Table 2 General characteristics of patients with primary immunodeficiency diseases
Category | No. of Cases | Gender Ratio (F/M) | No. of Death | Age at death (mo) | No. of HSCT | Age at onset (mo) | Age at diagnosis (mo) | Family history |
|---|---|---|---|---|---|---|---|---|
SCID | 32 | 22/10 | 30 | 7.03 ± 1.25 (1-28) | 7 | 4.03 ± 0.88 (0.5-23) | 5.94 ± 1.06 (1-28) | 7 |
HIGM | 27 | 27/0 | 4 | 88.50 ± 44.66 (16-205) | 24 | 9.536 ± 2.23 (1-60) | 31.25 ± 6.23 (3-120) | 4 |
WAS | 9 | 9/0 | 0 | 9 | 1.00 ± 0.14 (0.5-2) | 7.22 ± 1.98 (2-22) | 0 | |
DGS | 1 | 1/0 | 0.5 | 0.5 | 0 | |||
DKC | 2 | 2/0 | 1 | 26 | 1 | 37.00 ± 23.00 | 44.00 ± 20.00 | 1 |
EDA-ID | 1 | 1/0 | 1 | 4 | 0.5 | 3 | 1 | |
HIES | 1 | 1/0 | 1 | 133 | 60 | 60 | 0 | |
Severe reduction in all serum immunologlobulin isotypes with profoundly decreased or absent B cells | 16 | 15/1 | 3 | 46.00 ± 23.29 (3-83) | 26.50 ± 6.89 (2-60) | 41.80 ± 10.44 (3-120) | 3 | |
CVID | 3 | 3/0 | 0 | 29.33 ± 13.13 (4-48) | 46.33 ± 26.21 (7-96) | 0 | ||
APDS | 1 | 1/0 | 0 | 96 | 96 | 0 | ||
IPEX syndrome | 2 | 2/0 | 0 | 2 | 6, 28 | 6, 28 | 0 | |
Immune dysregulation with colitis | 1 | 0/1 | 1 | 13 | 1 | 4 | 0 | |
CHS | 1 | 0/1 | 0 | 1 | 2 | 68 | 0 | |
Congenital neutropenias | 5 | 4/1 | 0 | 1 | 2 | |||
CGD | 7 | 6/1 | 1 | 12 | 1 | 2.17 ± 1.17 (1-8) | 5.00 ± 2.11 (1-15) | 2 |
IFN-γ receptor deficiency | 1 | 0/1 | 0 | 1 | 2 | 60 | 0 | |
Predisposition to invasive fungal diseases (CARD9 deficiency) | 1 | 1/0 | 0 | 117 | 130 | 0 | ||
Complement deficiencies | 1 | 1/0 | 0 | 48 | 60 | 0 |