Skip to main content
SpringerLink
Log in
Book cover

JIMD Reports, Volume 26 pp 45–51Cite as

Bioimpedance Analysis as a Method to Evaluate the Proportion of Fatty and Muscle Tissues in Progressive Myopathy in Pompe Disease

  • Research Report
  • Chapter
  • First Online:

Part of the book series: JIMD Reports ((JIMD,volume 26))

Abstract

During progressive myopathy, the space of atrophic muscle tissue is gradually filled by fatty tissue. The proportion of these two tissue types relative to body mass provides an indication of the extent of muscle tissue destruction, i.e., the progression and severity of the disease.

In this study we use Pompe disease as an example to report the new possibility of using bioimpedance analysis (BIA) to assess the relative proportion of fatty and muscle tissue in diseases associated with muscle atrophy, thus enabling the assessment of disease progression and the effectiveness of treatment. Results from BIA analysis were compared with magnetic resonance images.

The results of muscle magnetic resonance images and BIA analysis were similar, which suggests that BIA may provide valuable diagnostic guidance for the assessment of the progression of the disorder.

Competing interests: None declared

This is a preview of subscription content, log in via an institution.

Chapter
USD   29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD   39.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD   54.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Learn about institutional subscriptions

References

  • Alejaldre A, Diaz-Manera J, Ravaglia S et al (2012) Trunk muscle involvement in late-onset Pompe disease: study of thirty patients. Neuromuscul Disord 22:148–154

    Article  Google Scholar 

  • Angelini C, Nascimbeni AC, Semplicini C (2013) Therapeutic advances in the management of Pompe disease and other metabolic myopathies. Ther Adv Neurol Disord 6:311–321

    Article  PubMed  PubMed Central  Google Scholar 

  • Cole TJ, Flegal KM, Nicholls D et al (2007) Body mass index cut off to define thinness in children and adolescents: international survey. BMJ 335:194

    Google Scholar 

  • De Jager AEJ, van der Vliet TM, van der Ree TC et al (1998) Muscle Computed Tomography in adult-onset acid maltase deficiency. Muscle Nerve 21:398–400

    Article  PubMed  Google Scholar 

  • Del Gaizo A, Banerjee S, Terk M (2009) Adult onset glycogen storage disease type II (adult onset Pompe disease): report and magnetic resonance images of two cases. Skeletal Radio 38:1205–1208

    Article  Google Scholar 

  • Fisher D, Walter MC, Kesper K et al (2005) Diagnostic value of muscle MRI in differentiating LGMD2I from other LGMDs. J Neurol 252:538–547

    Article  Google Scholar 

  • Fomon SJ, Haschke F, Ziegler EE (1982) Body composition of reference children from birth to age 10 years. Am J Chin Nutr 35:1169–1175

    CAS  Google Scholar 

  • Gaeta M, Barca E, Ruggeri P et al (2013) Late-onset Pompe disease (LOPD): correlations between respiratory muscles CT and MRI features and pulmonary function. Mol Genet Metab 110:290–296

    Article  CAS  PubMed  Google Scholar 

  • Heymsfield SB, Smith R, Aulet M (1990) Appendicular skeletal muscle mass: measurement by dual-photon absorptiometry. Am J Clin Nutr 52:214–218

    CAS  PubMed  Google Scholar 

  • Heyward VH, Stolarczyk LM (1996) Applied body composition assessment. Human kinetics, Campaign, p 82

    Google Scholar 

  • Ito H, Ohshima A, Ohto N et al (2001) Relation between body composition and age in healthy Japanese subjects. Eur J Clin Nutr 55:462–470

    Article  CAS  PubMed  Google Scholar 

  • Kaido T, Ogawa K, Fujimoto Y (2013) Impact of Sarcopenia on survival in patients undergoing living donor liver transplantation. Am J Transplant 20:1–8

    Google Scholar 

  • Lewitt A, Mądro E, Krupienicz A (2010) Podstawy teoretyczne i zastosowania analizy impedancji bioelektrycznej (BIA). Endokrynologia, Otyłość, Zaburzania Przemiany Materii 3:79–84

    Google Scholar 

  • Lohman TG (1992) Advanced in body composition assessment – current issues in exercise science series. Human Kinetics, Champaign, p 80

    Google Scholar 

  • Mercuri E, Cini C, Counsell S et al (2002a) Muscle MRI findings in a 3 generation family affected by Bethlem myopathy. Eur J Paediatr Neurol 6:309–314

    Article  PubMed  Google Scholar 

  • Mercuri E, Picchiechio A, Consuel S et al (2002b) A short protocol for muscle MRI in children with muscular dystrophies. Eur J Paediatr Neurol 6:305–307

