Mohamed El Sayed

171 Plasma globotriaosylsphingosine and the natural Fabry disease course Table 2: Summary of the performed statistical analyses for every included disease manifestation of FD FD disease manifestation Included variables In analyses Estimate Of effect Confidence Interval (95%) P value Min Max eGFR (mL/min/1.73m2) - Including CMFD* Log10(LysoGb3) 3.64 -6.95 14.1 0.494 Age (years) -1.08 -1.35 -0.83 <0.001 Log10(LysoGb3)*Age -0.26 -0.511 -0.01 0.037 - Excluding CMFD* Log10(LysoGb3) 18.6 -1.78 39.1 0.074 Age (years) -0.85 -1.17 -0.53 >0.001 Log10(LysoGb3)*Age -0.58 -0.98 -0.18 0.005 Log10(uACR*) - Including CMFD* Log10(LysoGb3) -0.17 -0.42 0.08 0.190 Age (years) -0.001 -0.008 0.006 0.852 Log10(LysoGb3)*Age 0.013 0.006 0.019 <0.001 - Excluding CMFD* Log10(LysoGb3) 0.82 0.204 1.433 0.009 Age (years) 0.02 0.006 0.026 0.002 Log10(LysoGb3)*Age -0.01 -0.023 0.002 0.087 LVMi (g/m2) - Including CMFD* Log10(LysoGb3) -2.839 -16.23 10.39 0.674 Age (years) 0.471 0.077 0.858 0.017 Log10(LysoGb3)*Age 1.022 0.682 1.366 <0.001 Any CV risk factor 11.09 3.760 18.50 0.003 - Excluding CMFD* Log10(LysoGb3) -23.61 -57.53 10.11 0.170 Age (years) 0.075 -0.500 0.643 0.794 Log10(LysoGb3)*Age 1.582 0.893 2.276 <0.001 Any CV risk factor 12.20 3.834 20.68 0.005 RWT - Including CMFD* Log10(LysoGb3) 0.079 0.037 0.120 <0.001 Age (years) 0.006 0.005 0.007 <0.001 Sex (male) -0.053 -0.102 -0004 0.035 - Excluding CMFD* Log10(LysoGb3) 0.102 0.040 0.163 0.001 Age (years) 0.006 0.005 0.007 <0.001 Sex (male) -0.045 -0.102 0.011 0.115 e’ (cm/s) - Excluding CMFD* Log10(LysoGb3) -2.780 -0.209 -0.171 <0.001 Age (years) -0.190 -3.717 -1.844 0.001 E/e’ - Excluding CMFD* Log10(LysoGb3) 0.176 1.269 5.160 0.001 Age (years) 3.212 0.138 0.214 <0.001 5

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