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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.