Fabry Disease, an Under-Recognized Multisystemic Disorder: Expert Recommendations for Diagnosis, Management, and Enzyme Replacement Therapy

Figure 1. . Electron µgraph showing the vascular endothelium of a small vessel from a patient with Fabry disease. Note the electron-dense vesicles (lysosomes) in the endothelium containing undegraded glycosphingolipid. The progressive lysosomal accumulation in the vascular endothelium leads to ischemia and infarction of these vessels. . Electrocardiogram of a 41-year-old man with classic Fabry disease showing sinus bradycardia with short PR interval (88 msec) and left ventricular hypertrophy with QRS widening and a repolarization abnormality.
Figure 1. . Electron µgraph showing the vascular endothelium of a small vessel from a patient with Fabry disease. Note the electron-dense vesicles (lysosomes) in the endothelium containing undegraded glycosphingolipid. The progressive lysosomal accumulation in the vascular endothelium leads to ischemia and infarction of these vessels. . Electrocardiogram of a 41-year-old man with classic Fabry disease showing sinus bradycardia with short PR interval (88 msec) and left ventricular hypertrophy with QRS widening and a repolarization abnormality. Distinctive laboratory findings in Fabry disease. AB

This Article

  1. Ann Intern Med February 18, 2003 vol. 138 no. 4 338-346
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