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Table 2 Restriction of joint range of motion

From: Musculoskeletal manifestations in mucopolysaccharidosis type I (Hurler syndrome) following hematopoietic stem cell transplantation

No.

Age at evaluation (y)

Follow-up after HSCT (y)

Shoulders

Elbows

Hips

Knees

1

0.7

0

mild

mild

moderate

severe

2.7

0.7

severe

none

mild

moderate

2

1.1

0

severe

none

mild

none

2.5

1.0

severe

mild

mild

mild

3.5

2.0

severe

mild

n.d.

n.d.

3

3.2

1.9

severe

mild

none

mild

4

1.9

0

moderate

mild

none

mild

2.1

0.2

moderate

none

none

none

4.1

1.8

mild

none

none

none

4.5

2.3

severe

none

none

none

5

1.5

0

severe

none

mild

none

3.9

2.1

severe

mild

mild

mild

5.1

3.3

n.d.

mild

n.d.

mild

6.0

4.2

severe

mild

none

none

6

1.3

0

none

none

none

none

3.5

2.0

mild

none

none

none

4.7

3.2

mild

none

n.d.

none

7

1.9

0

severe

mild

moderate

none

3.0

1.0

mild

moderate

moderate

mild

4.0

2.0

mild

none

mild

none

8

1.2

0.3

none

none

moderate

none

4.0

2.5

mild

mild

moderate

mild

5.1

3.6

mild

mild

n.d.

mild

6.0

4.6

moderate

none

none

none

9

3.0

0.8

none

none

none

none

3.9

1.7

mild

none

mild

none

6.4

4.2

none

mild

mild

none

10

2.2

1.0

moderate

mild

none

mild

3.4

2.1

moderate

mild

mild

mild

6.0

4.7

moderate

mild

none

none

11

2.6

1.0

none

none

none

none

5.1

3.5

mild

mild

none

mild

6.6

5.0

severe

mild

none

mild

12

1.8

0

severe

moderate

none

none

4.0

2.1

moderate

moderate

severe

mild

4.6

2.7

moderate

mild

moderate

mild

13

1.0

0

severe

none

none

none

2.5

1.0

n.d.

none

none

mild

9.3

7.8

moderate

none

none

none

14

1.7

0.7

moderate

mild

none

mild

9.6

8.5

severe

mild

moderate

mild

10.4

9.4

severe

severe

severe

mild

12.0

10.9

severe

severe

severe

severe

15

1.7

0.6

severe

moderate

mild

mild

7.0

5.9

severe

severe

severe

mild

10.7

9.6

severe

severe

severe

mild

11.4

10.3

moderate

moderate

severe

mild

16

1.5

0

mild

mild

none

mild

6.9

5.1

mild

mild

none

none

11.8

9.9

mild

mild

none

none

12.4

10.6

mild

mild

none

none

17

3.9

2.1

mild

mild

none

mild

8.1

6.3

mild

mild

n.d.

mild

12.9

11.0

mild

mild

mild

none

15.2

13.3

mild

mild

moderate

none

18

2.7

0.6

n.d.

mild

n.d.

mild

7.2

5.1

mild

mild

mild

mild

8.8

6.7

severe

mild

n.d.

mild

19

2.3

1.0

n.d.

mild

n.d.

mild

7.2

6.0

n.d.

mild

severe

mild

11.4

10.2

mild

none

nonea

none

19.8

18.6

mild

none

nonea

none

23.8

22.5

mild

none

milda

none

  1. Shoulder abduction was classified as mild (deficit ≤40°), moderate (deficit of 50–80°) or severe (deficit ≥90°)
  2. Flexion joint contractures of elbows, hips and knees were classified as mild (5–15°), moderate (≥15°) or severe (≥25°)
  3. y year, n.d.not done
  4. aStatus after hip release surgery