Extracellular matrix turnover and disease severity in Anderson–Fabry disease

  title={Extracellular matrix turnover and disease severity in Anderson–Fabry disease},
  author={Jaymin S Shah and Derralynn Hughes and Mazahir H Tayebjee and Robert J Macfadyen and Atul B. Mehta and Perry M Elliott},
  journal={Journal of Inherited Metabolic Disease},
Background: Anderson–Fabry Disease (AFD) is an inherited metabolic disease associated with premature death secondary to cardiovascular and renal disease. Patients with AFD develop progressive left ventricular (LV) remodelling and heart failure. We hypothesized that altered extracellular matrix (ECM) turnover contributes to the pathophysiology of cardiac disease in AFD. Methods and Results: Twenty-nine consecutive patients (44.1 ± 11.7 years, 15 male) with AFD and 21 normal controls (39.7 ± 11.3… CONTINUE READING