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Table 5 Primary immunodeficiencies associated with non-CF bronchiectasis in childhood (n = 131)

From: The etiologies of non-CF bronchiectasis in childhood: a systematic review of 989 subjects

  Total number % of total
B cell disorders 97 74%
IgG deficiency* 63 48%
IgG subclass deficiency 24 18%
IgA deficiency 9 7%
B cell deficiency NOS 1 1%
T cell disorders 9 7%
Hyper IgE syndrome 3 2%
Hyper IgM syndrome 2 2%
T Cell deficiency 3 2%
Chronic mucocutaneous candidiasis 1 1%
Combined immunodeficiency 13 10%
Severe combined immunodeficiency** 9 7%
Ataxia-telangiectasia 2 2%
Wiskott-Aldrich syndrome 2 2%
Chronic granulomatous disease 7 5%
Barre lymphocyte syndrome/MHC class II deficiency 2 2%
Mannose-binding protein deficiency 1 1%
Other disorders 2 2%
  1. *Includes patients identified as common variable immunodeficiency (30), IgG deficiency (13), agammaglobulinemia (10) and antibody deficiency or dysfunction (10).
  2. **Not otherwise specified.