TABLE 1.
PID with indicated level of susceptibility: | ||
---|---|---|
High | Intermediate | Low |
XL-agammaglobulinemia (19, 64, 88) | SCID (105) | Chronic granulomatous disease (1, 15, 113) |
CVID (18, 22) | X-linked hyper-IgM syndrome (CD40L deficiency) (66, 114) | Leukocyte adhesion deficiencies (LADs) (97) |
IgG2 deficiency (8, 46, 84, 101) | Deficiencies of MAC components (C5-9) and factor H of complement system (43, 107) | |
Selective anti-polysaccharide antibody deficiency (46) | Hyper-IgM type 2 (AID deficiency) (72, 91) | |
IL-12/IL-23-IFN-γ axis deficiencies (33, 36, 42, 85, 96) | ||
Selective IgA deficiency (21, 46, 54, 89) | ||
Deficiencies of early components of classical pathway of complement system (C1, C4, C2), C3, factors I and D (43, 107) | Selective IgA deficiency (21, 46, 54, 89) | |
Ataxia-telangiectasia syndrome (79) | ||
MHC-II deficiency (45, 57) | ||
IRAK-4 deficiencya (23, 38, 61, 70, 86) | Neutropenias (9, 25) | |
Defects of NEMO-dependent NF-κB activation (XL-EDA-ID) (60, 61, 83) | Hyper-IgE syndrome (10, 50, 51) | ) |
Asplenia (48, 99) | ||
MHC-I deficiencies (due to TAP-1 or TAP-2 deficiencies) (32, 45, 47) |
H. influenzae b was not isolated from IRAK-4-deficient patients (61).