    Article  PubMed  Google Scholar 

  • Nescolarde L, Doñate T, Piccoli A, Rosell J (2008) Comparison of segmental with whole-body impedance measurements in peritoneal dialysis patients. Med Eng Phys 30(7):817–824

    Article  PubMed  Google Scholar 

  • Paradas C, Llauger J, Diaz-Manera J et al (2010) Redefining dysferlinopathy phenotypes based on clinical findings and muscle imaging studies. Neurology 75:316–323

    Article  CAS  PubMed  Google Scholar 

  • Pellegrini N, Laforet P, Orlikowski D, Pellegrini M et al (2005) Respiratory insufficiency and limb muscle weakness in adults with Pompe’s disease. Eur Respir J 26:1024–1031

    Article  CAS  PubMed  Google Scholar 

  • Pichiecchio A, Uggetti C, Ravaglia S et al (2004) Muscle MRI in adult-onset acid maltase deficiency. Neuromuscul Disord 14:51–55

    Article  PubMed  Google Scholar 

  • Rutkove SB, Geisbush TR, Mijailovic A et al (2014) Cross-sectional evaluation of electrical impedance myography and quantitative ultrasound for the assessment of Duchenne muscular dystrophy in a clinical trial setting. Pediatr Neurol 51(1):88–92

    Article  PubMed  PubMed Central  Google Scholar 

  • Salinari S, Bertuzzi A, Mingrone G et al (2002) New bioimpedance model accurately predicts lower limb muscle volume: validation by magnetic resonance imaging. Am J Physiol Endocrinol Metab 282:960–966

    Article  Google Scholar 

  • Tahara Y, Moji K, Aoyagi K et al (2002) Age-related pattern of body density and body composition of Japanese men and women 18-59 years of age. Am J Hum Biol 14:743–752

    Article  CAS  PubMed  Google Scholar 

  • Wattjes MP, Klay RA, Fisher D (2010) Neuromuscular imaging in inherited muscle diseases. Eur Radiol 20:2447–2460

    Article  PubMed  PubMed Central  Google Scholar 

Download references

Acknowledgments

We thank Maniac Gym for lending us an InBody J10 device to perform this study. We are also grateful to the subjects and their families who gave their time and participated in this study.

Author information

Authors and Affiliations

  1. Anthropology Laboratory, The Children’s Memorial Health Institute, Al. Dzieci Polskich 20, 04-730, Warsaw, Poland

    Agnieszka Różdżyńska-Świątkowska

  2. Department of Diagnostic Imaging, The Children’s Memorial Health Institute, Al. Dzieci Polskich 20, 04-730, Warsaw, Poland

    Elżbieta Jurkiewicz

  3. Department of Pediatric, Nutrition and Metabolic Diseases, The Children’s Memorial Health Institute, Al. Dzieci Polskich 20, 04-730, Warsaw, Poland

    Anna Tylki-Szymańska

Authors
  1. Agnieszka Różdżyńska-Świątkowska
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Elżbieta Jurkiewicz
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Anna Tylki-Szymańska
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Anna Tylki-Szymańska .

Editor information

Editors and Affiliations

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

    Eva Morava

  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, United Kingdom

    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, Baden-Württemberg, Germany

    Verena Peters

Additional information

Communicated by: Jörn Oliver Sass

Compliance with Ethics Guidelines

Compliance with Ethics Guidelines

Conflict of Interest

Agnieszka Różdżyńska-Świątkowska, Elżbieta Jurkiewicz, and Anna Tylki-Szymańska declare that they have no conflict of interest. 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 (5). Informed consent was obtained from all patients for being included in the study.

Rights and permissions

Reprints and permissions

Copyright information

© 2015 SSIEM and Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Różdżyńska-Świątkowska, A., Jurkiewicz, E., Tylki-Szymańska, A. (2015). Bioimpedance Analysis as a Method to Evaluate the Proportion of Fatty and Muscle Tissues in Progressive Myopathy in Pompe Disease. In: Morava, E., Baumgartner, M., Patterson, M., Rahman, S., Zschocke, J., Peters, V. (eds) JIMD Reports, Volume 26. JIMD Reports, vol 26. Springer, Berlin, Heidelberg. https://doi.org/10.1007/8904_2015_473

Download citation

  • DOI: https://doi.org/10.1007/8904_2015_473

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-49832-3

  • Online ISBN: 978-3-662-49833-0

  • eBook Packages: Biomedical and Life SciencesBiomedical and Life Sciences (R0)

Publish with us

Policies and ethics

Search

Navigation