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Table 1 Characteristics of the study population

From: Positive association between physical outcomes and patient-reported outcomes in late-onset Pompe disease: a cross sectional study

Demographic and clinical characteristics Patients (n = 121)
 Women: number (%) 64 (53)
 Age at examination in years: median (range) 53 (23–79)
 Disease durationa at examination in years: median (range) 16 (0.2–50)
 Wheelchairb use at examination: number (%) 54 (45)
 Respiratory support at examination: number (%) 32 (26)
Physical outcomes Median (range)
 FVC upright (% pred) 77 (10–117)
 FVC supine (% pred) 63 (13–107)
 HHD sum score (% max) 81 (21–100)
 MRC sum score (% max) 85 (39–100)
 6MWT (% pred) 67 (6–120)
PROMs Median (range)
 SF-36 PCS score (norm-based) 33 (17–63)
 SF-36 MCS score (norm-based) 48 (19–72)
 RHS score 29 (11–36)
 R-PAct score 60 (7–100)
  1. FVC Forced Vital Capacity, HHD Hand-Held Dynamometry, MRC Medical Research Council, 6MWT Six-Minute Walk Test, SF-36 Short-Form 36 Health Survey, RHS Rotterdam Handicap Scale, R-Pact Rash-built Pompe-Specific Activity scale
  2. aduration from symptom onset
  3. bpartial and permanent wheelchair use