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Inflammatory consequences of inherited disorders affecting neutrophil function

Journal

BLOOD
Volume 133, Issue 20, Pages 2130-2139

Publisher

AMER SOC HEMATOLOGY
DOI: 10.1182/blood-2018-11-844563

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Funding

  1. National Institutes of Health, National Heart, Lung, and Blood Institute [R01HL045635]
  2. National Institutes of Health, National Institute of Arthritis and Musculoskeletal and Skin Diseases [R01AR072212]
  3. Children's Discovery Institute of Washington University
  4. St. Louis Children's Hospital

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Primary immunodeficiencies affecting the function of neutrophils and other phagocytic leukocytes are notable for an increased susceptibility to bacterial and fungal infections as a result of impaired leukocyte recruitment, ingestion, and/or killing of microbes. The underlying molecular defects can also impact other innate immune responses to infectious and inflammatory stimuli, leading to inflammatory and autoimmune complications that are not always directly related to infection. This review will provide an update on congenital disorders affecting neutrophil function in which a combination of host defense and inflammatory complications are prominent, including nicotinamide dinucleotide phosphate oxidase defects in chronic granulomatous disease and beta 2 integrin defects in leukocyte adhesion deficiency.

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