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- W2904064013 abstract "ABSTRACT This investigation evaluated the reliability and validity of the 6‐Minute Walk Test (6MWT) in patients with pediatric hypophosphatasia (HPP). Children (aged 6 to 12 years; n = 11), adolescents (13 to 17 years; n = 4), and adults (18 to 65 years; n = 9) completed the 6MWT at screening and baseline in two clinical studies of asfotase alfa. Test‐retest reliability of the 6MWT, evaluated with Pearson's correlation coefficients ( r ) for screening versus baseline, was high for children ( r = 0.95; p < 0.0001), adolescents ( r = 0.81; p = 0.125), and adults ( r = 0.94; p = 0.0001). The most conservative minimal clinically important differences, estimated using distribution‐based methods, were 31 m (children and adults) and 43 m (adolescents). In children, the 6MWT correlated significantly with scores on measures of skeletal disease, which included the Radiographic Global Impression of Change scale ( r = 0.50; p < 0.0001) and the Rickets Severity Scale ( r = −0.78; p < 0.0001), such that distance walked increased as the severity of skeletal disease decreased. Significant ( p < 0.0001) correlations with the 6MWT distance walked were also observed for children with scores on parent‐reported measures of disability ( r = −0.67), ability to function in activities of daily living ( r = 0.71 to 0.77), and parent‐reported measures of pain ( r = −0.39). In adolescents and adults, 6MWT distance walked correlated significantly ( p < 0.05) with measures of lower extremity function ( r = 0.83 and 0.60, respectively), total pain severity ( r = −0.41 and −0.36, respectively), and total pain interference ( r = −0.41 and −0.49, respectively). Collectively, these data indicate that the 6MWT is a reliable, valid measure of physical functioning in patients with pediatric HPP. © 2018 The Authors. JBMR Plus Published by Wiley Periodicals, Inc. on behalf of the American Society for Bone and Mineral Research." @default.
- W2904064013 created "2018-12-22" @default.
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- W2904064013 date "2019-03-01" @default.
- W2904064013 modified "2023-10-18" @default.
- W2904064013 title "Reliability and Validity of the 6-Minute Walk Test in Hypophosphatasia" @default.
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- W2904064013 doi "https://doi.org/10.1002/jbm4.10131" @default.
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