Muscle MRI of classic infantile pompe patients: Fatty substitution and edema-like changes.

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Abstract

The aim of this study was to evaluate the muscle MRI pattern of 9 patients (median age: 6.5 ± 2.74 years) affected by classic infantile-onset Pompe disease who were treated with enzyme replacement therapy.

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Journal

Journal ID (iso-abbrev): Muscle Nerve

Title: Muscle & nerve

Publisher: Wiley-Blackwell

ISSN (Electronic): 1097-4598

ISSN (Print): 0148-639X

Publication date (Electronic): Jun 2017

Volume: 55

Issue: 6

Affiliations

[1 ] Neuroradiology Department, C. Mondino National Neurological Institute, Via Mondino, 2 - 27100, Pavia, Italy.

[2 ] Child Neuropsychiatry Unit, Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy.

[3 ] Unit of Neuroradiology, Department of Neuroscience, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, University of Milan, Milan, Italy.

[4 ] Neuroradiology Unit, Bambino Gesù Children's Hospital, Rome, Italy.

[5 ] Neurology Unit, Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy.

[6 ] Unit of Rare Metabolic Diseases, San Gerardo Hospital, Monza, Italy.

[7 ] Pediatric Highly Intensive Care Unit, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy.

[8 ] Unit of Metabolic Diseases, Azienda Ospedaliera Universitaria, Padua, Italy.

[9 ] Metabolic and Neuromuscular Unit, AOU Meyer Hospital, Florence, Italy.

[10 ] Department of Translational Medicine-Section of Pediatrics, Federico II University, Naples, Italy.

[11 ] Unit of Metabolism, Bambino Gesù Children's Hospital, Rome, Italy.

[12 ] Unit of Rare Diseases, Department of Pediatrics, Giannina Gaslini Institute, Genoa, Italy.

Article

DOI: 10.1002/mus.25417

PubMed ID: 27668838

SO-VID: d954953c-b372-4065-8b64-b9a0692d088f

History

Keywords: Pompe disease,STIR,T2,glycogenosis II,infantile-onset Pompe disease,muscle MRI

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Keywords: Pompe disease, STIR, T2, glycogenosis II, infantile-onset Pompe disease, muscle MRI

Muscle MRI of classic infantile pompe patients: Fatty substitution and edema-like changes.

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