Fig. 1.

Mean plasma ceramide levels (a), mean lyso-sphingomyelin levels in dried blood spots (DBS) (b), and mean serum chitotriosidase activity (c) at Baseline (BL) and during treatment (30 months) with olipudase alfa. Normal range for plasma ceramide was 1.8–6.5 mg/L. The upper limit of normal for lyso-sphingomyelin in dried blood spots was <69 μg/L, and normal chitotriosidase serum levels were ≤181 nmol/h/mL (note: activity was not corrected for 2 patients heterozygous for a chitotriosidase null mutation)