肯尼迪病1234量表的初步设计和检验

Establishment and validation of a new assessment scale for Kennedy＇ s disease

目的根据肯尼迪病患者的临床特点，设计一种针对肯尼迪病患者的临床评估量表，并初步验证量表的信度和效度。方法在肌萎缩侧索硬化功能等级量表的基础上设计出“肯尼迪病1234量表”，对2009年6月至2014年3月在北京大学第三医院神经科门诊或病房就诊的81例基因确诊的肯尼迪病患者进行“肯尼迪病1234量表”评分，进而检验“肯尼迪病1234量表”的信度和效度。采用Cronbaeh’Sd系数和分半信度检验量表的信度，效度检验采用内容效度和结构效度。结果总量表的Cronbaeh’s仪系数为0．789，分半信度为0．868；呼吸与总分的相关系数0．3-0．4，语言、吞咽、书写与总分的相关系数为0．4-0．5，流涎、切分食物及手持器皿、穿衣和个人卫生、卧床和调整被褥、行走、上楼梯6项条目与总分的相关系数均大于0．5（P〈0．01）；探索性因素分析提取的4个公因子符合量表编制的理论结构，各条目在其公因子的负荷值为0．541～0．864；量表评分与患者病程的回归系数为-0．274，随着病程的延长，总分逐渐降低。结论“肯尼迪病1234量表”设计简便、易操作，具有良好的信度和效度，与病程具有相关性，可较好地对肯尼迪病患者的病情进行定量评价，为临床研究提供服务。

Objective To develop a scale to assess Kennedy＇s disease （KD） based on patients＇ clinical characteristics and to validate its application in patients. Methods We developed KD1234 Scale following the model of Amyotrophic Lateral Sclerosis-Functional Rating Scale. Using this new scale, we assessed patients with genetic diagnosis of KD and evaluated the reliability and validity of the new scale. Reliability was analyzed using Cronbach＇ s α coefficient and split-half reliability. Validity was analyzed by content validity and structure validity. Results The Cronbach＇ s α coefficient of the total scale was 0. 789 and split-half reliability coefficient was 0. 868. The α of breathing with total score was 0. 3 - 0. 4, α of written, language and swallowing with the total score was 0. 4 - 0. 5, α of other items with the total score was 〉0. 5 （P 〈0. 01 in each of above α）. Four common factors extracted by exploratory factor analysis had well correspondence between the scale construction and the theoretical construction. Factor loading was ranged from 0. 541 to 0. 864 for each item. The duration of the disease showed some correlation with the score of KD1234 Scale. Conclusions The KD1234 Scale demonstrates high reliability and validity. The duration of the disease indicates negative correlation with KD1234 Scale scores. The KD1234 Scale can be used in clinical research to evaluate the condition of KD patients quantitatively.