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Table 4 Scoliosis severity with respect to non-genetic factors, including clinical phenotypes and treatment history

From: Scoliosis in osteogenesis imperfecta: identifying the genetic and non-genetic factors affecting severity and progression from longitudinal data of 290 patients

Non-genetic factors

Scoliosis severity

Row summary

p value

Non-scoliotic

Mild

Moderate

Severe

No. of patients

85

106

40

59

n = 290

 

Age of triradiate cartilage closure

Still open

67

80

17

15

n = 179

 

Closed

10

18

14

19

n = 61

 

 Closed at 11 years

0

1

0

0

n = 1

 

 Closed at 12 years

1

0

1

2

n = 4

 

 Closed at 13 years

4

5

3

2

n = 14

 

 Closed at 14 years

2

5

4

1

n = 12

 

 Closed at 15 years

2

2

4

7

n = 15

 

 Closed at 16 years

0

3

1

3

n = 7

 

 Closed at 17 years

0

2

1

3

n = 6

 

 Closed at 18 years

1

0

0

1

n = 2

 

Closed and overaged (> 18 years)

8

8

9

25

n = 50

 

Risser sign skeletal maturity age (based on last radiograph follow-up)

< 0.001

Grade = 0

68

76

14

11

n = 169

 

Grade = 1–2

4

6

4

6

n = 20

 

Grade = 3–4

6

11

6

11

n = 34

 

Grade = 5

11

15

15

36

n = 77

 

Lower-limb discrepancy (LLD)

No. of patients with LLD

28

54

18

40

n = 140

< 0.001

(%with LLD)

(32.9%)

(50.9%)

(45.0%)

(67.8%)

(48.3%)

 

BMD Z-scores on last follow-ups

Average BMD Z-scoresb

− 1.8 ± 2.0

− 1.8 ± 2.2

− 2.6 ± 2.1

− 3.5 ± 1.9

− 2.2 ± 2.2

< 0.001

Surgical interventions of the spine

Never treateda

85

106

40

34

265 (91.4%)

 

SZH

0

0

0

22

22 (7.6%)

 

Extramural

0

0

0

3

3 (1.0%)

 

BMD improvement drugs used

0.011

Zoledronate

59

68

22

21

n = 170

 

Pamidronate

20

29

11

24

n = 84

 

Ibandronate

0

1

1

0

n = 2

 

Alendronate

0

1

0

0

n = 1

 

Denosumab

0

0

0

1

n = 1

 

Never treatedc

6

7

6

13

n = 32

 

Average age of first drug used

6 ± 7.6

6.3 ± 5.1

8.1 ± 4.8

10.3 ± 6.0

7.2 ± 6.2

 
  1. aNever treated with spine surgeries
  2. bBased on 266 patients who had at least one BMD examination
  3. cNever treated with bisphosphonates or monoclonal antibodies
  4. dExcluding the 32 patients who never received drug treatment. Plus–minus values are means ± SD