**5.2 Pediatric Fabry patients**

In the pediatric population, renal damage is typically subclinical and identifiable only through biopsy. In young patients with Fabry disease, timely initiation of ERT is important because some early pathological changes are potentially

### *Enzyme-Replacement Therapy in Fabry Disease DOI: http://dx.doi.org/10.5772/intechopen.103799*

reversible by ERT [42, 43]. As discussed above, the 10-year study of the effectiveness of agalsidase beta in patients with classic Fabry disease has demonstrated that adults who initiated treatment at a younger age and with less renal involvement benefited more from therapy [17, 42]. Therefore, early diagnosis of patients with Fabry disease is vital for appropriate management and monitoring. In our practice, we follow the consensus guideline developed by specialists from the United States with expertise in Fabry disease about the management and treatment of children with Fabry disease [42], which has considerable overlap but also some differences compared to the recommendations by the European Fabry Working Group [44].

