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Pulmonary Involvement in Patients With Fabry Disease

University of Zurich (UZH) logo

University of Zurich (UZH)

Status

Completed

Conditions

Fabry Disease

Treatments

Other: Lung function measurement

Study type

Observational

Funder types

Other

Identifiers

Details and patient eligibility

About

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.

Full 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).

Enrollment

110 patients

Sex

All

Ages

16+ years old

Volunteers

No Healthy Volunteers

Inclusion criteria

  • All patients with established diagnosis of Fabry disease

Exclusion criteria

  • Missing informed consent

Trial contacts and locations

1

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Data sourced from clinicaltrials.gov

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