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.


METHODS
Literature review.


RESULTS
Twenty congenital immunodeficiency disorders were identified with increased sepsis susceptibility.


CONCLUSIONS
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.
Orange
Jordan S
JS
Department of Pediatrics, University of Pennsylvania School of Medicine, Division of Allergic and Immunologic diseases, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
D004198:Disease Susceptibility; D005260:Female; D006801:Humans; D007113:Immunity, Innate; D007153:Immunologic Deficiency Syndromes / Q000150:complications* / Q000151:congenital / Q000276:immunology; D007231:Infant, Newborn; D011247:Pregnancy; D018805:Sepsis / Q000209:etiology* / Q000276:immunology