Table 1.
TTR type | Area | Prevalence | Male/female ratio | Median age of onset | Affected organs |
---|---|---|---|---|---|
Wild-type | Worldwide | > 25%a | 25:1 to 50:1 | >70–75 y | Heart, Soft tissue |
Val122Ile | United States, Caribbean, Africa | 3–4%b Afro-Caribbean | 3:1 | > 70 y | Heart, PNS, Soft tissue |
Val30Met | Portugal, Sweden, Japan | 1:1000b | 2:1 | > 50 y (late-onset) 30 y (early-onset) |
Heart, PNS/ANS |
Thr60Ala | United Kingdom, Ireland | 1%b Northwest Ireland | 2:1 | > 45 y | Heart, PNS/ANS |
ANS, autonomic nervous system; PNS, peripheral nervous system; TTR, transthyretin; y, years.
Prevalence histologically rather than clinically.
Prevalence of the mutation.