...
  • 主题
高级搜索  >
历史搜索 清空历史
首页> 外文期刊>European journal of neurology: the official journal of the European Federation of Neurological Societies >Pseudo‐anticipation in Creutzfeldt–Jakob disease is due to a rhomboid‐shaped artifact
【24h】

Pseudo‐anticipation in Creutzfeldt–Jakob disease is due to a rhomboid‐shaped artifact

机译：在Creutzfeldt-Jakob疾病中的伪预期是由于菱形形状的伪影

获取原文
获取原文并翻译 | 示例
           
页面导航
  • 摘要
  • 著录项
  • 相似文献
  • 相关主题

摘要

Background and purpose Previous studies have reported conflicting results regarding possible anticipation in familial E200K Creutzfeldt–Jakob disease (fCJD). Our objective was to use a large database to assess the age of disease onset (AODO) in CJD. Methods The study population included 477 CJD patients [266 with fCJD, 145 with sporadic CJD (sCJD) and 66 patients of Libyan origin but negative family history] from the Israeli registry of CJD conducted since 1954. In all patients, AODO in relatives and family trees was documented. Comparison of AODO was done using a paired t test and regression using Pearson correlation for birth and year of onset. Results The initial analysis in 52/73 families in which more than one generation was affected revealed an AODO of 63.30?±?9.44 in the first generation compared to 56.96?±?8.99 in the second generation ( P ??0.001). However, inspection of individual AODO values plotted by year of birth showed a clear rhomboid methodological artifact generated by missing data of many young onset CJD patients who died before the database began to function in 1954 and of many late onset CJD patients missing at the present time since they will only develop the disease in the future. The ‘generation’ effect completely disappears if analysis is performed by year of disease onset or for the periods in which complete data are available. Conclusions In this very large dataset, true anticipation in fCJD patients was not detected. It is plausible that previous reports supporting the presence of anticipation are biased by a rhomboid‐shaped data availability artifact.
机译：背景和目的以前的研究报告了关于家庭E200K Creutzfeldt-Jakob疾病（FCJD）可能预期的矛盾的结果。我们的目标是使用大型数据库来评估CJD中疾病的年龄（AODO）。方法研究人群包括477名CJD患者[266，与FCJD，145名与零星CJD（SCJD）和66名利比亚患者]来自1954年以来的CJD注册机构。在所有患者中，亲属和家庭的AODO树被记录在案。使用Pearson相关性与出生年份的Pearson相关性进行AODO对AODO进行的比较。结果52/73家族的初步分析，其中多一代受到影响，在第一代中显示了63.30的AODO，而第二代中的56.96？±8.99（p≤≤0.001）。然而，在出生年份绘制的个人AODO值的检查表明，在1954年在数据库开始在数据库开始运作之前，许多年轻发病CJD患者的缺失数据产生了明确的菱形方法工件，并且许多晚期发病CJD患者目前缺失因为他们将来只会发展这种疾病。如果分析在疾病发作年份或可用数据的期间进行分析，则“生成”效果完全消失。结论在这一非常大的数据集中，未检测到FCJD患者的真正预期。它是合理的，之前的报告支持预期的存在是由菱形数据可用性伪影偏置的。

著录项

相似文献

  • 外文文献
  • 中文文献
  • 专利
获取原文

客服邮箱：kefu@zhangqiaokeyan.com

京公网安备：11010802029741号 ICP备案号：京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有

  • 客服微信

  • 服务号