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Table 1 Prevalence studies of MPS I

From: The prevalence of and survival in Mucopolysaccharidosis I: Hurler, Hurler-Scheie and Scheie syndromes in the UK

Study Region Ascertainment period No. of cases No. of live births Syndrome subtype Birth Prevalence/100,000
Lowry (1971) [8]British Columbia 1952 – 1968 7 606,157 All 1.15
     Hurler 0.99
     Scheie 0.16
Lowry (1990) [9]British Columbia 1952 – 1986 7 665,702 All 0.77
     Hurler 0.69
     Scheie 0.08
Nelson (1997) [10]N. Ireland 1958 – 1985 14 839,520 All 1.66
     Hurler 1.30
     Hurler-Scheie 0.36
     Scheie No cases
Nelson (2003) [11]Western Australia 1969 – 1996 6 641,178 All 0.93
     Hurler 0.93
     Hurler-Scheie No cases
     Scheie No cases
Poorthuis (1999) [12]Netherlands 1970 – 1996 82 6,871,909 All 1.19
Meikle (1999) [13]Australia 1980 – 1996 38 3,344,000 All 1.14
Hutchesson (1998) [14]West Midlands UK 1981 – 1991 7 707,720 All 0.99
Baehner (2005) [15]Germany 1980 – 1995 93 13,410,924 All 0.69
     Hurler 0.61
     Hurler-Scheie 0.03
     Scheie 0.05
This Study England & Wales 1981 – 2003 167 15,611,220 All 1.07
     Hurler 0.76
     Hurler-Scheie 0.24
     Scheie 0.07
  1. † 10 prenatal diagnoses were additionally observed.