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Table 2 The LVMI and serum lyso-Gb3 change after ERT in IVS4ā€‰+ā€‰919Gā€‰>ā€‰A Fabry disease male and female patients

From: Globotriaosylsphingosine (lyso-Gb3) might not be a reliable marker for monitoring the long-term therapeutic outcomes of enzyme replacement therapy for late-onset Fabry patients with the Chinese hotspot mutation (IVS4+919G>A)

Cardiac variant, IVS4ā€‰+ā€‰919Gā€‰>ā€‰A FD patients

Parameters, patient number

Baseline (Meanā€‰Ā±ā€‰SD)

After ERT (Meanā€‰Ā±ā€‰SD)

P value

Male

LVMI (g/H2.7) Nā€‰=ā€‰16

81.1ā€‰Ā±ā€‰32.2

71.0ā€‰Ā±ā€‰25.6

0.016*

Compare between baseline and 1st Lyso-Gb3 (nM) Nā€‰=ā€‰16

8.5ā€‰Ā±ā€‰3.3

6.7ā€‰Ā±ā€‰2.2

0.012*

Compare between baseline and the last Lyso-Gb3 (nM) Nā€‰=ā€‰16

8.5ā€‰Ā±ā€‰3.3

7.4ā€‰Ā±ā€‰2.8

0.163

Female

LVMI (g/H2.7) Nā€‰=ā€‰7

59.4ā€‰Ā±ā€‰12.8

42.0ā€‰Ā±ā€‰10.2

<0.001*

Compare between baseline and 1st Lyso-Gb3 (nM) Nā€‰=ā€‰7

2.6ā€‰Ā±ā€‰1.0

1.2ā€‰Ā±ā€‰0.9

0.018*

Compare between baseline and the last Lyso-Gb3 (nM) Nā€‰=ā€‰7

2.6ā€‰Ā±ā€‰1.0

2.6ā€‰Ā±ā€‰1.6

0.938

  1. *Asterisk represents statistically significant difference between the data of baseline and that after ERT (pā€‰<ā€‰0.05). ERT: enzyme replacement therapy; FD: Fabry disease; LVMI: left ventricular mass index; lyso-Gb3: globotriaosylsphingosine; SD: standard deviation.