TABLE 1.
Subjects | Genotype | ||
PI*SZ | PI*ZZ | ||
ADAPT: The UK Registry [15] | 1203 | 135 (11.2) | 930 (77.3) |
AlphaNet (USA) [16, 17] | 5523 | 504 (9.13) | 3031 (54.9) |
Italian Registry [18] | 422 | 74 (17.5) | 258 (61.1) |
German Registry [20] | 1066 | 109 (10.2) | 820 (76.9) |
Spanish Registry [21] | 469# | 100 (21.3) | 348 (74.2) |
AIR [19, 41] | 4758 | 538 (11.3) | 4031 (84.7) |
Alpha One Foundation (Ireland) [25] | 5520¶ | 275 (4.9) | 305 (5.5) |
NHLBI [22] | 1021 | 10 (1.0) | 993 (97.3) |
AOF-RNR [23] | 712 | 15 (2.1) | 503 (70.7) |
Data are presented as n or n (%). ADAPT: Antitrypsin Deficiency Assessment and Programme for Treatment; AIR: Alpha One International Registry; NHLBI: National Heart, Lung, and Blood Institute; AOF-RNR: Alpha One Foundation Research Network Registry. #: only adult patients included; ¶: excluding PI*MM individuals.