Resumen:
METHODS:
Eighty-one adult patients with McArdle disease underwent aerobic capacity testing to determine peak oxygen uptake (V˙O2peak), among other variables. HRQoL (Short Form 36-Item Health Survey questionnaire version 2 (SF-36 version 2)) and PA (International Physical Activity Questionnaire) questionnaires were completed by 45 of the patients. HRQoL and V˙O2peak data were compared with published normative data.
RESULTS:
Positive correlations were observed between V˙O2peak and leisure time PA versus the physical component summary score and scores for several domains of the SF-36 questionnaire after adjusting for age, body mass index, and disease severity (R values, 0.42-0.68; all P < 0.01). In a regression analysis, the physical component summary score was directly linked to V˙O2peak (B = 1.28; 95% confidence interval, 0.78-1.78; P < 0.001; R = 0.422). The mean V˙O2peak recorded for patients with McArdle disease was 57% lower than the normative value (17.1 ± 5.3 vs 40.0 ± 9.5 mL·kg·min, respectively; P < 0.001). All patients showed a CRF below their age-/sex-matched normality value and scored clinically lower in the physical component summary and in most SF-36 domains compared with t...