Fabry Disease
Conditions
Brief summary
The objective of this study is to investigate whether Agalsidase alpha, a drug commonly prescribed in patients with Fabry disease, is associated with improvement of the pulmonary involvement. According to the Global Initiative for Obstructive Lung Disease (GOLD), the surrogate markers for obstructive lung diseases are a decrease in both forced expiratory volume in one second (FEV1) and FEV1/FVC ratio, whereas FVC is the forced vital capacity. However, the measurement of these lung function parameters is indicated as yearly follow-up examinations with or without the treatment of Agalsidase alpha in patients with Fabry disease.
Detailed description
Pulmonary function tests und DLCO measurements are performed yearly in relation with yearly follow up examinations in the Department of Internal Medicine from the University Hospital of Zurich. We will retrospectively collect the results of the pulmonary functions test (spirometry).
Interventions
Spirometry
Sponsors
University of Zurich
Study design
Observational model
COHORT
Time perspective
RETROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
16 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* All patients with established diagnosis of Fabry disease
Exclusion criteria
* Missing informed consent
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Lung function measurements | From first consultation until present | Retrospective analysis of yearly measured lung function parameters to investigate on the lung function changes in patients with Fabry disease |
Countries
Switzerland
Outcome results
None listed