CAJ | 학술논문

目的通过前瞻性研究方法 ,揭示诊断时的修改版神经功能评分(AISFRS-R)总分和ALSFRS-R的线性变化率(AFS)与生存时间的相关性.方法连续收集2002年1月至2005年12月我院收治的确诊和拟诊的肌萎缩侧索硬化(ALS)患者129例,记录临床资料、用力肺活量(FVC)和ALSFRS-R,患者每3个月进行随访,直至死亡或气管切开.结果患者的平均发病年龄为(52±11)岁,中位生存时间为45.71个月(95%的可信区间为35～51个月).用Kaplan-Meier单因素分析的方法 ,发现ALSFRS-R和AFS均为生存时间的影响因素,差异有统计学意义(P<0.05).进一步用Cox风险比例模型分析,△FS和用力肺活量为生存时间最有力的影响因子(P<0.05).△FS下降一个单位,死亡风险下降73.3%.结论 ALSFRS-R可以敏感反应病情变化,预测生存时间,可以作为临床试验的主要观测指标.△FS代表疾病早期的发展速度,是较ALSFRS-R和确诊时间更有力的影响因素.
목적 통과전첨성연구방법 ,게시진단시적수개판신경공능평분(AISFRS-R)총분화ALSFRS-R적선성변화솔(AFS)여생존시간적상관성.방법 련속수집2002년1월지2005년12월아원수치적학진화의진적기위축측색경화(ALS)환자129례,기록림상자료、용력폐활량(FVC)화ALSFRS-R,환자매3개월진행수방,직지사망혹기관절개.결과 환자적평균발병년령위(52±11)세,중위생존시간위45.71개월(95%적가신구간위35～51개월).용Kaplan-Meier단인소분석적방법 ,발현ALSFRS-R화AFS균위생존시간적영향인소,차이유통계학의의(P<0.05).진일보용Cox풍험비례모형분석,△FS화용력폐활량위생존시간최유력적영향인자(P<0.05).△FS하강일개단위,사망풍험하강73.3%.결론 ALSFRS-R가이민감반응병정변화,예측생존시간,가이작위림상시험적주요관측지표.△FS대표질병조기적발전속도,시교ALSFRS-R화학진시간경유력적영향인소.
Objective To identify the correlation between the revised amyotrophic lateral sclerosis (ALS) functional rating scale (ALSFRS-R)and the progression rate of ALSFRS-R (△FS) at time of diagnosis and survival time in a prospective survey. Methods A total of 129 consecutive patents with definite or probable ALS were enrolled at our hospital from January 2002 to December 2005. The patients were monitored with a standard evaluation form recording clinical features, ALSFRS-R and forced vital capacity (FVC) every 3 months from visit to death or tracheostomy. Results The mean age at onset was (52±11)years old. The median survival time from symptom onset was 45.71 months (95% CI = 35 to 51). In univariate analysis of Kaplan-Meier method, the outcome was significantly related to total ALSFRS-R and △FS (P < 0.05). In Cox multivariate model, △FS and FVC% were both statistically significant predictors of survival after adjusting for age and onset site(P <0.05). Al-unit decrease in △FS was associated with a 73.3% decreased risk of death. Conclusion ALSFRS-R is sensitive to the change over time and can predict survival at diagnosis. It can be used as a primary outcome measure in ALS clinical trial. △FS at diagnosis can provide us the information of disease progression at one particular point. It is a more significant predictor of survival in patients with ALS as compared to total ALSFRS-R and time from onset to diagnosis.