Current Concepts of Hyperinflammation in Chronic Granulomatous Disease
Joint Authors
Hector, Andreas
Rieber, Nikolaus
Roos, Dirk
Hartl, Dominik
Kuijpers, Taco
Source
Clinical and Developmental Immunology
Issue
Vol. 2012, Issue 2012 (31 Dec. 2012), pp.1-6, 6 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2011-07-25
Country of Publication
Egypt
No. of Pages
6
Main Subjects
Abstract EN
Chronic granulomatous disease (CGD) is the most common inherited disorder of phagocytic functions, caused by genetic defects in the leukocyte nicotinamide dinucleotide phosphate (NADPH) oxidase.
Consequently, CGD phagocytes are impaired in destroying phagocytosed microorganisms, rendering the patients susceptible to bacterial and fungal infections.
Besides this immunodeficiency, CGD patients suffer from various autoinflammatory symptoms, such as granuloma formation in the skin or urinary tract and Crohn-like colitis.
Owing to improved antimicrobial treatment strategies, the majority of CGD patients reaches adulthood, yet the autoinflammatory manifestations become more prominent by lack of causative treatment options.
The underlying pathomechanisms driving hyperinflammatory reactions in CGD are poorly understood, but recent studies implicate reduced neutrophil apoptosis and efferocytosis, dysbalanced innate immune receptors, altered T-cell surface redox levels, induction of Th17 cells, the enzyme indolamine-2,3-dioxygenase (IDO), impaired Nrf2 activity, and inflammasome activation.
Here we discuss immunological mechanisms of hyperinflammation and their potential therapeutic implications in CGD.
American Psychological Association (APA)
Rieber, Nikolaus& Hector, Andreas& Kuijpers, Taco& Roos, Dirk& Hartl, Dominik. 2011. Current Concepts of Hyperinflammation in Chronic Granulomatous Disease. Clinical and Developmental Immunology،Vol. 2012, no. 2012, pp.1-6.
https://search.emarefa.net/detail/BIM-457583
Modern Language Association (MLA)
Rieber, Nikolaus…[et al.]. Current Concepts of Hyperinflammation in Chronic Granulomatous Disease. Clinical and Developmental Immunology No. 2012 (2012), pp.1-6.
https://search.emarefa.net/detail/BIM-457583
American Medical Association (AMA)
Rieber, Nikolaus& Hector, Andreas& Kuijpers, Taco& Roos, Dirk& Hartl, Dominik. Current Concepts of Hyperinflammation in Chronic Granulomatous Disease. Clinical and Developmental Immunology. 2011. Vol. 2012, no. 2012, pp.1-6.
https://search.emarefa.net/detail/BIM-457583
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-457583