title: Cardiorespiratory fitness, physical activity, and quality of life in patients with McArdle disease.
abstract: BACKGROUND
  This study sought to determine whether health-related quality of life (HRQoL) could be related to cardiorespiratory fitness (CRF) and/or physical activity (PA) in patients with McArdle disease and to compare the CRF and HRQoL data obtained with normative data for age- and sex-matched healthy subjects.
  
  
  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 the Spanish general population.
  
  
  CONCLUSIONS
  Patients showed a consistent link between higher physical HRQoL scores and higher CRF. Patients fulfilling leisure time PA recommendations showed higher CRF and physical HRQoL scores than those not meeting guideline recommendations. According to normative data for healthy subjects, CRF and physical HRQoL are severely impaired in adult patients with McArdle disease.
authors: 1Department of Sport and Informatics, Section of Physical Education and Sports, Faculty of Sport, Universidad Pablo de Olavide, Sevilla, SPAIN; and 2European University and Research Institute Hospital 12 de Octubre, Madrid, SPAIN.
authors: Diego
authors: D
authors: Munguía-Izquierdo
authors: Alfredo
authors: A
authors: Santalla
authors: Alejandro
authors: A
authors: Lucia
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015992:Body Mass Index; D002320:Cardiovascular Physiological Phenomena; D005260:Female; D006012:Glycogen Storage Disease Type V / Q000503:physiopathology*; D006801:Humans; D007899:Leisure Activities; D008297:Male; D008875:Middle Aged; D009043:Motor Activity / Q000502:physiology*; D010101:Oxygen Consumption; D010809:Physical Fitness; D011788:Quality of Life; D012143:Respiratory Physiological Phenomena; D011795:Surveys and Questionnaires; D055815:Young Adult