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

  • Saskia Koene
  • Ilse Dirks
  • Esmee van Mierlo
  • Pascal R. de Vries
  • Anjo J. W. M. Janssen
  • Jan A. M. Smeitink
  • Arjen Bergsma
  • Hans Essers
  • Kenneth Meijer
  • Imelda J. M. de Groot
Research Report
Part of the JIMD Reports book series (JIMD, volume 36)

Abstract

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.

Keywords

3D accelerometry Children Daily physical activity Mitochondrial disease Outcome measures 

Supplementary material

459230_1_En_35_MOESM1_ESM.xlsx (13 kb)
Supplementary Table 1 Characteristics of patients and their age- and sex-matched controls. A level of p = 0.05 was used for significance; significant values are indicated in bold; BMI Body Mass Index (XLSX 13 kb)
459230_1_En_35_MOESM2_ESM.xlsx (15 kb)
Supplementary Table 2 Patient characteristics. Genetic, biochemical and clinical details for each patient. Psychomotor retardation is defined: IQ <50 = severe; IQ 50–70 = mild and IQ >70 = normal. 1 = genetically confirmed primary mitochondrial disease; 2 = genetically confirmed secondary mitochondrial disease; 3 = biochemically confirmed mitochondrial dysfunction. # siblings, A ankle, CI complex I, F female, GMFM gross motor function measure, L leukocytes, M male, Mu muscle, ND not done, PEDI pediatric evaluation of disability inventory, PMR psychomotor retardation, UEC urinary epithelial cells (XLSX 14 kb)
459230_1_En_35_MOESM3_ESM.xlsx (9 kb)
Supplementary Table 3 Correlations between the GMFM and the PEDI and activity parameters measured with the accelerometer. A level of p = 0.001 was used for significance; significant values are indicated in bold; AUC area under the curve, GMFM gross motor function measure, PEDI pediatric evaluation of disability inventory (XLSX 9 kb)
459230_1_En_35_MOESM4_ESM.gif (198 kb)
Supplementary Fig. 1 Percentage of rest during the weekend for patients versus matched controls. The dark bars represent the patients and the light bars represent their matched controls. Group 1 = genetically confirmed primary mitochondrial disease; group 2 = genetically confirmed secondary mitochondrial disease; group 3 = biochemically confirmed mitochondrial dysfunction (PNG 10 kb)

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Copyright information

© SSIEM and Springer-Verlag Berlin Heidelberg 2017

Authors and Affiliations

  • Saskia Koene
    • 1
  • Ilse Dirks
    • 1
  • Esmee van Mierlo
    • 1
  • Pascal R. de Vries
    • 1
  • Anjo J. W. M. Janssen
    • 2
  • Jan A. M. Smeitink
    • 1
  • Arjen Bergsma
    • 3
  • Hans Essers
    • 4
  • Kenneth Meijer
    • 4
  • Imelda J. M. de Groot
    • 1
    • 3
  1. 1.Department of Paediatrics, Radboud Center for Mitochondrial Medicine (RCMM)Radboud University Medical CenterNijmegenThe Netherlands
  2. 2.Donders Center for Neuroscience, Department of Rehabilitation, Pediatric Physical TherapyRadboud University Medical CenterNijmegenThe Netherlands
  3. 3.Donders Center for Neuroscience, Department of RehabilitationRadboud University Medical CenterNijmegenThe Netherlands
  4. 4.Department of Human Movement SciencesMaastricht University Medical CentreMaastrichtThe Netherlands

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