Skip to main content

Advertisement

Table 1 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)       Treatment HID
     IgG (g/l) IgG2 (g/m) IgG3 (g/l) IgA (g/l) IgM (g/l) Pneumococcal antibodies (before-after vaccination) (U/ml)  
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)   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)     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)   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)     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)    0,56 (0,81-2,32) 1,28 (0,3-1,59)   No IG treatment
  1. Legend to the figure: B: bronchiectasis, C: chronic cough, CR: chronic rhinosinusitis, E: recurrent ear infections, Si: situs inversus, SPAD: specific polysaccharide antibody deficiency, SCIG: subcutaneous immunoglobulin, IVIG: intravenous immunoglobulin, * and #pair of sisters. Normal reference values are reported between brackets.