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Table 5 Baseline and Month 12 CAJIS Scores of the NHS Cohort, by ECG category. Mean CAJIS scores of baseline data and 12 month follow up data based on ECG classification. * represent significant differences from CAJIS scores of the Normal ECG subgroup (p < 0.05). Scores were obtained by assigning each joint/region as: 0 = uninvolved; 1 = partially involved; 2 = completely ankylosed. Total (cumulative) scores ranged from 0 to 30, with higher scores indicating more severe limitations in mobility and function

From: Patients with ACVR1R206H mutations have an increased prevalence of cardiac conduction abnormalities on electrocardiogram in a natural history study of Fibrodysplasia Ossificans Progressiva

 

Baseline Data

12 Month Follow Up Data

 

Normal ECG (N = 44)

Abnormal ECG (N = 61)

Conduction Abnormality (N = 47)

Normal ECG (N = 42)

Abnormal ECG (N = 54)

Conduction Abnormality (N = 41)

Total

12.15 ± 5.52

11.92 ± 6.52

11.21 ± 6.52

13.55 ± 7.41

11.65 ± 7.05

9.93 ± 6.17*

Cervical Spine

1.43 ± 0.65

1.51 ± 0.56

1.38 ± 0.57

1.59 ± 0.58

1.37 ± 0.59

1.27 ± 0.59*

Jaw

0.77 ± 0.88

0.70 ± 0.84

0.53 ± 0.74

0.86 ± 0.91

0.69 ± 0.81

0.56 ± 0.70

Thoraco-Lumbar Spine

1.66 ± 0.56

1.46 ± 0.64

1.36 ± 0.67*

1.71 ± 0.50

1.44 ± 0.71

1.29 ± 0.74*

Left Ankle

0.55 ± 0.69

0.52 ± 0.69

0.43 ± 0.64

0.67 ± 0.68

0.56 ± 0.68

0.49 ± 0.67

Left Elbow

0.75 ± 0.80

0.70 ± 0.73

0.64 ± 0.70

0.71 ± 0.80

0.67 ± 0.72

0.49 ± 0.59

Left Hip

0.86 ± 0.79

0.87 ± 0.80

0.73 ± 0.74

1.17 ± 0.75

0.78 ± 0.79*

0.63 ± 0.69*

Left Knee

0.41 ± 0.61

0.49 ± 0.76

0.34 ± 0.63

0.50 ± 0.73

0.56 ± 0.74

0.49 ± 0.74

Left Shoulder

1.20 ± 0.69

1.23 ± 0.69

1.11 ± 0.66

1.38 ± 0.65

1.13 ± 0.75

0.95 ± 0.73*

Left Wrist

0.27 ± 0.54

0.34 ± 0.54

0.28 ± 0.49

0.21 ± 0.46

0.33 ± 0.51

0.29 ± 0.45

Right Ankle

0.55 ± 0.72

0.56 ± 0.83

0.40 ± 0.61

0.62 ± 0.79

0.57 ± 0.71

0.54 ± 0.70

Right Elbow

0.80 ± 0.76

0.67 ± 0.80

0.57 ± 0.74

0.81 ± 0.88

0.65 ± 0.77

0.46 ± 0.67

Right Hip

0.86 ± 0.89

0.89 ± 0.77

0.72 ± 0.71

1.02 ± 0.86

0.89 ± 0.81

0.73 ± 0.73

Right Knee

0.590 ± 0.81

0.43 ± 0.73

0.28 ± 0.57

0.52 ± 0.76

0.54 ± 0.69

0.46 ± 0.67

Right Shoulder

1.23 ± 0.70

1.26 ± 0.72

1.11 ± 0.72

1.40 ± 0.69

1.20 ± 0.70

1.05 ± 0.70*

Right Wrist

0.23 ± 0.47

0.28 ± 0.52

0.21 ± 0.46

0.36 ± 0.65

0.28 ± 0.49

0.22 ± 0.47