Cardiac Imaging in Anderson-Fabry Disease: Past, Present and Future

Anderson-Fabrydisease is an X-linked lysosomal storage disorder caused by a deficiency in the lysosomal enzyme α-galactosidase A. This results in pathological accumulation of glycosphingolipids in several tissues and multi-organ progressive dysfunction. The typical clinical phenotype of Anderson-Fab...

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Autores principales: Esposito, Roberta, Santoro, Ciro, Mandoli, Giulia Elena, Cuomo, Vittoria, Sorrentino, Regina, La Mura, Lucia, Pastore, Maria Concetta, Bandera, Francesco, D’Ascenzi, Flavio, Malagoli, Alessandro, Benfari, Giovanni, D’Andrea, Antonello, Cameli, Matteo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Review
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8124634/
https://www.ncbi.nlm.nih.gov/pubmed/34066467
http://dx.doi.org/10.3390/jcm10091994
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author Esposito, Roberta
Santoro, Ciro
Mandoli, Giulia Elena
Cuomo, Vittoria
Sorrentino, Regina
La Mura, Lucia
Pastore, Maria Concetta
Bandera, Francesco
D’Ascenzi, Flavio
Malagoli, Alessandro
Benfari, Giovanni
D’Andrea, Antonello
Cameli, Matteo
author_facet Esposito, Roberta
Santoro, Ciro
Mandoli, Giulia Elena
Cuomo, Vittoria
Sorrentino, Regina
La Mura, Lucia
Pastore, Maria Concetta
Bandera, Francesco
D’Ascenzi, Flavio
Malagoli, Alessandro
Benfari, Giovanni
D’Andrea, Antonello
Cameli, Matteo
author_sort Esposito, Roberta
collection PubMed
description Anderson-Fabrydisease is an X-linked lysosomal storage disorder caused by a deficiency in the lysosomal enzyme α-galactosidase A. This results in pathological accumulation of glycosphingolipids in several tissues and multi-organ progressive dysfunction. The typical clinical phenotype of Anderson-Fabry cardiomyopathy is progressive hypertrophic cardiomyopathy associated with rhythm and conduction disturbances. Cardiac imaging plays a key role in the evaluation and management of Anderson-Fabry disease patients. The present review highlights the value and perspectives of standard and advanced cardiovascular imaging in Anderson-Fabry disease.
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spelling pubmed-81246342021-05-17 Cardiac Imaging in Anderson-Fabry Disease: Past, Present and Future Esposito, Roberta Santoro, Ciro Mandoli, Giulia Elena Cuomo, Vittoria Sorrentino, Regina La Mura, Lucia Pastore, Maria Concetta Bandera, Francesco D’Ascenzi, Flavio Malagoli, Alessandro Benfari, Giovanni D’Andrea, Antonello Cameli, Matteo J Clin Med Review Anderson-Fabrydisease is an X-linked lysosomal storage disorder caused by a deficiency in the lysosomal enzyme α-galactosidase A. This results in pathological accumulation of glycosphingolipids in several tissues and multi-organ progressive dysfunction. The typical clinical phenotype of Anderson-Fabry cardiomyopathy is progressive hypertrophic cardiomyopathy associated with rhythm and conduction disturbances. Cardiac imaging plays a key role in the evaluation and management of Anderson-Fabry disease patients. The present review highlights the value and perspectives of standard and advanced cardiovascular imaging in Anderson-Fabry disease. MDPI 2021-05-06 /pmc/articles/PMC8124634/ /pubmed/34066467 http://dx.doi.org/10.3390/jcm10091994 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Esposito, Roberta
Santoro, Ciro
Mandoli, Giulia Elena
Cuomo, Vittoria
Sorrentino, Regina
La Mura, Lucia
Pastore, Maria Concetta
Bandera, Francesco
D’Ascenzi, Flavio
Malagoli, Alessandro
Benfari, Giovanni
D’Andrea, Antonello
Cameli, Matteo
Cardiac Imaging in Anderson-Fabry Disease: Past, Present and Future
title Cardiac Imaging in Anderson-Fabry Disease: Past, Present and Future
title_full Cardiac Imaging in Anderson-Fabry Disease: Past, Present and Future
title_fullStr Cardiac Imaging in Anderson-Fabry Disease: Past, Present and Future
title_full_unstemmed Cardiac Imaging in Anderson-Fabry Disease: Past, Present and Future
title_short Cardiac Imaging in Anderson-Fabry Disease: Past, Present and Future
title_sort cardiac imaging in anderson-fabry disease: past, present and future
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8124634/
https://www.ncbi.nlm.nih.gov/pubmed/34066467
http://dx.doi.org/10.3390/jcm10091994
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