BACKGROUND This report highlights a rapidly progressive case of Creutzfeldt-Jakob Disease (CJD) whose time from symptom onset to death spanned less than two months. We also explore the most recently available in-patient demographics data for discharges with CJD in the United States. METHODS We reviewed a CJD case and systematically analyzed a retrospective cohort of CJD discharges using the Healthcare Cost and Utilization Project (HCUP) to evaluate the existing national data on the status of CJD demographics and dispositions in the United States in 2016. RESULTS An estimated total of 710 hospital discharges with a diagnosis of CJD were seen across the United States in 2016. According to HCUP, the average age of patients was 66.15 ± 11.54 years with 48.6 % female. Average time to intubation from admission to hospital was 4.71 ± 7.32 days with a rate of intubation of 6.34 %. The mean hospital cost was $19,901.25 ± $18,743.48. The rate of in-hospital mortality was 8.45 %. No significant geographical differences were noted (p = 0.49). No significant differences were seen among incidence in specific ethnic groups (p = 0.33) or income quartiles (p = 0.90). CONCLUSIONS Our data shows that the incidence of CJD in 2016 appears to be equally distributed among individuals in the United States by demographic categories. Additionally, our case-study from 2019 illustrates an important example for diagnosing a rapidly-progressing case of CJD.

中文翻译：

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克雅氏病：2016年美国全国数据住院人口统计报告及快速进展病例回顾


背景本报告重点介绍了一个快速进展的克雅氏病（CJD）病例，其从症状出现到死亡的时间不到两个月。我们还探讨了美国克雅氏病出院患者的最新人口统计数据。方法 我们回顾了一个克雅氏病病例，并使用医疗保健成本和利用项目 (HCUP) 系统地分析了克雅氏病出院的回顾性队列，以评估 2016 年美国克雅氏病人口统计和处置状况的现有国家数据。 结果 估计总数2016 年，美国有 710 例被诊断为克雅氏病的患者出院。根据 HCUP 的数据，患者的平均年龄为 66.15 ± 11.54 岁，其中 48.6% 为女性。从入院到插管的平均时间为 4.71 ± 7.32 天，插管率为 6.34%。平均住院费用为 19,901.25 美元 ± 18,743.48 美元。院内死亡率为8.45%。没有发现显着的地理差异（p = 0.49）。特定种族群体 (p = 0.33) 或收入四分位数 (p = 0.90) 的发病率没有显着差异。结论 我们的数据显示，2016 年克雅氏病的发病率似乎在美国不同人口类别的个体中分布均匀。此外，我们 2019 年的案例研究展示了诊断快速进展的克雅氏病病例的一个重要例子。