{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,7]],"date-time":"2026-07-07T23:23:42Z","timestamp":1783466622341,"version":"3.55.0"},"reference-count":85,"publisher":"American Academy of Pediatrics (AAP)","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2019,2,1]]},"abstract":"<jats:p>Primary immunodeficiency disorders (PIDs) are genetic diseases that lead to increased susceptibility to infection. Hundreds of PIDs have now been described, but a select subset commonly presents in the neonatal period. Neonates, especially premature newborns, have relative immune immaturity that makes it challenging to differentiate PIDs from intrinsic immaturity. Nonetheless, early identification and appropriate management of PIDs are critical, and the neonatal clinician should be familiar with a range of PIDs and their presentations. The neonatal clinician should also be aware of the importance of consulting with an immunologist when a PID is suspected. The role of newborn screening for severe combined immunodeficiency, as well as the initial steps of laboratory evaluation for a PID should be familiar to those caring for neonates. Finally, it is important for providers to be familiar with the initial management steps that can be taken to reduce the risk of infection in affected patients.<\/jats:p>","DOI":"10.1542\/neo.20-2-e67","type":"journal-article","created":{"date-parts":[[2019,2,1]],"date-time":"2019-02-01T14:29:52Z","timestamp":1549031392000},"page":"e67-e78","source":"Crossref","is-referenced-by-count":10,"title":["Primary Immunodeficiency in the NICU"],"prefix":"10.1542","volume":"20","author":[{"given":"Amy E.","family":"O\u2019Connell","sequence":"first","affiliation":[{"name":"Division of Newborn Medicine, Boston Children\u2019s Hospital, and Department of Pediatrics, Harvard Medical School, Boston, MA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"417","published-online":{"date-parts":[[2019,2,1]]},"reference":[{"issue":"2","key":"2021121007330831200_B1","doi-asserted-by":"crossref","first-page":"311","DOI":"10.1002\/eji.201141847","article-title":"Developmental regulation of Th17-cell capacity in human neonates","volume":"42","author":"Black","year":"2012","journal-title":"Eur J Immunol"},{"issue":"7","key":"2021121007330831200_B2","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1016\/j.it.2014.04.007","article-title":"Ontogeny of early life immunity","volume":"35","author":"Dowling","year":"2014","journal-title":"Trends Immunol"},{"key":"2021121007330831200_B3","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1186\/1471-2431-11-29","article-title":"Phagocytic ability of neutrophils and monocytes in neonates","volume":"11","author":"Filias","year":"2011","journal-title":"BMC Pediatr"},{"issue":"5","key":"2021121007330831200_B4","doi-asserted-by":"crossref","first-page":"503","DOI":"10.1038\/pr.2013.145","article-title":"Phagocytosis of neonatal pathogens by peripheral blood neutrophils and monocytes from newborn preterm and term infants","volume":"74","author":"Prosser","year":"2013","journal-title":"Pediatr Res"},{"issue":"3","key":"2021121007330831200_B5","doi-asserted-by":"crossref","first-page":"607","DOI":"10.1189\/jlb.4A0116-001RR","article-title":"Reduced TNF-\u03b1 response in preterm neonates is associated with impaired nonclassic monocyte function","volume":"100","author":"Wisgrill","year":"2016","journal-title":"J Leukoc Biol"},{"issue":"25","key":"2021121007330831200_B6","doi-asserted-by":"crossref","first-page":"6419","DOI":"10.1182\/blood-2008-07-171629","article-title":"Impaired neutrophil extracellular trap (NET) formation: a novel innate immune deficiency of human neonates","volume":"113","author":"Yost","year":"2009","journal-title":"Blood"},{"issue":"1","key":"2021121007330831200_B7","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1007\/s10875-017-0464-9","article-title":"International Union of Immunological Societies: 2017 Primary Immunodeficiency Diseases Committee report on inborn errors of immunity","volume":"38","author":"Picard","year":"2018","journal-title":"J Clin Immunol"},{"issue":"3","key":"2021121007330831200_B8","doi-asserted-by":"crossref","first-page":"736","DOI":"10.1007\/s12026-016-8784-z","article-title":"Primary immunodeficiencies worldwide: an updated overview from the Jeffrey Modell Centers Global Network","volume":"64","author":"Modell","year":"2016","journal-title":"Immunol Res"},{"issue":"1","key":"2021121007330831200_B9","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1007\/s10875-013-9954-6","article-title":"Attending to warning signs of primary immunodeficiency diseases across the range of clinical practice","volume":"34","author":"Costa-Carvalho","year":"2014","journal-title":"J Clin Immunol"},{"issue":"3","key":"2021121007330831200_B10","doi-asserted-by":"crossref","first-page":"798","DOI":"10.1542\/peds.108.3.798","article-title":"Clinical practice guideline: management of sinusitis","volume":"108","author":"American Academy of Pediatrics. 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