Table 2.
Enzymatic activities (µmol/L/h) of 825 neonates determined by three-hour incubation and Turboflow chromatography method.
| ABG |
ASM |
GAA |
GLA |
IDUA |
|
|---|---|---|---|---|---|
| n | n | n | n | n | |
| mean | 19.6 | 4.4 | 18.6 | 6.4 | 12.4 |
| percentile 0.5% | 6.97 | 1.06 | 5.63 | 2.60 | 3.27 |
| percentile 1.0% | 7.99 | 1.12 | 6.90 | 2.68 | 3.87 |
| percentile 25% | 15.41 | 2.34 | 13.55 | 4.35 | 9.21 |
| median | 18.78 | 3.80 | 17.17 | 5.52 | 11.73 |
| percentile 75% | 23.06 | 5.67 | 22.49 | 7.38 | 14.77 |
| percentile 99% | 39.30 | 13.41 | 43.52 | 18.88 | 27.18 |
| percentile 99.5% | 41.49 | 13.73 | 50.31 | 22.24 | 31.12 |
| min | 5.19 | 1.06 | 4.75 | 2.54 | 2.67 |
| max | 44.02 | 21.34 | 69.25 | 60.02 | 62.72 |
ABG, β-Glucocerebrosidase; ASM, acid-Sphingomyelinase; GAA α-Glucosidase; GLA, α -Galactosidase; IDUA, Iduronidase α-L; nbs, newborns;
1
Maximum enzyme activity observed among the affected patients divided by the mean activity measured for newborns.