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JIMD Reports, Volume 26

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Cover of 'JIMD Reports, Volume 26'

Table of Contents

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    Book Overview
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    Chapter 449 Causes of Death in Adults with Mitochondrial Disease
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    Chapter 455 TMEM165 Deficiency: Postnatal Changes in Glycosylation
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    Chapter 470 Pearson Syndrome: A Retrospective Cohort Study from the Marrow Failure Study Group of A.I.E.O.P. (Associazione Italiana Emato-Oncologia Pediatrica)
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    Chapter 471 News on Clinical Details and Treatment in PGM1-CDG
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    Chapter 473 Bioimpedance Analysis as a Method to Evaluate the Proportion of Fatty and Muscle Tissues in Progressive Myopathy in Pompe Disease
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    Chapter 474 Transaldolase Deficiency: A New Case Expands the Phenotypic Spectrum
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    Chapter 477 Friedreich Ataxia in Classical Galactosaemia
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    Chapter 478 Phenotypic Expansion of Congenital Disorder of Glycosylation Due to SRD5A3 Null Mutation.
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    Chapter 479 Periventricular Calcification, Abnormal Pterins and Dry Thickened Skin: Expanding the Clinical Spectrum of RMND1?
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    Chapter 482 Normal Neurodevelopmental Outcomes in PNPO Deficiency: A Case Series and Literature Review
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    Chapter 484 Screening for Attenuated Forms of Mucopolysaccharidoses in Patients with Osteoarticular Problems of Unknown Etiology.
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    Chapter 485 Mucopolysaccharidosis (MPS) Physical Symptom Score: Development, Reliability, and Validity
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    Chapter 487 Infantile Refsum Disease: Influence of Dietary Treatment on Plasma Phytanic Acid Levels
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    Chapter 488 Safety and Efficacy of Chronic Extended Release Cornstarch Therapy for Glycogen Storage Disease Type I
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    Chapter 500 Energy Expenditure in Chilean Children with Maple Syrup Urine Disease (MSUD)
Attention for Chapter 473: Bioimpedance Analysis as a Method to Evaluate the Proportion of Fatty and Muscle Tissues in Progressive Myopathy in Pompe Disease
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Chapter title
Bioimpedance Analysis as a Method to Evaluate the Proportion of Fatty and Muscle Tissues in Progressive Myopathy in Pompe Disease
Chapter number 473
Book title
JIMD Reports, Volume 26
Published in
JIMD Reports, January 2015
DOI 10.1007/8904_2015_473
Pubmed ID
Book ISBNs
978-3-66-249832-3, 978-3-66-249833-0
Authors

Agnieszka Różdżyńska-Świątkowska, Elżbieta Jurkiewicz, Anna Tylki-Szymańska, Różdżyńska-Świątkowska, Agnieszka, Jurkiewicz, Elżbieta, Tylki-Szymańska, Anna

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.

Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 17 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 17 100%

Demographic breakdown

Readers by professional status Count As %
Researcher 4 24%
Student > Postgraduate 2 12%
Other 1 6%
Student > Master 1 6%
Student > Bachelor 1 6%
Other 2 12%
Unknown 6 35%
Readers by discipline Count As %
Medicine and Dentistry 3 18%
Biochemistry, Genetics and Molecular Biology 2 12%
Philosophy 1 6%
Nursing and Health Professions 1 6%
Linguistics 1 6%
Other 2 12%
Unknown 7 41%