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Table 3 CMR parameters in cases in relation to reference values

From: Clinical manifestations in Egyptian Pompe disease patients: Molecular variability and enzyme replacement therapy (ERT) outcomes

 

LVEF

LVMI

LVEDVI

LVESVI

LVGLS

LVGRS

LVGCS

ECV

LGE

AD (10− 3)

Global T2 time

patients

40.4 ± 5.9

116.2 ± 14.02

118.02 ± 41.2

74.8 ± 30.5

11.4 ± 2.7

12.6 ± 4.02

11.24 ± 3.11

33.5 ± 15.3

8.07 ± 6.07

4.3 ± 2.01

65 ± 4.3

Ref values

( at z scores − 2 - +2) at mean BSA

66 ± 9.2

65.2 ± 1.9

65.8 ± 0.55

27.2 ± 1.35

21.4 ± 0.34

22.8 ± 0.94

23.4 ± 0.83

28.2 ± 0.8

0.7 ± 0.07

9.3 ± 0.5

56.5 ± 1.61

P value

0.008

0.000

0.000

0.000

0.000

0.000

0.000

0.42

0.000

0.001

0.000