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Peak Jump Power Reflects the Degree of Ambulatory Ability in Patients with Mitochondrial and Other Rare Diseases

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JIMD Reports, Volume 33

Part of the book series: JIMD Reports ((JIMD,volume 33))

Abstract

Metabolic diseases that lead to neuromuscular, bone, and joint involvement can reduce ambulation and quality of life. Using jumping mechanography, we developed a novel assessment, peak jump power (PJP), and related this to ambulatory ability in patients either having a known or suspected underlying rare disease. From adults and children, we recruited 88 healthy controls and 115 patients (61 with mitochondrial disease and 54 with another diagnosis). Patients were categorized as having no complaints of weakness or ambulation (ambulatory competent; AC), weakness but able to ambulate without aids (ambulatory weakness; AW), or not able to ambulate without aids such as a walker, cane, or wheelchair (ambulatory assistance; AA). Subjects were asked to perform five successive jumps from a squat position. Instantaneous power (W; watts) was calculated and the highest result was divided by the body mass (kg) to calculate PJP (W/kg). Between healthy controls and AC patients, there was no difference in mean PJP (20.5 ± 7.0 W/kg vs. 19.0 ± 7.4 W/kg, p = 0.601; mean ± SD). Progressively lower results were found in patients with AW with a mean PJP of 11.7 ± 5.1 W/kg (p < 0.001 versus AC) and further those with AA with a mean PJP of 5.8 ± 3.2 W/kg (p < 0.001 versus AW). A subgroup analysis of subjects showed that those who did not use ambulatory aids all had a PJP above 10 W/kg. Using this threshold, the receiver operating characteristic curve (ROC) analysis showed PJP to be highly sensitive evaluation of ambulatory ability (sensitivity 95.8%, specificity 52.1%).

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Abbreviations

6MWT:

Six-minute walk test

ANOVA:

Analysis of variance

AUROC:

Area under the ROC curve

DCMA:

Dilated cardiomyopathy with ataxia

DMD:

Duchene muscular dystrophy

F :

Force

FSHD:

Facioscapulohumeral muscular dystrophy

GSD:

Glycogen storage disorder type V or VII

MPS-I or II:

Mucopolysaccharide storage disease

MRC:

Medical Research Council

MS:

Multiple sclerosis

P :

Power

PJP:

Peak jump power

ROC:

Receiver operating characteristic curve

SD:

Standard deviation

SSC:

Stretch shortening cycle

V :

Velocity

W:

Watts

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Acknowledgments

This research was supported by the following: Alberta Health Services, Riddell Movement Assessment Centre, and Alberta Children’s Hospital for the use of facilities. Funding support was provided through the Alberta Children’s Hospital Foundation. The authors would also like to thank Shelly Jelinksi, Connie Mohan, and Laurel Ryan for research support and the Metabolic Clinic (Karin Klassen, Sheryl Jackson, Karen Sabo, Patricia Moar, Deanne Durand) for logistical support. Chris Newell was supported by a grant from MitoCanada. Fariha Ahmed helped with recruitment of healthy controls.

Author information

Authors and Affiliations

  1. Department of Medical Science, Cumming School of Medicine, University of Calgary, Calgary, AB, Canada

    Christopher Newell

  2. Department of Neurosciences, Cumming School of Medicine, University of Calgary, Calgary, AB, Canada

    Barbara Ramage & Ion Robu

  3. Departments of Pediatrics and Community Health Sciences, Cumming School of Medicine, University of Calgary, Calgary, AB, Canada

    Alberto Nettel-Aguirre

  4. Department of Medical Genetics and Pediatrics, Cumming School of Medicine, University of Calgary, Alberta Children’s Hospital, 2888 Shaganappi Trail NW, Calgary, AB, Canada, T3B 6A8

    Aneal Khan

Authors
  1. Christopher Newell
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  2. Barbara Ramage
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  3. Alberto Nettel-Aguirre
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  4. Ion Robu
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  5. Aneal Khan
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Corresponding author

Correspondence to Aneal Khan .

Editor information

Editors and Affiliations

  1. Tulane University Medical School, New Orleans, Louisiana, USA

    Eva Morava (Editor-in-Chief) (Editor-in-Chief)

  2. Division of Metabolism and Children’s Research Centre, University Children’s Hospital Zurich, Zurich, Switzerland

    Matthias Baumgartner

  3. Division of Child and Adolescent Neurology, Mayo Clinic, Rochester, Minnesota, USA

    Marc Patterson

  4. Clinical and Molecular Genetics Unit, UCL Institute of Child Health, London, UK

    Shamima Rahman

  5. Division of Human Genetics, Medical University Innsbruck, Innsbruck, Austria

    Johannes Zschocke

  6. Center for Child and Adolescent Medicine, Heidelberg University Hospital, Heidelberg, Germany

    Verena Peters (Managing Editor) (Managing Editor)

Additional information

Communicated by: Daniela Karall

Electronic Supplementary Material

Below is the link to the electronic supplementary material.

Table S1

Prevalence of neuromuscular patient diagnoses (DOCX 13 kb)

Compliance with Ethics Guidelines

Compliance with Ethics Guidelines

Conflict of Interest

Christopher Newell, Barbara Ramage, Ion Robu, and Aneal Khan declare that they have no conflict of interest.

Informed Consent

All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1975, as revised in 2000 (Enright and Sherrill 1998). Informed consent was obtained from all patients for being included in the study.

Author Contributions

Christopher Newell collected and analyzed data, generated figures, and helped develop the manuscript. Alberto Nettel-Aguirre assisted in the development of the primary study design, statistical analysis, interpretation of results, and contribution to the manuscript. Barbara Ramage, Ion Robu, and Aneal Khan contributed to the study design, methodologies described, administration of the study on subjects, data analysis, and manuscript content. Aneal Khan made the primary study design, was the principal investigator, and had primary responsibility for final content. All authors read and approved the final manuscript.

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© 2016 SSIEM and Springer-Verlag Berlin Heidelberg

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Newell, C., Ramage, B., Nettel-Aguirre, A., Robu, I., Khan, A. (2016). Peak Jump Power Reflects the Degree of Ambulatory Ability in Patients with Mitochondrial and Other Rare Diseases. In: Morava, E., Baumgartner, M., Patterson, M., Rahman, S., Zschocke, J., Peters, V. (eds) JIMD Reports, Volume 33. JIMD Reports, vol 33. Springer, Berlin, Heidelberg. https://doi.org/10.1007/8904_2016_583

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  • DOI: https://doi.org/10.1007/8904_2016_583

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  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-55011-3

  • Online ISBN: 978-3-662-55012-0

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