Enzyme replacement therapy decreases forearm and hand compliance in Fabry disease

Background: In Fabry patients, limb perfusion is compromised due to glycosphingolipid deposition in various tissues including vessel walls and vasomotor nerve fibers (Stemper & Hilz. J. Neurol., 2003). Replacement of the deficient enzyme alpha-galactosidase A improves organ function and should therefore also improve limb perfusion and compliance. Objective: To evaluate whether enzyme replacement therapy (ERT) positively influences forearm and hand compliance. Methods: In 22 Fabry patients (29±8 years) we assessed forearm and hand compliance using strain gauge plethysmography. Before and after 23 months of biweek…