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Long-term follow-up of pulmonary function in Fabry disease: A bi-center observational study

INTRODUCTION: Fabry disease (FD) is a lysosomal storage disorder leading to decreased α-galactosidase A enzyme activity and subsequent abnormal accumulation of glycosphingolipids in various organs. Although histological evidence of lung involvement has been demonstrated, the functional impact of the...

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Detalles Bibliográficos
Autores principales: Franzen, Daniel P., Nowak, Albina, Haile, Sarah R., Mottet, Dominique, Bonani, Marco, Dormond, Olivier, Kohler, Malcolm, Krayenbuehl, Pierre A., Barbey, Frederic
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5526574/
https://www.ncbi.nlm.nih.gov/pubmed/28742806
http://dx.doi.org/10.1371/journal.pone.0180437