Table 3 Clinical manifestations and complications of patients with primary immunodeficiency diseases
Type | Respiratory infections | Repeated Diarrhea | Bacterial Infection Of Skin and Mucous Membrane | Fungal Infection | Meningitis | BCG-osis |
|---|---|---|---|---|---|---|
SCID | 19 | 13 | 8 | 6 | 4 | 9 |
HIGM | 26 | 6 | 10 | 7 | 0 | 4 |
WAS | 5 | 2 | 4 | 0 | 0 | 0 |
DGS | 1 | 0 | 0 | 0 | 0 | 0 |
DKC | 2 | 1 | 1 | 0 | 0 | 0 |
EDA-ID | 1 | 0 | 0 | 0 | 0 | 0 |
HIES | 1 | 0 | 0 | 0 | 0 | 0 |
Severe reduction in all serum immunologlobulin isotypes with profoundly decreased or absent B cells | 14 | 1 | 2 | 1 | 2 | 0 |
CVID | 3 | 0 | 0 | 0 | 1 | 0 |
APDS | 1 | 0 | 0 | 0 | 0 | 0 |
IPEX syndrome | 1 | 2 | 1 | 1 | 0 | 0 |
Immune dysregulation with colitis | 0 | 1 | 1 | 0 | 0 | 0 |
CHS | 0 | 0 | 1 | 0 | 0 | 0 |
Congenital neutropenias | 7 | 0 | 1 | 2 | 1 | 0 |
CGD | 6 | 1 | 6 | 0 | 0 | 7 |
IFN-γ receptor deficiency | 1 | 0 | 1 | 0 | 0 | 1 |
CARD9 deficiency | 0 | 0 | 1 | 1 | 1 | 0 |
Complement deficiencie | 1 | 0 | 1 | 0 | 1 | 0 |
Total (%) | 89 (79.5) | 26 (23.2) | 38 (33.9) | 18 (16.1) | 10 (8.9) | 21 (18.8) |