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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.