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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)         
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