Update: Creutzfeldt-Jakob disease associated with cadaveric dura mater grafts--Japan, 1979-2003

In 1997, a nongovernment surveillance group for Creutzfeldt-Jakob disease (CJD) in Japan supported financially by the Ministry of Health and Welfare (MHW) reported 43 cases of CJD associated with receipt of cadaveric dura mater grafts. In all but one case, the most probable vehicle of transmission was a single brand of dural graft (LYODURA [B. Braun Melsungen AG, Melsungen, Germany]) produced before May 1987. As of March 2003, ongoing surveillance in Japan had identified an additional 54 dura mater graft--associated cases. This report summarizes the investigation of the 97 cases, which indicated that during 1983-1987, the estimated minimum risk for CJD within 17 years of receipt of the implicated product in Japan was approximately one case per 1,250 grafts. No cases have been reported among patients who received their first dural graft after 1991; however, because of the long latency period between graft placement and symptom onset, additional cases of graft-associated CJD are likely to be reported.     

Epidemiologic features of 65 Creutzfeldt-Jakob disease patients with a history of cadaveric dura mater transplantation in Japan

A total of 65 cases of Creutzfeldt-Jakob disease with a history of cadaveric dura transplantation in Japan were analysed to clarify the epidemiologic features of such patients and to explore whether other such patients will appear in the future. The age at transplantation averaged 44.4 years with a standard deviation of 14.4 years. The age at onset had an average of 53.0 years with a standard deviation of 14.1 years. The shortest latent period was 14 months, and the longest was 218 months with an average of 103.1 months and a standard deviation of 49.9 months. From the relationship between the calendar year at transplantation and the latent period, other such patients will appear in the near future. The current data suggested that several patients with Creutzfeldt-Jakob disease will occur from those receiving cadaveric dura mater grafts in the near future.     

A case-control study of Creutzfeldt-Jakob disease in Japan: transplantation of cadaveric dura mater was a risk factor

A case-control study was conducted to reveal the relative risk of cadaveric dura mater graft transplantation for Creutzfeldt-Jakob disease. Fifty-two cases with Creutzfeldt-Jakob disease that were reported to the surveillance of the disease, and 102 age- and sex-matched hospital controls were selected. Information on family history, occupations, and medical history was collected. Eight cases and no control had a history of cadaveric dura mater graft transplantation. Surgical operations without the dura mater graft, blood transfusion, and acupuncture did not elevate the risk.     

A case of Creutzfeldt-Jakob disease associated with a dura mater graft in the United States.

We describe a case of Creutzfeldt-Jakob disease associated with a dura mater graft (Lyodura brand) in a 26-year-old man who underwent several neurosurgical procedures as a child. Clinicians and infection control personnel should be aware that recipients of Lyodura brand dura mater grafts processed before May 1987 may remain at increased risk for Creutzfeldt-Jakob disease throughout their lives.     

Probable Creutzfeldt-Jakob disease after a cadaveric dural graft

A 26-year old female who had received a cervical cadaveric dural graft 7 years earlier developed psychotic behavior and generalized myoclonic jerks. Following rapid deterioration and appearance of high-charge sharp periodic waves on electroencephalography she died within two months. We believe that this patient is the first German who acquired Creutzfeldt-Jakob disease by dural implant.     

Surveillance of Creutzfeldt-Jakob disease in Australia: 2008

Australia-wide surveillance of all human transmissible spongiform encephalopathies (TSEs) is performed by the Australian National Creutzfeldt-Jakob Disease Registry (ANCJDR), since establishment in October 1993. During the surveillance period 1 April 2007 to 31 March 2008, the ANCJDR received 78 new suspect case notifications of TSEs (67 in 2007, 13 in 2008). This level of suspect case notification aligns with the previous 2006/2007 surveillance period, which was elevated in comparison to the previous 5 years. Based on the total number of probable and definite Creutzfeldt-Jakob disease (CJD) cases, encompassing retrospective cases to 1970 and prospectively ascertained cases from 1993 to 31 March 2008, the average age-adjusted mortality rate is 1.16 deaths per million per year. In this short report, we provide updated Australian TSE figures and describe recent changes in surveillance mechanisms and review their impact on case notifications and eventual CJD classification.     

