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. Author manuscript; available in PMC: 2024 Feb 1.
Published in final edited form as: Mol Genet Metab. 2022 Nov 9:106963. doi: 10.1016/j.ymgme.2022.11.002

Table 2.

GL-3 scores in SSCE, assessed by light microscopy, at baseline and at Weeks 12, 26, 52, and 156, and fractions of cytoplasm volume occupied by GL-3 inclusions, as assessed by electron microscopy, at baseline and at Weeks 12, 26, and 156.

Patient #b GL-3 scores in SSCE (light microscopy)a, and fractions of cytoplasm volume of SSCE occupied by GL-3 inclusions (electron microscopy, italic)
Baseline Week 12 Week 26 Week 52 (optional) Week 156

1 2 1 1 1 1
0.296 0.331 0.249 - 0.144

2 1 1 1 1 NS
0.254 0.287 0.254 - -

3 2 1 1 2 1
0.329 0.374 0.276 - 0.210

4 1 1 1 NS 0
0.304 0.303 - 0.171

5 1 NS NS NS NS
-

6 2 1 1 NS NS
0.271 0.221 0.221

7 1 1 1 NS NS
0.307 0.335 0.210

8 1 2 2 1 0
0.296 0.234 0.200 - 0.200

9 1 1 1 1 1
0.257 0.218 0.170 - 0.205

10 2 2 2 1 1
0.329 0.297 0.262 - 0.168

11 1 NS NS NS NS
-
a

GL-3 inclusions scoring: 0=no/trace; 1=mild; 2=moderate.

b

Patient numbers randomly assigned.

GL-3, globotriaosylceramide; NS, no sample; SSCE, superficial skin capillary endothelium.