TABLE 1.
Estimated numbers of α1-antitrypsin protease inhibitor ZZ (PiZZ) deficiency and COPD individuals in 20 European countries
| Geographic region and country | Country population/total population ≥40 years n | Total PiZZ (95% CI) | PiZZ ≥40 years (95% CI) | PiZZ ≥40 years and penetrance 60% (95% CI) | Total COPD (95% CI) | PiZZ/COPD ratio % | PiZZ/COPD prevalence (1/x) n |
| Northern Europe | |||||||
| Denmark | 5 627 235/2 835 230 | 4090 (3459–4834) | 2061 (1743–2436) | 1236 (1046–1461) | 326 051 (249 500–399 767) | 0.38 | 264 |
| Estonia | 1 315 819/663 339 | 752 (481–1170) | 379 (242–590) | 227 (145–354) | 76 284 (58 373–93 530) | 0.30 | 335 |
| Finland | 5 451 270/2 811 795 | 1929 (1553–2394) | 995 (801–1235) | 597 (481–741) | 323 356 (247 437–396 463) | 0.18 | 542 |
| Latvia | 2 943 472/1 040 757 | 4005 (1802–8648) | 1416 (637–3058) | 850 (382–1835) | 119 687 (91 586–146 746) | 0.71 | 141 |
| Lithuania | 2 001 468/1 530 171 | 717 (405–1261) | 548 (310–964) | 329 (186–578) | 175 970 (134 655–215 754) | 0.19 | 535 |
| Norway | 5 107 970/2 410 416 | 1798 (1321–2442) | 848 (623–1152) | 509 (374–691) | 277 198 (212 116–339 868) | 0.18 | 545 |
| Sweden | 9 644 864/4 814 821 | 2262 (1674–3052) | 1129 (836–1524) | 678 (501–914) | 553 704 (423 704–678 889) | 0.12 | 817 |
| Subtotal | 32 092 098/16 106 529 | 15 553 (10 695–23 801) | 7377 (5192–10 958) | 4426 (3115–6575) | 1 852 251 (1417–375-) | 0.24 | 418 |
| Western Europe | |||||||
| Belgium | 11 180 840/5 633 352 | 3193 (1745–5787) | 1609 (879–2916) | 965 (528–1749) | 799 936 (495 734–1 104 136) | 0.12 | 829 |
| France | 66 458 153/33 219 475 | 17 191 (13 255–22 270) | 8593 (6626–11 132) | 5156 (3975–6679) | 4 717 165 (2 923 313–6 511 017) | 0.11 | 915 |
| Netherlands | 16 829 289/8 646 510 | 5353 (3057–9298) | 2750 (1571–4777) | 1650 (942–2866) | 1 227 804 (760 892–1 694 715) | 0.13 | 744 |
| Republic of Ireland | 4 637 852/1 938 181 | 2265 (1264–4019) | 947 (528–1,80) | 568 (317–1008) | 275 222 (170 559–379 883) | 0.21 | 485 |
| UK | 64 351 203/31 282 515 | 13 044 (8315–20 421) | 6341 (4042–9927) | 3805 (2425–5956) | 4 442 117 (2 752 861–6 131 372) | 0.09 | 1168 |
| Subtotal | 163 457 337/ 80 720 033 | 41 046 (27 636–61 795) | 20 240 (13 646–30 431) | 13 666 (8187–18 259) | 11 462 245 (7 103 363–15 821 126) | 0.11 | 944 |
| Central Europe | |||||||
| Austria | 8 507 786/4 484 191 | 1529 (646–3536) | 806 (350–1 864) | 484 (210–1 118) | 632 271 (466 355–802 670) | 0.08 | 1308 |
| Germany | 80 767 463/45 778 502 | 20 611 (13 380–31 626) | 11 682 (7584–17 925) | 7009 (4550–10 755) | 6 454 769 (4 760 964–8 194 351) | 0.11 | 921 |
| Poland | 38 017 856/18 015 506 | 6791 (4395–10 454) | 3218 (2083–4954) | 1931 (1250–2972) | 2 540 186 (1 873 612–3 224 775) | 0.08 | 1316 |
| Switzerland | 8 139 631/4 199 289 | 972 (434–2139) | 501 (224–1104) | 301 (134–662) | 592 100 (436 726–751 672) | 0.05 | 1968 |
| Subtotal | 135 432 736/ 72 477 488 | 29 903 (18 873–47 755) | 16 208 (10 240–25 846) | 9725 (6144–15 508) | 10 219 326 (7 537 659–12 973 470) | 0.10 | 1051 |
| Southern Europe | |||||||
| Italy | 60 782 668/33 982 240 | 10 652 (7046–16 049) | 5955 (3939–8973) | 3573 (2364–5384) | 3 670 082 (2 650 614–4 689 549) | 0.10 | 1027 |
| Portugal | 10 427 301/5 546 011 | 4944 (2403–10 004) | 2630 (1278–5321) | 1578 (767–3193) | 598 969 (432 588–765 349) | 0.26 | 380 |
| Spain | 46 512 199/23 903 789 | 14 522 (9405–22 331) | 7479 (4833–11 476) | 4487 (2900–6886) | 2 581 609 (1 864 495–3 298 722) | 0.17 | 575 |
| Subtotal | 117 722 168/63 432 040 | 30 148 (18 854–48 384) | 16 064 (10 051–25 770) | 9638 (6030–15 462) | 6 850 660 (4 947 699–8 753 622) | 0.14 | 711 |
| Eastern Europe | |||||||
| Serbia | 7 146 759/3 751 833 | 1159 (525–2510) | 608 (276–1318) | 365 (165–791) | 465 227 (405 197–525 256) | 0.08 | 1274 |
| Subtotal | 7 146 759/3 751 833 | 1159 (525–2510) | 608 (276–1318) | 365 (165–791) | 465 227 (405 197–525 256) | 0.08 | 1274 |
| TOTAL | 455 851 098/236 487 923 | 117 809 (76 583–184 245) | 60 496 (39 405–94 323) | 36 298 (23 643–56 594) | 30 849 709 (21 411 293–40 344 496) | 0.12 | 850 |