Table 3 Lysosomal enzyme activities in patients with ML II/III
Case no. | Reference range | Reference range a | |||||
|---|---|---|---|---|---|---|---|
1 | 2 | 5a | 6a | 7 | (nmol/hr/mg) | (nmol/min/mL) | |
Arylsulfatase A | |||||||
Plasma | 847 | 312 | 42 | 43 | 2228 | NA | 0.1–1.6 |
Leukocyte | 66 | 26 | NA | NA | 38 | 25–80 | NA |
α-N-Acetylglucosaminidase | |||||||
Plasma | 234 | NA | 9 | NA | NA | 22.3–60.9 | 0.1–0.6 |
Leukocyte | 0.93 | NA | NA | NA | NA | 0.90–1.51 | NA |
β-Hexosaminidase | |||||||
Plasma | 4520 | 12247 | 14 | 13 | 8196 | 374–666 | 0.5–3.1 |
Leukocyte | 728 | 482 | NA | NA | 358 | 611–991 | NA |
β-Glucosidase | |||||||
Plasma | 0.7 | 0.4 | NA | NA | 0.86 | NA | NA |
Leukocyte | 7.0 | 9.2 | NA | NA | 8.3 | 5.1–11.32 | NA |
β-Glucuronidase | |||||||
Plasma | NA | NA | 18 | 19 | NA | NA | 0.1–2.0 |
Leukocyte | NA | NA | NA | NA | NA | NA | NA |