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Introduction Mucopolysaccharidoses (MPSs) are a group of rare diseases caused by an intralysosomal accumulation of glycosaminoglycans, resulting in a multisystemic clinical condition characterized by variable degrees of physical‐functional impairment. Objective To evaluate the functional capacity (FC) of MPS patients and compare with a healthy control group. Methods This is a cross‐sectional study of 6‐ to 39‐year‐old patients followed at a medical reference center and compared with their control peers, matched by age and sex. FC was assessed using the Sit‐to‐Stand Test (SST) and Incremental Shuttle Walk Test (ISWT). Heart rate (HR) and Borg rating of perceived exertion were measured before and after ISWT. HR recovery (HRR) was defined as the HR at the end of the test minus the HR in the second minute after ISWT. Results Nineteen (19) MPS patients, 69% with type II MPS and mean age 17 ± 11 years were evaluated. Every patient was under enzyme replacement therapy. The time to perform the SST was longer in the MPS group (10.6 ± 2.5 s vs. 6.7 ± 1.2 s; p < .01). The MPS group achieved lower values of distance covered on the ISWT (407.6 ± 329.8 m vs. 1131.9 ± 183.3 m; p < .01), with a significantly higher Borg (6 [5–8] vs. 2 [1–4]; p = .02). The MPS group's HRR was slower than the controls (32.9 ± 20.2 beats per minute [bpm] vs. 69.1 ± 25.9 bpm; p < .001). Discussion We observed a pronounced reduction in the MPS group's FC compared to their healthy peers and a worse HRR after completing the test.

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This method depends on patient understanding; the operator and measurement variability must also be considered. Several studies have assessed the reliability of chest wall mobility. Malaguti et al. (9) found a substantial intraclass correlation coeffi cient (ICC: 0.89 [95% confi dence interval, CI, 0.75 -0.95]) between two chest wall mobility measurements in chronic obstructive pulmonary disease (COPD) patients. Similar ICC values were reported in other studies of healthy volunteers (10,11).External stimuli can infl uence tests reliant on patient cooperation. AbstractBackground: Visual feedback could optimize the evaluation of chest wall mobility. Objective: To compare chest wall mobility performed with and without visual feedback. Methods: Cross-sectional study, 64 healthy volunteers, average age 24 years old. The thorax circumference was measured at two locations, the axillary and xiphisternum regions, by a tape placed at the anterior axillary line and at the xiphoid process. The evaluations were performed: (i) after verbal explanation (without visual feedback), (ii) after verbal explanation plus visual feedback (with visual feedback); for this evaluation the volunteer remained in front of the computer, which provides visual feedback. Results: There was no difference between axillary measurement with vs. without feedback (7.5 Ϯ 2.4 cm vs. 7.9 Ϯ 3.3 cm, respectively, p ϭ 0.215), but signifi cant difference between xiphisternum measurement 7.1 Ϯ 2.7cm with feedback vs. 6.5 Ϯ 2.3cm without feedback, p ϭ 0.043. Low intraclass correlation coeffi cients were observed for the axillary measurement [0.51 (0.19 -0.72, p ϭ 0.003) and for xiphisternum measurement [0.60 (0.35 -0.76, p Ͻ 0.001) performed with and without feedback, respectively. Conclusion: Thoracic mobility presents great variability when compared with and without feedback, so the visual stimulus must be taken into account in this evaluation.