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. 2020 Sep 18;10(4):300–304. doi: 10.1055/s-0040-1716707

Table 2. Distribution of mucopolysaccharidosis (MPS) subtypes and corresponding enzyme activity ( n  = 40) .

Type of MPS No. of patients Range of enzyme activity (nmol/h/mg protein) Laboratory reference range (nmol/h/mg protein)
I
IH/IHS/IS
4 (10)
2/1/1
α-L-iduronidase
1–14
20–108
II 9 (22.5) Iduronate-2-sulfatase
 6 0–2.3 15–57
 1 0 494–1113
 1 0.05 17–46
 1 4.5 167–475
III
A
7 (17.5)
2
Heparan–N-sulfatase
0.17–0.6
1.3–6.8
B 4 α-N-acetylglucosaminidase
0–1.2
6–20.5
C 1 Acetyl CoA: glucosaminide acetyltransferase
0.5
11.1–48
IVA 16 (40) N-acetylgalactoseamine-6-sulphate sulphatase
 7 0–2.9 23–283
 1 12 24–205
 2 0–7 23–152
 4 2.3–8.6 40–70
 2 0–2.1 3.9–21
VI 4 (10) N-acetyl galactosamine-4-sulfatase
 1 12.6 115–226
 2 6.6–6.8 8.4–45.2
 1 0.12 0.6–8.5

Abbreviations: IH, Hurler; IHS, Hurler-Scheie; IS, Scheie.

Note: Figures in parentheses represent percentage.