Table 1
From: Primary ciliary dyskinesia and humoral immunodeficiency - what is the missing link?
Patient | Age (y) | Clinical presentation | Immuno deficiency | Laboratory results (before start of replacement therapy) | Â | Â | Â | Â | Â | Treatment HID |
|---|---|---|---|---|---|---|---|---|---|---|
| Â | Â | Â | Â | IgG (g/l) | IgG2 (g/m) | IgG3 (g/l) | IgA (g/l) | IgM (g/l) | Pneumococcal antibodies (before-after vaccination) (U/ml) | Â |
1* | 13 | SI, C, B, CR, E | CVID | 7,54 (6,35-14,89) | 0,5 (0,63-3,0) | 0,13 (0,17-0,88) | 0,29 (0,46-2,51) | 0,23 (0,47-2,2) | Type 3: 33-114, type 4: 38-23, type 9N: 6-33 | SCIG |
2* | 16 | C, B, CR | CVID | 8,55 (4,78-11,29) | 0,38 (0,72-3,4) | 0,27 (0,13-1,33) | 0 (0,35-1,9) | 0,41 (0,34-1,34) | Type 3: 26-57, type 4: 8-54, type 9N:7-66 | SCIG |
3 | 32 | SI, C, B, CR, E | IgG2 and IgG3 deficiency | 8,3 (7-16) | 1,8 (2,42-7,0) | 0,18 (0,22-1,76) | 1,37 (0,7-4,0) | 0,37 (0,4-2,3) | Â | SCIG |
4 | 43 | C, B, CR | IgG3 deficiency | 9,34 (7,51-15,6) | 3,38 (1,50-6,40) | 0,11 (0,20-1,10) | Â | Â | Â | Intermittent IVIG |
5 | 62 | SI, C, B, CR | IgG2 deficiency | 6,64 (7,51-15,6) | 1,14 (1,5-6,4) | 0,96 (0,2-1,1) | 1,43 (0,82-4,53) | 2,71 (0,46-3,04) | Â | Intermittent IVIG |
6 | 33 | C, B, CR, E | IgG3 deficiency | 14,0 (7,51-15,6) | 4,06 (1,17-7,47) | 0,29 (0,41-1,29) | Â | Â | Â | No IG treatment |
7# | 10 | SI, C, B, CR, E | IgA deficiency, SPAD | 14,5 (5,3-13,06) | 1,27 (0,98-4,8) | 0,59 (0,15-1,49) | 0,29 (0,6-2,7) | 2,56 (0,43 - 2,7) | Type 3: 30-21, type 4: 9-7, type 9N: 8-6 | No IG treatment |
8# | 14 | C, B, CR, E | IgA deficiency | 12,6 (5,76-12,65) | Â | Â | 0,56 (0,81-2,32) | 1,28 (0,3-1,59) | Â | No IG treatment |