The study aims to identify which Syde®-derived digital outcomes are reliable in FGFR3-related Skeletal Dysplasia. This requires to set-up a natural history study to measure limb movements in patients with ACH or HCH.
Study Type
OBSERVATIONAL
Enrollment
40
Hopital Necker
Paris, France
RECRUITINGReliability of digital endpoint derived from Syde® assessed by the Intra-Class Correlation (ICC) for each recording period timepoint.
reliability assessed by the ICC (Intra-Class Correlation)for each recording period timepoint (baseline, 6 months, 12 months)
Time frame: 12 months
number of participants reaching 180 hours of recording during the recording periods defined in the protocol
Number of patients reaching 180 hours of recordings during the recording periods defined in the protocol (supposed optimal threshold for Syde®-derived digital endpoint computation) at baseline, 6 months, 12 months
Time frame: 12 months
French System Usability Score (F-SUS) score at 6 months of Syde® use
French System Usability Score (F-SUS) at 6 months. The System Usability Scale is a 10-item questionnaire . It is a simple tool to measure the usability of an interactive system and it is widely used. For each item, the user is asked to express their level of agreement or disagreement from 1 (strongly disagree) and 5 (strongly agree).
Time frame: 6 months
Correlation between digital endpoints derived from Syde® and 6-minutes walk test (6MWT)
Correlation at baseline and at different timepoints between Syde® derived digital endpoint and 6MWT
Time frame: 12 months
Correlation between digital endpoints derived from Syde® and growth parameters
Correlation at baseline and at different timepoints between Syde® derived digital endpoints and annualized growth velocity, height Z-score, body proportion ratios
Time frame: 12 months
Correlation between digital endpoints derived from Syde® and Patient-reported Outcomes (PROs)
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Correlation at baseline and at different timepoints between Syde® derived digital endpoints and PROs
Time frame: 12 months
Longitudinal Change in digital endpoints derived from Syde®
Change from baseline in digital endpoints derived from Syde® at 6 and 12 months
Time frame: 12 months
Syde® movement reconstruction accuracy compared to Optical Motion Capture measures.
Stride detection accuracy of Syde®, stride length and stride velocity accuracy of Syde® and upper limb measures, compared to Optical Motion Capture measures
Time frame: Baseline
Qualitative interview exploring limitations on mobility and impact on daily life
caregiver and/or participant qualitative interview exploring limitations on mobility daily life
Time frame: baseline