上海市2006-2012年克雅氏病病例监测

目的 了解2006——2012年上海市克雅氏病(CJD)监测病例的检测结果及流行病学特征。方法 分析上海市CJD监测网络中可疑CJD病例的临床和流行病学资料,采集病例脑脊液及血液标本检测14-3-3蛋白和129位氨基酸多态性及PRNP基因突变。应用EpiData 3．0软件建立数据库,使用SPSS 17．0软件分析。结果 2006-2012年上海市发现散发型CJD确诊病例1例,临床诊断病例56例,疑似病例17例;家族遗传型CJD确诊病例l例,临床诊断1例,均为E200K突变。病例分布无季节聚集性,历年报告病例数保持稳定。CJD病例居住地散在分布,但2例家 族遗传型CJD患者住址相近。确诊和临床诊断病例的平均年龄为62岁,大于疑似病例的年龄(56岁),差异有统计学意义。结论2006-2012年上海市CJD历年发病例数稳定,均以散发型为主,职业分布广泛,确诊和临床诊断病例的平均年龄大于疑似病例。     

Update: measles--United States, January-July 2008

Sporadic importations of measles into the United States have occurred since the disease was declared eliminated from the United States in 2000. During January-July 2008, 131 measles cases were reported to CDC, compared with an average of 63 cases per year during 2000-2007. This report updates an earlier report on measles in the United States during 2008 and summarizes two recent U.S outbreaks among unvaccinated school-aged children. Among those measles cases reported during the first 7 months of 2008, 76% were in persons aged <20 years, and 91% were in persons who were unvaccinated or of unknown vaccination status. Of the 131 cases, 89% were imported from or associated with importations from other countries, particularly countries in Europe, where several outbreaks are ongoing. The findings demonstrate that measles outbreaks can occur in communities with a high number of unvaccinated persons and that maintaining high overall measles, mumps, and rubella (MMR) vaccination coverage rates in the United States is needed to continue to limit the spread of measles.     

Late-in-life surgery associated with Creutzfeldt-Jakob disease: a methodological outline for evidence-based guidance

Background

There is increasing epidemiological evidence of etiological links between general surgery and sporadic Creutzfeldt-Jakob disease (sCJD) with long incubation periods. The purpose of this study was to identify specific surgical procedures potentially associated with sCJD to be targeted for preventive presurgical-intervention guidance.

Results

We propose a three-step clinical guidance outline where surgical procedures associated with sCJD clinical onset – potentially more contaminant - are taken into account. Data on hospital discharges and surgical procedures were obtained from Danish and Swedish national in-patient hospital registries for 167 sCJD cases, onset 1987–2003, and for 835 matched and 2,224 unmatched population controls. Surgery was allocated to different life-time periods as previously reported, and frequencies were compared using logistic regression analysis. In the year preceding clinical onset, persons with sCJD underwent a statistically significant higher number of minor surgical interventions (OR (95% CI): 17.50 (3.64-84.24)), transluminal endoscopies (OR: 2.73 (1.01–7.37)) and gastrointestinal operations (OR: 3.51 (1.21–10.19)) compared to matched controls. Surgical discharges clustered towards clinical onset. These differences increased during the clinical period, with statistically significant higher frequencies for both endoscopies and minor surgery (OR: 13.91 (5.87-32.95), and for main surgical procedures (OR: 2.10 (1.00-4.39)), particularly gastrointestinal surgery (OR: 6.00 (1.83-19.66)), and surgery contacting skeletal muscle. Comparisons with unmatched controls yielded similar results for neurosurgery in the clinical period (OR: 19.40 (2.22-168.34)).

