From: Spectrum of primary immunodeficiency disorders in Sri Lanka
History | Number (%) |
---|---|
(n = 942) | |
Age | |
Birth - < 1 year | 362 (38.4) |
1 - < 5 years | 359 (38.15) |
5 - < 12 years | 49 (5.2) |
12- < 18 years | 41 (4.35) |
18- < 30 years | 49 (5.2) |
> 30 years | 82 (8.7) |
Sex | |
Male | 526 (55.8) |
Female | 416 (44.16) |
Recurrent (proven) infection | |
≥ 2 severe infections (pneumonia, sepsis, meningitis, osteomyelitis) | 393 (42.7) |
Atypical presentation of infection | 6 (0.6) |
Unusually severe course, or impaired response to treatment | 230 (24.4) |
Unusual or opportunistic agent | 10 (1.0) |
Recurrent infections with same type of pathogen | 60 (6.3) |
Abscesses of internal organs, or recurrent subcutaneous abscesses | 115 (12.2) |
FTT with prolonged/recurrent diarrhoea | 24 (2.5) |
Generalized long lasting warts or molluscum contagiosum | 5 (0.5) |
Extensive prolonged candidiasis | 16 (1.7) |
Delayed separation (> 4 weeks) of umbilical cord | 2 (0.2) |
Delayed shedding of primary teeth | 2 (0.2) |
F/H of infant deaths, ID, consanguinity | 16 (1.7) |
Difficult to treat obstructive lung disease, unexplained bronchiectasis | 50 (5.3) |
Physical examination | |
Dysmorphic features, especially facial abnormalities, microcephaly | 0 |
Partial albinism, abnormal hair, severe eczema | 3 (0.3) |
Telangiectasia, ataxia | 10 (1.0) |
Gingivitis, oral ulcers/aphthae | 0 |
Absence of immunological tissue | 0 |
Organomegaly | 0 |
Digital clubbing | 0 |