| • | pathogenesis – group of heterogeneous disorders that share an inability to produce respiratory burst |
| • | (mnemonic – because you can’t produce the acute respiratory burst, you end up with chronic granulomatous lesions – in skin, lungs, etc…) ???) |
| • | diagnosis – NBT reduction assay: leukocytes unable to reduce dye – no blue color change |
| • | ddx – Hyper IgE, SCID, Chediak-Higashi |
| • | infections – recurrent pyoderma (staph); pneumonia with abscesses, empyema |
| • | prognosis – depending on control of infections; most with normal life span but poor quality of life |
| • | treatment – prophylactic antibiotics; leukocyte transfusions; bone-marrow transplant |
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