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Quantification of Diaphragm Mechanics in Pompe Disease Using Dynamic 3D MRI

BACKGROUND: Diaphragm weakness is the main reason for respiratory dysfunction in patients with Pompe disease, a progressive metabolic myopathy affecting respiratory and limb-girdle muscles. Since respiratory failure is the major cause of death among adult patients, early identification of respirator...

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Autores principales: Mogalle, Katja, Perez-Rovira, Adria, Ciet, Pierluigi, Wens, Stephan C. A., van Doorn, Pieter A., Tiddens, Harm A. W. M., van der Ploeg, Ans T., de Bruijne, Marleen
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4938606/
https://www.ncbi.nlm.nih.gov/pubmed/27391236
http://dx.doi.org/10.1371/journal.pone.0158912
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author Mogalle, Katja
Perez-Rovira, Adria
Ciet, Pierluigi
Wens, Stephan C. A.
van Doorn, Pieter A.
Tiddens, Harm A. W. M.
van der Ploeg, Ans T.
de Bruijne, Marleen
author_facet Mogalle, Katja
Perez-Rovira, Adria
Ciet, Pierluigi
Wens, Stephan C. A.
van Doorn, Pieter A.
Tiddens, Harm A. W. M.
van der Ploeg, Ans T.
de Bruijne, Marleen
author_sort Mogalle, Katja
collection PubMed
description BACKGROUND: Diaphragm weakness is the main reason for respiratory dysfunction in patients with Pompe disease, a progressive metabolic myopathy affecting respiratory and limb-girdle muscles. Since respiratory failure is the major cause of death among adult patients, early identification of respiratory muscle involvement is necessary to initiate treatment in time and possibly prevent irreversible damage. In this paper we investigate the suitability of dynamic MR imaging in combination with state-of-the-art image analysis methods to assess respiratory muscle weakness. METHODS: The proposed methodology relies on image registration and lung surface extraction to quantify lung kinematics during breathing. This allows for the extraction of geometry and motion features of the lung that characterize the independent contribution of the diaphragm and the thoracic muscles to the respiratory cycle. RESULTS: Results in 16 3D+t MRI scans (10 Pompe patients and 6 controls) of a slow expiratory maneuver show that kinematic analysis from dynamic 3D images reveals important additional information about diaphragm mechanics and respiratory muscle involvement when compared to conventional pulmonary function tests. Pompe patients with severely reduced pulmonary function showed severe diaphragm weakness presented by minimal motion of the diaphragm. In patients with moderately reduced pulmonary function, cranial displacement of posterior diaphragm parts was reduced and the diaphragm dome was oriented more horizontally at full inspiration compared to healthy controls. CONCLUSION: Dynamic 3D MRI provides data for analyzing the contribution of both diaphragm and thoracic muscles independently. The proposed image analysis method has the potential to detect less severe diaphragm weakness and could thus be used to determine the optimal start of treatment in adult patients with Pompe disease in prospect of increased treatment response.
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spelling pubmed-49386062016-07-22 Quantification of Diaphragm Mechanics in Pompe Disease Using Dynamic 3D MRI Mogalle, Katja Perez-Rovira, Adria Ciet, Pierluigi Wens, Stephan C. A. van Doorn, Pieter A. Tiddens, Harm A. W. M. van der Ploeg, Ans T. de Bruijne, Marleen PLoS One Research Article BACKGROUND: Diaphragm weakness is the main reason for respiratory dysfunction in patients with Pompe disease, a progressive metabolic myopathy affecting respiratory and limb-girdle muscles. Since respiratory failure is the major cause of death among adult patients, early identification of respiratory muscle involvement is necessary to initiate treatment in time and possibly prevent irreversible damage. In this paper we investigate the suitability of dynamic MR imaging in combination with state-of-the-art image analysis methods to assess respiratory muscle weakness. METHODS: The proposed methodology relies on image registration and lung surface extraction to quantify lung kinematics during breathing. This allows for the extraction of geometry and motion features of the lung that characterize the independent contribution of the diaphragm and the thoracic muscles to the respiratory cycle. RESULTS: Results in 16 3D+t MRI scans (10 Pompe patients and 6 controls) of a slow expiratory maneuver show that kinematic analysis from dynamic 3D images reveals important additional information about diaphragm mechanics and respiratory muscle involvement when compared to conventional pulmonary function tests. Pompe patients with severely reduced pulmonary function showed severe diaphragm weakness presented by minimal motion of the diaphragm. In patients with moderately reduced pulmonary function, cranial displacement of posterior diaphragm parts was reduced and the diaphragm dome was oriented more horizontally at full inspiration compared to healthy controls. CONCLUSION: Dynamic 3D MRI provides data for analyzing the contribution of both diaphragm and thoracic muscles independently. The proposed image analysis method has the potential to detect less severe diaphragm weakness and could thus be used to determine the optimal start of treatment in adult patients with Pompe disease in prospect of increased treatment response. Public Library of Science 2016-07-08 /pmc/articles/PMC4938606/ /pubmed/27391236 http://dx.doi.org/10.1371/journal.pone.0158912 Text en © 2016 Mogalle et al http://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/) , which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Research Article
Mogalle, Katja
Perez-Rovira, Adria
Ciet, Pierluigi
Wens, Stephan C. A.
van Doorn, Pieter A.
Tiddens, Harm A. W. M.
van der Ploeg, Ans T.
de Bruijne, Marleen
Quantification of Diaphragm Mechanics in Pompe Disease Using Dynamic 3D MRI
title Quantification of Diaphragm Mechanics in Pompe Disease Using Dynamic 3D MRI
title_full Quantification of Diaphragm Mechanics in Pompe Disease Using Dynamic 3D MRI
title_fullStr Quantification of Diaphragm Mechanics in Pompe Disease Using Dynamic 3D MRI
title_full_unstemmed Quantification of Diaphragm Mechanics in Pompe Disease Using Dynamic 3D MRI
title_short Quantification of Diaphragm Mechanics in Pompe Disease Using Dynamic 3D MRI
title_sort quantification of diaphragm mechanics in pompe disease using dynamic 3d mri
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4938606/
https://www.ncbi.nlm.nih.gov/pubmed/27391236
http://dx.doi.org/10.1371/journal.pone.0158912
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