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Table 5 Baseline and follow-up echocardiographic parameters of seven Taiwanese patients with MPS IVA who received ERT for 3-6 years

From: Cardiac features and effects of enzyme replacement therapy in Taiwanese patients with Mucopolysaccharidosis IVA

No. Age at start of ERT (years) ERT duration (years) LVMI (z score) IVSd (z score) LVPWd (z score)
Baseline Follow-up Baseline Follow-up Baseline Follow-up
1 1.4 6.0 0.44 -1.22 1.8 0.5 1.1 -0.5
2 2.8 3.0 0.46 -1.24 2.0 1.0 0.2 -2.2
6 7.3 4.0 2.47 2.64 1.2 2.2 -0.1 -0.2
11 13.9 3.0 -1.46 0.05 5.2 3.1 0.9 -0.5
20 14.9 4.1 1.09 1.47 4.3 1.9 0.4 0.0
23 16.8 4.1 -1.41 0.25 -0.1 0.5 -1.9 0.1
31 25.8 4.4 3.8 3.17 2.4 6.0 2.3 -0.2
Mean 11.8 4.1 0.77 0.73 2.42 2.17 0.41 -0.50
SD 8.6 1.0 1.92 1.76 1.81 1.94 1.29 0.78
  1. MPS mucopolysaccharidosis, ERT enzyme replacement therapy, LVMI left ventricular mass index, IVSd interventricular septum thickness in diastole, LVPWd left ventricular posterior wall thickness in diastole, SD standard deviation