#5718 ASSOCIATION OF MULTIPLE PLASMA BIOMARKER LEVELS WITH KIDNEY DISEASE ACTIVITY IN FABRY DISEASE

Abstract Background and Aims Fabry disease is a rare, systemic, genetic, metabolic lysosomal storage disease. Phenotypic heterogeneity prevents accurate predictions for the progression. We aimed to evaluate association of multiple plasma biomarkers with severity in Method In cross-sectional study, we examined 76 Fabry, 46 CKD patients 41 healthy controls. studied KIM-1, MCP-1, YKL-40, TNFR-1, TNFR-2, cystatin-C enzyme linked immunoassay. on renal replacement therapy were excluded from analysis. Results Demographic, clinical laboratory data study participants are shown Table 1. eGFR was calculated creatinine creatinine-cystatin C using CKD-EPI formula. While there no difference between controls regarding eGFR(cr); eGFR(cr-cys) significantly lower patients. Biomarker levels three groups 2. After performing age, sex BMI adjusted analysis, MCP-1 compared group (p:0.01). higher cardiac involvement than those without (p:0.039). KIM-1 comparing There significant biomarker kidney involvement. YKL-40 both control (p:0.025), probably reflecting effect ERT. Conclusion Cystatin seems be useful markers management Each associated different aspect might activity especially an early sign Cystatin-C should used better since it shows creatinine.