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Table 4 Meta-analysis of Rett prevalence (per 100,000 females)

From: Global prevalence of Rett syndrome: systematic review and meta-analysis

Case definition criteria

 Study

Country

Rett

cases

Female

population

Prevalence

estimate

95%

LCL

95%

UCL

Clinical diagnosis onlya

 Fombonne et al. (2003) [19]

UK

2

5227

38.3

4.6

138.0

 Chakrabarti et al. (2005) [20]

UK

1

13202

7.6

0.2

42.2

 Aguilera et al. (2007) [18]

Spain

1

63675

1.6

0.0

8.7

 Isaksen et al. (2012) [16]

Norway

3

15662

19.2

4.0

56.0

 Strømme et al. (2000) [15]

Norway

1

14542

6.9

0.2

38.3

 Magnússon et al. (2001) [14]

Iceland

0

41896

0.0

0.0

8.8

 Pooled prevalence

 

8

154204

6.7

2.0

22.0

Clinical diagnosis + genetic testingb

 Wong et al. (2007) [22]

China

7

123968

5.6

2.3

11.6

 Sarajlija et al. (2015) [17]

Serbia

102

857142

11.9

9.7

14.4

 Bienvenu et al. (2006) [13]

France

251

4337627

5.8

5.1

6.5

 Fehr et al. (2011) [21]

Australia

305

4094386

7.4

6.6

8.3

 Pooled prevalence

 

665

9413123

7.6

5.4

10.8

All studiesc

 

673

9567327

7.1

4.8

10.5

 Pooled prevalence

      
  1. aGenetic testing not reported in the study publications (n = 6 studies, Q = 13.0; df = 5; heterogeneity p = 0.0231; I2 =0.616)
  2. bGenetic testing reported in the study publications (n = 4 studies, Q = 36.6; df = 3; heterogeneity p<0.001; I2 = 0.918)
  3. cAll eligible publications (n = 10 studies, Q = 53.3; df = 9; heterogeneity p<0.001; I2 = 0.831, subgroup difference p=0.84)
  4. LCL Lower confidence limit, UCL Upper confidence limit