Table 2

ADA-SCID patients on ERT with PEG-ADA display reduced growth

Age at last follow-up, yPEG-ADA, yPercentile height, Z-scoreRANKL, pg/mLOPG, pg/mLRANKL/OPG, × 102
Patient no. 6 5.6 −1.69 78.1 
Patient no. 7 1.5 −2.06 120.6 
Patient no. 8 1.4 −0.29 1.61 153.2 1.05 
Patient no. 9 1.3 1.29 145.2 
Patient no. 10 6.2 −0.38 2.85 18.4 15.48 
Patient no. 11 2.5 −2.14 2.57 173.8 1.48 
Patient no. 12 12.5 10 −3.61 93.3 
Patient no. 13 22.5 21 −0.93 167.5 
Patient no. 14 19 17 −1.98 135.3 
ND 1-15 years of age (n = 14), mean (SEM)    8.06 (1.6) 106.9 (5.6) 8.4 (1.7) 
P    .004 .504 .033 
Age at last follow-up, yPEG-ADA, yPercentile height, Z-scoreRANKL, pg/mLOPG, pg/mLRANKL/OPG, × 102
Patient no. 6 5.6 −1.69 78.1 
Patient no. 7 1.5 −2.06 120.6 
Patient no. 8 1.4 −0.29 1.61 153.2 1.05 
Patient no. 9 1.3 1.29 145.2 
Patient no. 10 6.2 −0.38 2.85 18.4 15.48 
Patient no. 11 2.5 −2.14 2.57 173.8 1.48 
Patient no. 12 12.5 10 −3.61 93.3 
Patient no. 13 22.5 21 −0.93 167.5 
Patient no. 14 19 17 −1.98 135.3 
ND 1-15 years of age (n = 14), mean (SEM)    8.06 (1.6) 106.9 (5.6) 8.4 (1.7) 
P    .004 .504 .033 
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