Table 1:
Baseline characteristics
| Children (≥2 to <17 years) |
Adults (≥17 years) |
Total | ||||
|---|---|---|---|---|---|---|
| Main study (n = 38) |
Extension (n = 10) |
Main study (n = 37) |
Extension (n = 17) |
Main study (N = 75) |
Extension (N = 27) |
|
| Sex [N (%)] | ||||||
| Male | 29 (76.3) | 6 (60.0) | 10 (27.0) | 4 (23.5) | 39 (52.0) | 10 (37.0) |
| Female | 9 (23.7) | 4 (40.0) | 27 (73.0) | 13 (76.5) | 38 (48.0) | 17 (63.0) |
| Age [mean ± SD (range)], years | 8.7 ± 4.2 (2–16) |
9.4 ± 3.7 (5–14) |
47.5 ± 13.6 (20.0–73.0) |
56.1 ± 11.9 (25.0–73.0) |
27.8 ± 21.9 (2–73) |
39.3 ± 24.4 (6–73) |
| Body weight [mean ± SD (range)], kg | 36.4 ± 18.9 (13.0–86.4) |
50.5 ± 28.3 (22.7–97.0) |
68.7 ± 12.7 (44.3–98.6) |
72.1 ± 14.2 (47.7–101.0) |
52.37 ± 22.8 (13.0–98.6) |
64.07 ± 22.7 (22.7–101.0) |
| Etiology of PID [N (%)] | ||||||
| CVID | 20(52.6) | 10 (100) | 36 (97.3) | 14 (82.4) | 56 (74.7) | 24 (88.9) |
| XLA | 6 (15.8) | 0 | 0 | 0 | 6 (8.0) | 0 |
| Other* | 12 (31.6) | 0 | 1 (2.7) | 3 (17.6) | 13 (17.3) | 3 (11.1) |
| Region [N (%)] | ||||||
| Europe | 23 (60.5) | 0 | 17 (45.9) | 0 | 40 (53.3) | 0 |
| United States | 15 (39.5) | 10 (100) | 17 (45.9) | 11 (64.7) | 32 (42.7) | 21 (77.8) |
| Canada | 0 | 0 | 3 (8.1) | 6 (35.3) | 3 (4.0) | 6 (22.2) |
*Other: two cases of hypogammaglobulinemia, and one case each of agammaglobulinemia (not X–linked), selective deficiency of IgG1 and IgG2 with deficiency of specific antibodies; two cases of DiGeorge syndrome, and one case each of hyper IgM hyper IgM syndrome, X-linked hyper IgM syndrome, Nijmegen breakage syndrome, hypogammaglobulinemia IgG1, and Wiskott–Aldrich syndrome.
Abbreviations: CVID, common variable immunodeficiency; XLA, X-linked agammaglobulinemia.