Table 2 Spectrum of primary immune deficiency
From: Spectrum of primary immunodeficiency disorders in Sri Lanka
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) | Â | Â | Â | Â | Â | Â | Â | Â |
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) | Â | Â | Â | Â | Â | Â | Â | Â |
X Linked agammaglobulinemia | 15 (20.54) | 15 | 0 | 01 | 07 | 05 | 02 | - | - |
Autosomal recessive agammaglobulinemia | 02 (2.7) | 0 | 02 | - | 01 | 01 | Â | - | - |
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) | Â | Â | Â | Â | Â | Â | Â | Â |
Griscelli syndrome | 03 (4.1) | 02 | 01 | 03 | Â | Â | Â | Â | Â |
Phagocytic defects | 06 (8.2) | Â | Â | Â | Â | Â | Â | Â | Â |
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 | |