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Abstract：
Access to reliable, valid, accurate and responsive outcome measures is essential to ensure standards of care and clinical trial readiness in facioscapulohumeral dystrophy. Review aims: 1. identify and provide a descriptive summary of all outcome measures used to measure physical function. 2. systematically appraise the evidence on measurement properties (reliability, construct validity, measurement error and responsiveness) of performance-based outcome measures of physical function in individuals diagnosed with facioscapulohumeral dystrophy. Selected electronic health-related databases were searched from inception - Feb 2019. Two authors independently screened studies for eligibility and extracted data for psychometric evidence. The methodological quality of outcome measure studies was appraised using the consensus-based standards for the selection of health measurement instruments (COSMIN) checklist. Of 12 identified outcome measures, four required high-technology equipment. Only three were FSHD specific. The FSH-clinical score had \'moderate\' quality positive evidence for reliability. The remaining measures had \'low\' to \'very low\' quality evidence supporting properties of reliability, validity, responsiveness and measurement error. Identified studies tended towards low recruitment in middle-aged, ambulant individuals making results hard to generalise across lifespan and levels of severity. There is a paucity of measurement evidence supporting the use of outcome measures in people with facioscapulohumeral dystrophy.
摘要：
访问可靠，有效,准确和反应灵敏的结果测量对于确保面肩肱动脉营养不良的护理标准和临床试验准备情况至关重要.审查目的:1.识别并提供用于测量身体功能的所有结果测量的描述性摘要。2.系统地评估关于测量特性的证据(可靠性，构造效度，测量误差和响应性）诊断为面额肱骨营养不良的个体基于表现的身体功能结果测量。从2019年2月开始搜索选定的电子健康相关数据库。两位作者独立筛选了研究的资格，并提取了心理测量证据的数据。使用基于共识的健康测量仪器选择标准（COSMIN）清单评估结果测量研究的方法学质量。在确定的12项成果指标中，四个需要的高技术设备。只有三个是FSHD特异性的。FSH临床评分具有“中等”质量的可靠性阳性证据。其余措施具有“低”到“非常低”的质量证据，支持可靠性的属性，有效性，响应性和测量误差。已确定的研究倾向于中年人的低招募，使结果难以在寿命和严重程度上推广的走动个体。很少有测量证据支持在面额肱骨营养不良患者中使用结果测量。
