Pseudomonas
Severe Congenital Neutropenia - Patients are at high risk for developing gram negative bacteremia with organisms including Pseudomonas.
Cystic Fibrosis - Pseudomonas is a common respiratory pathogen in patients with CF. The respiratory tract becomes colonized early in life and acquisition of a mucoid phenotype can lead to an accelerated loss of lung function.
IRAK 4 deficiency - This disease has been associated with pseudomonas infections in some cases but it does not appear to be the main infectious phenotype. Patients have poor inflammatory response despite invasive infections.
MyD88 deficiency - In contrast, some kindreds with MyD88 deficiency have presented primarily with invasive pseudomonas infections (meningitis, sepsis, abscesses). Patients have poor inflammatory response despite invasive infections.
XLA - Pseudomonas is not a typical organism seen in patients with this disease. However, marked neutropenia can develop during acute infections which can lead to transient susceptibility to this organism. Pseudomonas was the most common cause of sepsis reported in the United States XLA registry.
STAT3 Deficiency - Pseudomonas is not a common cause of primary lung infections in this disease. However, superinfection of pneumatoceles with this organism can occur.