Table 2.
Spectrum of primary immune deficiency
|
Disease |
Number |
Sex |
Age |
||||||
|---|---|---|---|---|---|---|---|---|---|
|
(%) |
M |
F |
|||||||
| Combined | 10 (13.6) | < 1 year | 1 - < 5 years | 5 < 12 years | 12-<18 years | 18-<30 years | ≥30 years | ||
| Severe combined immuno deficiency (including X linked = 02 Omenn syndrome = 01) |
10 (13.6) |
04 |
06 |
10 |
- |
- |
- |
- |
- |
|
Well defined syndromes |
10 (13.6) |
|
|
|
|
|
|
|
|
| Ataxia telangiectasia |
06 (8.2) |
02 |
04 |
- |
03 |
03 |
- |
- |
- |
| Di George syndrome |
02 (2.7) |
01- |
01 |
02 |
- |
- |
- |
- |
- |
| Hyper IgE syndrome (autosomal dominant) |
02 (2.7) |
01 |
01 |
- |
- |
01 |
01 |
- |
- |
|
Antibody deficient |
44 (60.27) |
|
|
|
|
|
|
|
|
| X Linked agammaglobulinemia |
15 (20.54) |
15 |
0 |
01 |
07 |
05 |
02 |
- |
- |
| Autosomal recessive agammaglobulinemia |
02 (2.7) |
0 |
02 |
- |
01 |
01 |
|
- |
- |
| Common variable immune deficiency |
21 (28.76) |
10 |
11 |
- |
01 |
04 |
04 |
04 |
08 |
| Partial IgA deficiency |
01 (1.36) |
0 |
01 |
- |
- |
- |
01 |
- |
- |
| Hyper IgM syndrome (including CD 40 deficiency = 01) |
05 (6.8) |
03 |
02 |
- |
02 |
03 |
- |
- |
- |
|
Immune dysregulation |
03 (4.1) |
|
|
|
|
|
|
|
|
| Griscelli syndrome |
03 (4.1) |
02 |
01 |
03 |
|
|
|
|
|
|
Phagocytic defects |
06 (8.2) |
|
|
|
|
|
|
|
|
| Chronic granulomatous disease |
04 (5.4) |
04 |
0 |
01 |
02 |
01 |
- |
- |
- |
| Leucocyte adhesion deficiency type 1 |
02 (2.7) |
0 |
02 |
02 |
- |
- |
- |
- |
- |
|
Autoinflammatory |
0 |
- |
- |
- |
- |
- |
- |
- |
|
|
Innate immune defects |
0 |
- |
- |
- |
- |
- |
- |
- |
|
|
Complement defects |
0 |
- |
- |
- |
- |
- |
- |
- |
|
| Total | 73 | 42 31 | 19 | 16 | 18 | 08 | 04 | 08 | |