Conclusions

These results suggest that some types of surgical procedures are associated with sCJD, after clinical onset or particularly just before onset. Selective planning of such surgery to minimize instrument/device contamination or quarantining might be feasible. Conditional to progress in sCJD etiological research, results are relevant for guidance development.

     

Update on overall prevalence of major birth defects--Atlanta, Georgia, 1978-2005

Major structural or genetic birth defects affect approximately 3% of births in the United States, are a major contributor to infant mortality, and result in billions of dollars in costs for care. Although the causes of most major birth defects are unknown, concerns have been raised that certain factors, such as an increase in the prevalence of diabetes among women, might result in increased prevalence of birth defects over time. This report updates previously published data from the Metropolitan Atlanta Congenital Defects Program (MACDP), the oldest population-based birth defects surveillance system in the United States with active case ascertainment. For the period 1978-2005, CDC assessed the overall prevalence of major birth defects and their frequency relative to selected maternal and infant characteristics. The MACDP results indicated that the prevalence of major birth defects in metropolitan Atlanta, Georgia, remained stable during 1978-2005 but varied by maternal age and race/ethnicity, birthweight, and gestational age. Tracking the overall prevalence of major birth defects can identify subgroups that are affected disproportionately; additional measures focused on these subgroups might improve preconception care and care during pregnancy to prevent birth defects.     

Update: recall of dry dog and cat food products associated with human Salmonella Schwarzengrund infections--United States, 2008

On May 16, 2008, CDC reported on a 2006-2007 multistate outbreak of infection with Salmonella enterica serotype Schwarzengrund that was associated with dry dog food. At the time of that report, a total of 70 cases had been reported from 19 states, with the last case identified on October 1, 2007. Subsequently, an additional case was identified on December 29, 2007. Epidemiologic and environmental investigations have suggested the source of the outbreak was dry pet food produced by one manufacturer, Mars Petcare US. This report updates the previous CDC report, provides additional epidemiologic findings, and describes additional actions taken by public health agencies and the manufacturer. In 2008, eight more cases have been reported, bringing the total number of cases in the outbreak to 79. On September 12, 2008, the company announced a nationwide voluntary recall of all dry dog and cat food products produced during a 5-month period at one Pennsylvania plant. Dry pet food has a 1-year shelf life. Contaminated products identified in recalls might still be in the homes of purchasers and could cause illness. Persons who have these products should not use them to feed their pets but should discard them or return them to the store.     

中国2008年克雅氏病监测病例特征分析

目的 了解中国克雅氏病(CJD)的发病情况及流行病学、临床特征.方法 对2008年中国克雅氏病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,收集患者脑脊液及血液样品,利用Western blot方法榆测脑脊液中是否含有14-3-3蛋白,提取全血基因组DNA并利用PCR及测序方法对脚基因进行129位多态性及基因突变分析.结果 共发现散发型CJD临床诊断病例31例,疑似诊断病例11例,2例家族性CJD和1例格-斯-斯综合征.病例的地理分布及职业无明显聚集性;临床诊断病例发病平均年龄56.7岁,男女比例8:9;疑似诊断病例发病平均年龄为57.4岁,男女比例5:6.快速进行性痴呆为最常见的首发症状,占全部诊断病例的33.3%.临床诊断病例比疑似诊断病例出现更多的典型临床表现.结论 2008年中国监测到的CJD病例以散发型为主,病例的地理分布、职业、性别比例以及平均年龄均符合散发型CJD的分布特点.     

