旁深部白质区及内囊后肢皮质脊髓束走行区稍长T1、稍长T2信号;2例T2WI及DWI上表现为双侧尾状核和壳核区对称性稍高信号影和部分皮质带状异常高信号;1例T2WI及DWI上表现为部分皮质带状异常高信号,伴脑萎缩。结论克-亚二氏综合征的脑部MRI表现有比较典型的特点,MRI是诊断该病的重要手段。