165 Plasma globotriaosylsphingosine and the natural Fabry disease course associated with a faster increase in albuminuria (n=198, median 2 measurements per patient, range 1-16 ) (fig 3b, p<0.001), though this association seemed to be driven largely by male patients with a classical phenotype as the association was no longer statistically significant after exclusion of this group (p=0.09). Adding sex of the patient or the presence of cardiovascular risk factors to either of the models did not significantly impact the outcome and was thus not included in the final analyses. For full results see table 2. Figure 3: Relation between lysoGb3 and the progression of renal manifestations in untreated Fabry patients. All analyses are performed with actual plasma lysoGb3 levels (after log10 transformation). Grouping is done for visualization purposes only. (a) Association between plasma lysoGb3 and eGFR slope in untreated patients, thepink reference area visualizes the approximate 95th percentile eGFR range in healthy subjects, extrapolated from Baba et all [21]. (b) Association between plasma lysoGb3 levels and progression of albuminuria (uACR) in untreated Fabry patients. 5
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