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. 2020 Nov;9(4):196–204. doi: 10.5582/irdr.2020.03082

Table 1. The estimated prevalence and penetrance of AIP.

Country (region) or Population Prevalence of symptomatic AIP (case per million inhabitants) Prevalence of pathogenetic HMBS mutations Penetrance (%) Ref.
Caucasian 1/1,782 < 1 14
Europe 5.9 19
France 7.6 1/1,299 0.5-1 22.9 25
1/1,675 15
5.5 19
Sweden 23 23 19
1/10,000 30
Northern Sweden 1/1,000 30
192 1/2,000 42 27
Switzerland 10-15 50 52 31
9.9 19
Southeastern Spain 17.7 26
Spain 6.3 52 19
Finland 47 35 32
5.9 19
Norway 6.3 19
Northern Italy 5.0 19
Poland 7.2 19
UK 7.2 19
Netherlands 8.1 19
Russia 59 57 58
Argentina* 8 40 28
Western Australia* 24 29 29

*No gene sequencing was performed in the study