Pulmonary involvement in Fabry disease: effect of plasma globotriaosylsphingosine and time to initiation of enzyme replacement therapy.

Daniel FranzenSarah R HaileDavid C KasperThomas P MechtlerAndreas J FlammerPierre A KrayenbühlAlbina Nowak

Published in: BMJ open respiratory research (2018)

Early ERT initiation seems to preserve pulmonary function. Plasma Lyso-Gb3 is maybe a useful predictor for airflow limitation. Classic men need a closer monitoring of the lung function.

Keyphrases

replacement therapy
lung function
smoking cessation
cystic fibrosis
chronic obstructive pulmonary disease
air pollution
pulmonary hypertension
middle aged
heart failure