Jordan S Orange1. 1. Department of Pediatrics, University of Pennsylvania School of Medicine, Division of Allergic and Immunologic diseases, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Abstract
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.
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 immunodeficientpatient 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.
Authors: Benjamin J Staudinger; Jocelyn Fraga Muller; Skarphéðinn Halldórsson; Blaise Boles; Angus Angermeyer; Dao Nguyen; Henry Rosen; Olafur Baldursson; Magnús Gottfreðsson; Guðmundur Hrafn Guðmundsson; Pradeep K Singh Journal: Am J Respir Crit Care Med Date: 2014-04-01 Impact factor: 21.405
Authors: Sara Singhal; Mathias W Allen; John-Ryan McAnnally; Kenneth S Smith; John P Donnelly; Henry E Wang Journal: PeerJ Date: 2013-05-21 Impact factor: 2.984
Authors: Rosita Rigoni; Elena Fontana; Kerry Dobbs; Veronica Marrella; Valentina Taverniti; Virginia Maina; Amanda Facoetti; Giovanna D'Amico; Waleed Al-Herz; Mario Ernesto Cruz-Munoz; Catharina Schuetz; Andrew R Gennery; Elizabeth K Garabedian; Silvia Giliani; Deborah Draper; Ghassan Dbaibo; Raif S Geha; Isabelle Meyts; Thomas Tousseyn; Benedicte Neven; Despina Moshous; Alain Fischer; Ansgar Schulz; Andrea Finocchi; Douglas B Kuhns; Danielle L Fink; Michail S Lionakis; Muthulekha Swamydas; Simone Guglielmetti; Julie Alejo; Ian A Myles; Stefania Pittaluga; Luigi D Notarangelo; Anna Villa; Barbara Cassani Journal: J Allergy Clin Immunol Date: 2020-04-18 Impact factor: 10.793