{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T22:31:39Z","timestamp":1792708299278,"version":"4.5.0"},"reference-count":58,"publisher":"American Society of Hematology","issue":"1","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2016,12,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Immune deficiencies resulting from inherited defects in neutrophil function have revealed important features of the innate immune response. Although sharing an increased susceptibility to bacterial and fungal infections, these disorders each have distinctive features in their clinical manifestations and characteristic microbial pathogens. This review provides an update on several genetic disorders with impaired neutrophil function, their pathogenesis, and treatment strategies. These include chronic granulomatous disease, which results from inactivating mutations in the superoxide-generating nicotinamide dinucleotide phosphate oxidase. Superoxide-derived oxidants play an important role in the control of certain bacterial and fungal species, and also contribute to the regulation of inflammation. Also briefly summarized are updates on leukocyte adhesion deficiency, including the severe periodontal disease characteristic of this disorder, and a new immune deficiency associated with defects in caspase recruitment domain\u2013containing protein 9, an adaptor protein that regulates signaling in neutrophils and other myeloid cells, leading to invasive fungal disease.<\/jats:p>","DOI":"10.1182\/asheducation-2016.1.43","type":"journal-article","created":{"date-parts":[[2016,12,2]],"date-time":"2016-12-02T16:05:27Z","timestamp":1480694727000},"page":"43-50","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":69,"title":["Primary immune deficiencies with defects in neutrophil function"],"prefix":"10.1182","volume":"2016","author":[{"given":"Mary C.","family":"Dinauer","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Washington University School of Medicine in St. Louis, St. Louis, MO"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"234","reference":[{"key":"2019111703094453300_B1","first-page":"773","article-title":"Phagocyte system and disorders of granulopoiesis and granulocyte function","volume-title":"Nathan and Oski\u2019s Hematology of Infancy and Childhood","author":"Dinauer","year":"2015"},{"key":"2019111703094453300_B2","doi-asserted-by":"crossref","DOI":"10.1155\/2012\/252460","article-title":"Current concepts of hyperinflammation in chronic granulomatous disease","author":"Rieber","year":"2012"},{"issue":"1","key":"2019111703094453300_B3","doi-asserted-by":"crossref","first-page":"228","DOI":"10.1111\/imr.12378","article-title":"Ncf1 polymorphism reveals oxidative regulation of autoimmune chronic inflammation","volume":"269","author":"Holmdahl","year":"2016","journal-title":"Immunol Rev"},{"issue":"11","key":"2019111703094453300_B4","doi-asserted-by":"crossref","first-page":"1118","DOI":"10.1111\/tra.12115","article-title":"Regulation of the NADPH oxidase and associated ion fluxes during phagocytosis","volume":"14","author":"Nunes","year":"2013","journal-title":"Traffic"},{"issue":"3","key":"2019111703094453300_B5","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1097\/00005792-200005000-00003","article-title":"Chronic granulomatous disease. 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