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Table 4 ERT related immune response

From: Sleep disordered breathing in mucopolysaccharidosis I: a multivariate analysis of patient, therapeutic and metabolic correlators modifying long term clinical outcome

Patient no.

Phenotype

Age at start of ERT (Yrs)

Age at assessment of antibody status

Antibody titre

Cellular uptake inhibition %

Inhibitory antibodies beyond 1 year

1

Hurler

11

13*

262336

86.1

Yes*

2

Hurler

7

7

65536

41.8

No

3

Hurler

6

7

262144

88.3

Yes

4

Hurler-Scheie

5

7*

262336

0

No

5

Hurler-Scheie

4

4*

32768

33.0

Yes*

6

Hurler-Scheie

5

13*

32768

95.4

Yes*

7

Hurler-Scheie

8

15

256

0

No

8

Hurler-Scheie

6

13*

512

0

No

9

Hurler-Scheie

3

9*

1

n/a

No

10

Hurler-Scheie

3

10*

256

0

No

11

Hurler-Scheie

7

14*

1

n/a

No

12

Hurler-Scheie

12

12

4096

0

No

13

Hurler-Scheie

2

7*

1

n/a

No

14

Hurler-Scheie

4

4

32768

25.5

No

15

Hurler-Scheie

4

7*

256

30.09

No

16

Hurler-Scheie

4

7*

32

1

No

17

Scheie

30

30

131072

58.10

No

18

Scheie

27

28*

262144

30.12

Yes*

19

Scheie

15

15

4096

11.6

No

20

Scheie

9

10*

2048

31.4

No

  1. *Sleep study and biochemical data available within 6 months of determination of antibody status.