Natural variability of daily physical activity measured by accelerometry in children with a mitochondrial disease.

In this exploratory study we aimed to select the most valid and feasible accelerometer to measure daily physical activity at home in 10 children with mitochondrial disease. Using the experimentally-selected GENEActiv, good to excellent short- and long-term test-retest reliability of daily physical activity was found. Especially in children with more severe limitations daily physical activity seemed more stable and less susceptible to weather conditions. Moreover, small standard errors of measurement were found, indicating high precision of this measuring method. We conclude that measuring daily physical activity using accelerometry is a promising outcome measure for future studies in this unique population.

Domains of Daily Physical Activity in Children with Mitochondrial Disease: A 3D Accelerometry Approach.

Feasible, sensitive and clinically relevant outcome measures are of extreme importance when designing clinical trials. For paediatric mitochondrial disease, no robust end point has been described to date. The aim of this study was to select the domains of daily physical activity, which can be measured by 3D accelerometry, that could serve as sensitive end points in future clinical trials in children with mitochondrial disorders.In this exploratory observational study, 17 patients with mitochondrial disease and 16 age- and sex-matched controls wore 3D accelerometers at the upper leg, upper arm, lower arm and chest during one weekend. Using the raw data obtained by the accelerometers, we calculated the following outcome measures: (1) average amount of counts per hour the sensors were worn; (2) the maximal intensity; (3) the largest area under the curve during 30 min and (4) categorized activities lying, standing or being dynamically active. Measuring physical activity during the whole weekend was practically feasible in all participants. We found good face validity by visually correlating the validation videos and activity diaries to the accelerometer data-graphs. Patients with mitochondrial disorders had significantly lower peak intensity and were resting more, compared to their age- and sex-matched peers.Finally, we suggest domains of physical activity that could be included when measuring daily physical activity in children with mitochondrial disorders, preferably using more user-friendly devices. These include peak activity parameters for the arms (all patients) and legs (ambulatory patients). We recommend using or developing devices that measure these domains of physical activity in future clinical studies.