Table 4.
Meta-analysis of Rett prevalence (per 100,000 females)
|
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 | ||||||
aGenetic testing not reported in the study publications (n = 6 studies, Q = 13.0; df = 5; heterogeneity p = 0.0231; I2 =0.616)
bGenetic testing reported in the study publications (n = 4 studies, Q = 36.6; df = 3; heterogeneity p<0.001; I2 = 0.918)
cAll eligible publications (n = 10 studies, Q = 53.3; df = 9; heterogeneity p<0.001; I2 = 0.831, subgroup difference p=0.84)
LCL Lower confidence limit, UCL Upper confidence limit