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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.