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. 2016 Mar 24;31:15–27. doi: 10.1007/8904_2016_548

Table 2.

Demographic details of the cases (n = 246) used in correlation studies

Diseases with enzyme Total cases Countries Region Cases Age Gender Positive Normal
Gaucher (β-glucosidase) 54 India North India 23 3 months–7 years 12 M, 11 F 15 8
South India 13 1–10 years 7 M, 6 F 8 5
East India 1 13 years F 1 0
West India 3 2–5 years 1 M, 2 F 2 1
Pakistan 11 11 months–13 years 8 M, 3 F 9 2
Sri Lanka 3 2.6–9 years 3 M 2 1
Pompe (α-glucosidase) 35 India North India 10 10 months–35 years 8 M, 2 F 3 7
South India 15 3 months–22 years 7 M, 8 F 8 7
East India 2 9 months–1 year 2 F 2 0
West India 5 5–12 years 4 M, 1 F 3 2
Pakistan 1 1 year M 1 0
Sri Lanka 2 6 years, 7 years 2 F 0 2
Fabry (α-galactosidase) 11 India North India 7 8–65 years 4 M, 3 F 2 4
South India 4 11–35 years 3 M, 1 F 1 4
Tay Sachs (β-hexosaminidase A) 14 India North India 9 3 months–2.5 years 6 M, 3 F 6 3
South India 5 6 months–4 years 3 M, 2 F 3 2
Sandhoff (Total hexosaminidase) 10 India North India 4 1–3.5 years 2 M, 2 F 2 2
South India 3 2–4 years 1 M, 2 F 2 1
West India 3 3–5 years 2 M, 1 F 1 2
GM1 (β-galactosidase) 11 India North India 8 1–5 years 4 M, 4 F 4 4
West India 3 1.5–8 years 3 M 2 1
Niemann Pick (sphingomyelinase) 5 India North India 5 2.5 months–2.5 years 3 M, 2 F 3 2
NCL1 (Infantile) (palmitoyl-protein thioesterase) 10 India North India 4 1–3 years 1 M, 3 F 2 2
South India 6 4 months–1 year 3 M, 3 F 3 3
NCL2 (late infantile) (tripeptidyl peptidase) 8 India North India 8 7–15 years 6 M, 2 F 3 5
Fucosidosis (α-fucosidase) 7 India North India 5 2–4 years 2 M, 3 F 2 3
East India 2 1–3 years 2 M 0 2
Mannosidosis (α-mannosidase) 5 India North India 5 3.5–8 years 3 M, 2 F 0 5
MPS-I (α-iduronidase) 25 India North India 8 2–4.5 years 3 M, 3 F 4 4
South India 9 1–13 years 4 M, 5 F 6 3
Pakistan 6 1–15 years 3 M, 3 F 3 3
Sri Lanka 2 1–1.6 years 1 M, 1 F 0 2
MPS-II (iduronate 2 sulfatase) 10 India North India 5 1–12 years 4 M, 1 F 4 1
West India 5 2.5–15 years 4 M, 1 F 1 4
MPS-IIIb (α-hexosaminidase) 9 India North India 5 2–5 years 2 M, 3 F 0 5
South India 4 2–2.5 years 2 M, 2 F 2 2
MPS-IVa (galactose 6-sulfatase) 10 India North India 5 2–7 years 4 M, 1 F 3 2
South India 5 5–10 years 2 M, 3 F 2 3
MPS-VI (aryl sulfatase B) 11 India North India 4 6 months–6 years 2 M, 2 F 2 2
South India 5 1–5.3 years 3 M, 2 F 2 3
East India 2 1.5–2.5 years 2 F 2 0
MPS-VII (β-glucuronidase) 8 India North India 5 12–18 years 4 M, 1 F 1 2
West India 3 14–23 years 3 F 2 3
I-Cell 3 India North India 3 3–5 years 3 F 3 0
Total 246 246 127 119