中国2008年克雅氏病监测病例特征分析

目的 了解中国克雅氏病(CJD)的发病情况及流行病学、临床特征.方法 对2008年中国克雅氏病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,收集患者脑脊液及血液样品,利用Western blot方法榆测脑脊液中是否含有14-3-3蛋白,提取全血基因组DNA并利用PCR及测序方法对脚基因进行129位多态性及基因突变分析.结果 共发现散发型CJD临床诊断病例31例,疑似诊断病例11例,2例家族性CJD和1例格-斯-斯综合征.病例的地理分布及职业无明显聚集性;临床诊断病例发病平均年龄56.7岁,男女比例8:9;疑似诊断病例发病平均年龄为57.4岁,男女比例5:6.快速进行性痴呆为最常见的首发症状,占全部诊断病例的33.3%.临床诊断病例比疑似诊断病例出现更多的典型临床表现.结论 2008年中国监测到的CJD病例以散发型为主,病例的地理分布、职业、性别比例以及平均年龄均符合散发型CJD的分布特点.     

中国2008年克雅氏病监测病例特征分析

目的 了解中国克雅氏病(CJD)的发病情况及流行病学、临床特征.方法 对2008年中国克雅氏病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,收集患者脑脊液及血液样品,利用Western blot方法榆测脑脊液中是否含有14-3-3蛋白,提取全血基因组DNA并利用PCR及测序方法对脚基因进行129位多态性及基因突变分析.结果 共发现散发型CJD临床诊断病例31例,疑似诊断病例11例,2例家族性CJD和1例格-斯-斯综合征.病例的地理分布及职业无明显聚集性;临床诊断病例发病平均年龄56.7岁,男女比例8:9;疑似诊断病例发病平均年龄为57.4岁,男女比例5:6.快速进行性痴呆为最常见的首发症状,占全部诊断病例的33.3%.临床诊断病例比疑似诊断病例出现更多的典型临床表现.结论 2008年中国监测到的CJD病例以散发型为主,病例的地理分布、职业、性别比例以及平均年龄均符合散发型CJD的分布特点.     

中国2008年克雅氏病监测病例特征分析

目的 了解中国克雅氏病(CJD)的发病情况及流行病学、临床特征.方法 对2008年中国克雅氏病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,收集患者脑脊液及血液样品,利用Western blot方法榆测脑脊液中是否含有14-3-3蛋白,提取全血基因组DNA并利用PCR及测序方法对脚基因进行129位多态性及基因突变分析.结果 共发现散发型CJD临床诊断病例31例,疑似诊断病例11例,2例家族性CJD和1例格-斯-斯综合征.病例的地理分布及职业无明显聚集性;临床诊断病例发病平均年龄56.7岁,男女比例8:9;疑似诊断病例发病平均年龄为57.4岁,男女比例5:6.快速进行性痴呆为最常见的首发症状,占全部诊断病例的33.3%.临床诊断病例比疑似诊断病例出现更多的典型临床表现.结论 2008年中国监测到的CJD病例以散发型为主,病例的地理分布、职业、性别比例以及平均年龄均符合散发型CJD的分布特点.     

中国2008年克雅氏病监测病例特征分析

目的 了解中国克雅氏病(CJD)的发病情况及流行病学、临床特征.方法 对2008年中国克雅氏病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,收集患者脑脊液及血液样品,利用Western blot方法榆测脑脊液中是否含有14-3-3蛋白,提取全血基因组DNA并利用PCR及测序方法对脚基因进行129位多态性及基因突变分析.结果 共发现散发型CJD临床诊断病例31例,疑似诊断病例11例,2例家族性CJD和1例格-斯-斯综合征.病例的地理分布及职业无明显聚集性;临床诊断病例发病平均年龄56.7岁,男女比例8:9;疑似诊断病例发病平均年龄为57.4岁,男女比例5:6.快速进行性痴呆为最常见的首发症状,占全部诊断病例的33.3%.临床诊断病例比疑似诊断病例出现更多的典型临床表现.结论 2008年中国监测到的CJD病例以散发型为主,病例的地理分布、职业、性别比例以及平均年龄均符合散发型CJD的分布特点.     

