Seefried Lothar, Genest Franca
University of Würzburg, Würzburg, Germany.
Bone Rep. 2025 May 29;25:101853. doi: 10.1016/j.bonr.2025.101853. eCollection 2025 Jun.
Hypophosphatasia (HPP) is a rare disease characterized by skeletal and nonskeletal manifestations that can increase patient disability. The 6-Minute Walk Test (6MWT) is frequently used to assess mobility in patients with HPP, although the test is laborious to conduct in clinical practice. The purpose of the current study was to determine correlations between time to complete the 6MWT, time to complete the Chair-Rise Test (CRT), and scores on the Lower Extremity Functional Scale (LEFS) in adults with HPP.
Pearson correlations between time to complete outcomes on the 6MWT and CRT, time to complete the 6MWT and scores on the LEFS, and time to complete the CRT and scores on the LEFS were calculated using de-identified data from adults with HPP who had first onset of symptoms in childhood. All patients were enrolled in the previously conducted, observational EmPATHY study.
Pearson correlation analyses showed inverse correlations between 6MWT and CRT outcomes ( = -0.584) and between CRT and LEFS outcomes ( = -0.596) and a direct correlation between 6MWT and LEFS outcomes ( = 0.808).
Time to complete the 6MWT was correlated with time to complete the CRT and scores on the LEFS in adults with HPP. CRT and LEFS may be suitable, expeditious options to amend or substitute 6MWT when assessing functional status in patients with HPP.
低磷性佝偻病(HPP)是一种罕见疾病,其特征为骨骼和非骨骼表现,可增加患者残疾程度。6分钟步行试验(6MWT)常用于评估HPP患者的活动能力,尽管该试验在临床实践中实施起来较为费力。本研究的目的是确定成年HPP患者完成6MWT的时间、完成椅子起立试验(CRT)的时间与下肢功能量表(LEFS)评分之间的相关性。
利用来自童年期首次出现症状的成年HPP患者的匿名数据,计算6MWT和CRT完成结果的时间之间、6MWT完成时间与LEFS评分之间以及CRT完成时间与LEFS评分之间的Pearson相关性。所有患者均纳入先前进行的观察性同理心研究。
Pearson相关性分析显示,6MWT与CRT结果之间呈负相关(r = -0.584),CRT与LEFS结果之间呈负相关(r = -0.596),6MWT与LEFS结果之间呈正相关(r = 0.808)。
成年HPP患者完成6MWT的时间与完成CRT的时间及LEFS评分相关。在评估HPP患者的功能状态时,CRT和LEFS可能是修正或替代6MWT的合适、快捷的选择。