Congenital immunodeficiencies and sepsis

OBJECTIVE: Because a hallmark of congenital immunodeficiency disorders is susceptibility to recurrent, unusual and/or severe infections, an effort was undertaken to identify a subset of these patients with an increased risk for sepsis. DESIGN: Literature review. RESULTS: Twenty congenital immunodeficiency disorders were identified with increased sepsis susceptibility. CONCLUSION: Distinguishing patients with congenital immunodeficiencies from others with sepsis has important implications for the future well-being of the immunodeficient patient because many of the diseases are modified favorably by appropriate treatment. In addition, better understanding of sepsis in the setting of congenital immunodeficiency has numerous implications for both immunodeficiency and sepsis research. As a group, these disorders define components of the human immune system that are essential for defense against severe infection and demonstrate immunologic themes underlying sepsis susceptibility.