中国2008年克雅氏病监测病例特征分析

目的 了解中国克雅氏病(CJD)的发病情况及流行病学、临床特征.方法 对2008年中国克雅氏病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,收集患者脑脊液及血液样品,利用Western blot方法榆测脑脊液中是否含有14-3-3蛋白,提取全血基因组DNA并利用PCR及测序方法对脚基因进行129位多态性及基因突变分析.结果 共发现散发型CJD临床诊断病例31例,疑似诊断病例11例,2例家族性CJD和1例格-斯-斯综合征.病例的地理分布及职业无明显聚集性;临床诊断病例发病平均年龄56.7岁,男女比例8:9;疑似诊断病例发病平均年龄为57.4岁,男女比例5:6.快速进行性痴呆为最常见的首发症状,占全部诊断病例的33.3%.临床诊断病例比疑似诊断病例出现更多的典型临床表现.结论 2008年中国监测到的CJD病例以散发型为主,病例的地理分布、职业、性别比例以及平均年龄均符合散发型CJD的分布特点.     

中国2008年克雅氏病监测病例特征分析

目的 了解中国克雅氏病(CJD)的发病情况及流行病学、临床特征.方法 对2008年中国克雅氏病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,收集患者脑脊液及血液样品,利用Western blot方法榆测脑脊液中是否含有14-3-3蛋白,提取全血基因组DNA并利用PCR及测序方法对脚基因进行129位多态性及基因突变分析.结果 共发现散发型CJD临床诊断病例31例,疑似诊断病例11例,2例家族性CJD和1例格-斯-斯综合征.病例的地理分布及职业无明显聚集性;临床诊断病例发病平均年龄56.7岁,男女比例8:9;疑似诊断病例发病平均年龄为57.4岁,男女比例5:6.快速进行性痴呆为最常见的首发症状,占全部诊断病例的33.3%.临床诊断病例比疑似诊断病例出现更多的典型临床表现.结论 2008年中国监测到的CJD病例以散发型为主,病例的地理分布、职业、性别比例以及平均年龄均符合散发型CJD的分布特点.     

中国2008年克雅氏病监测病例特征分析

目的 了解中国克雅氏病(CJD)的发病情况及流行病学、临床特征.方法 对2008年中国克雅氏病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,收集患者脑脊液及血液样品,利用Western blot方法榆测脑脊液中是否含有14-3-3蛋白,提取全血基因组DNA并利用PCR及测序方法对脚基因进行129位多态性及基因突变分析.结果 共发现散发型CJD临床诊断病例31例,疑似诊断病例11例,2例家族性CJD和1例格-斯-斯综合征.病例的地理分布及职业无明显聚集性;临床诊断病例发病平均年龄56.7岁,男女比例8:9;疑似诊断病例发病平均年龄为57.4岁,男女比例5:6.快速进行性痴呆为最常见的首发症状,占全部诊断病例的33.3%.临床诊断病例比疑似诊断病例出现更多的典型临床表现.结论 2008年中国监测到的CJD病例以散发型为主,病例的地理分布、职业、性别比例以及平均年龄均符合散发型CJD的分布特点.     

中国2008年克雅氏病监测病例特征分析

目的 了解中国克雅氏病(CJD)的发病情况及流行病学、临床特征.方法 对2008年中国克雅氏病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,收集患者脑脊液及血液样品,利用Western blot方法榆测脑脊液中是否含有14-3-3蛋白,提取全血基因组DNA并利用PCR及测序方法对脚基因进行129位多态性及基因突变分析.结果 共发现散发型CJD临床诊断病例31例,疑似诊断病例11例,2例家族性CJD和1例格-斯-斯综合征.病例的地理分布及职业无明显聚集性;临床诊断病例发病平均年龄56.7岁,男女比例8:9;疑似诊断病例发病平均年龄为57.4岁,男女比例5:6.快速进行性痴呆为最常见的首发症状,占全部诊断病例的33.3%.临床诊断病例比疑似诊断病例出现更多的典型临床表现.结论 2008年中国监测到的CJD病例以散发型为主,病例的地理分布、职业、性别比例以及平均年龄均符合散发型CJD的分布特点.