Pulmonary involvement in Fabry disease

Détails

ID Serval
serval:BIB_2F20A5A9912F
Type
Partie de livre
Sous-type
Chapitre: chapitre ou section
Collection
Publications
Institution
Titre
Pulmonary involvement in Fabry disease
Titre du livre
Fabry Disease: Perspectives from 5 Years of FOS
Auteur⸱e⸱s
Aubert J.D., Barbey F.
Editeur
Oxford PharmaGenesis
Lieu d'édition
Oxford
ISBN
1-903539-03-X
Statut éditorial
Publié
Date de publication
2006
Editeur⸱rice scientifique
Mehta A., Beck M., Sunder-Plassmann G.
Numéro de chapitre
27
Pages
-
Langue
anglais
Résumé
Pulmonary involvement in Fabry disease has received less attention than the effects of the disease on the kidneys, nervous system or heart. However, data from FOS -the Fabry Outcome Survey - are now helping to elucidate the pulmonary manifestations of Fabry disease. Twenty-three patients out of a cohort of 67 analysed in FOS have been identified with airway obstruction, as defined by a ratio of forced expiratory volume in 1 second to forced vital capacity of less than 0.7. This prevalence is much greater than would be expected in the general population, with the main risk factors appearing to be increasing age and male gender. Spirometric analysis has revealed that the airway obstruction is clinically much more similar to chronic obstructive pulmonary disease than to asthma. Although little is known about the anatomical changes responsible for airway obstruction in patients with Fabry disease, airway wall hyperplasia and/or fibrosis are potential causes. Treatment of patients with moderate or severe airway obstruction should include inhaled bronchodilators, and individuals who smoke should be encouraged to stop. Further studies and future analyses of FOS data should determine whether enzyme replacement therapy is able to help or prevent the pulmonary manifestations of Fabry disease.
Pubmed
Création de la notice
04/09/2011 15:53
Dernière modification de la notice
20/08/2019 14:13
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