Australian National Creuztfeldt-Jakob Disease Registry, Department of Pathology, The University of Melbourne, , Parkville, Australia.
J Neurol Neurosurg Psychiatry. 2013 Dec;84(12):1372-7. doi: 10.1136/jnnp-2012-304820. Epub 2013 Aug 21.
Prospective national screening and surveillance programmes serve a range of public health functions. Objectively determining their adequacy and impact on disease may be problematic for rare disorders. We undertook to assess whether objective measures of disease surveillance intensity could be developed for the rare disorder sporadic Creutzfeldt-Jakob disease (CJD) and whether such measures correlate with disease incidence.
From 10 countries with national human prion disease surveillance centres, the annual number of suspected prion disease cases notified to each national unit (n=17,610), referrals for cerebrospinal fluid (CSF) 14-3-3 protein diagnostic testing (n=28,780) and the number of suspect cases undergoing diagnostic neuropathological examination (n=4885) from 1993 to 2006 were collected. Age and survey year adjusted incidence rate ratios with 95% CIs were estimated using Poisson regression models to assess risk factors for sporadic, non-sporadic and all prion disease cases.
Age and survey year adjusted analysis showed all three surveillance intensity measures (suspected human prion disease notifications, 14-3-3 protein diagnostic test referrals and neuropathological examinations of suspect cases) significantly predicted the incidence of sporadic CJD, non-sporadic CJD and all prion disease.
Routine national surveillance methods adjusted as population rates allow objective determination of surveillance intensity, which correlates positively with reported incidence for human prion disease, especially sporadic CJD, largely independent of national context. The predictive relationship between surveillance intensity and disease incidence should facilitate more rapid delineation of aberrations in disease occurrence and assessment of the adequacy of disease monitoring by national registries.
前瞻性国家筛查和监测计划具有多种公共卫生功能。对于罕见疾病，客观确定其充分性和对疾病的影响可能存在问题。我们着手评估是否可以为罕见疾病散发性克雅氏病（CJD）制定客观的疾病监测强度指标，以及这些指标是否与疾病发病率相关。
从 10 个拥有国家朊病毒疾病监测中心的国家中，收集了每个国家单位报告的疑似朊病毒疾病病例的年度数量（n=17610）、脑脊液（CSF）14-3-3 蛋白诊断检测转诊数量（n=28780）和进行诊断性神经病理学检查的疑似病例数量（n=4885），这些数据均来自 1993 年至 2006 年。使用泊松回归模型估计年龄和调查年份调整后的发病率比及其 95%可信区间，以评估散发性、非散发性和所有朊病毒疾病病例的危险因素。
年龄和调查年份调整分析表明，所有三种监测强度指标（疑似人类朊病毒疾病报告、14-3-3 蛋白诊断检测转诊和疑似病例的神经病理学检查）均显著预测了散发性 CJD、非散发性 CJD 和所有朊病毒疾病的发病率。
调整为人群率的常规国家监测方法允许客观确定监测强度，与人类朊病毒疾病，尤其是散发性 CJD 的报告发病率呈正相关，在很大程度上独立于国家背景。监测强度与疾病发病率之间的预测关系应有助于更快速地确定疾病发生的异常情况，并评估国家登记处对疾病监测的充分性。
