Version May 2024
| B cell defects |
| Recurrent pyogenic infections with extracellular encapsulated organisms, such as Streptococcus pneumoniae, Haemophilus influenzae type b, and group A Streptococcus. |
| Otitis, sinusitis, recurrent pneumonia, bronchiectasis, and conjunctivitis. |
| Few problems with fungal or viral infections (except enterovirus and poliomyelitis). |
| Diarrhea common, especially secondary to infection with Giardia lamblia. |
| Minimal growth retardation. |
| Compatible with survival to adulthood or for several years after onset unless complications occur. |
| Complement defects |
| Recurrent bacterial infections with extracellular encapsulated organisms, such as S. pneumoniae and H. influenzae. |
| Susceptibility to recurrent infections with Neisseria meningitides. |
| Increased incidence of autoimmune disease. |
| Severe or recurrent skin and respiratory tract infection. |
| T cell defects |
| Recurrent infections with less virulent or opportunistic organisms, such as fungi, Candida sp mycobacteria, viruses, and protozoa as well as bacteria. |
| Growth retardation, malabsorption, diarrhea, and failure to thrive common. |
| Anergy. |
| Susceptible to graft-versus-host disease from nonirradiated blood or from maternal engraftment. |
| Fatal reactions may occur from live virus or Bacille Calmette-Guérin vaccination. |
| High incidence of malignancy. |
| Poor survival beyond infancy or early childhood. |
| Neutrophil defects |
| Recurrent dermatologic infections with bacteria such as Staphylococcus spp, Pseudomonas spp, and Escherichia coli, and fungi such as Aspergillus. |
| Subcutaneous, lymph node, lung, and liver abscesses. Pulmonary infections common, including abscess and pneumatocele formation, contributing to chronic disease. |
| Bone and joint infection common. |
| Delayed separation of umbilical cord. |
| Absence of pus at site(s) of infection. Poor wound healing. |
| Innate TLR signaling defects (eg, MyD88 and IRAK4 deficiencies) |
| Early life infection with Staphylococcus spp, Streptococcus spp, and Pseudomonas aeruginosa. |
| Impaired/delayed systemic response (fever, acute phase response [increased CRP]) to infection. |
| Affects newborns, infants, and young children; lack of invasive infections after the teenage years. |
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