{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T16:12:04Z","timestamp":1753891924338,"version":"3.41.2"},"reference-count":21,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2021,7,7]],"date-time":"2021-07-07T00:00:00Z","timestamp":1625616000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Pediatr."],"abstract":"<jats:p>Nuclear factor \u03baappa-B (NF\u03baB) is a family of transcription factors involved in regulating inflammation and immunity. Mutations in the <jats:italic>NF\u03baB1<\/jats:italic> pathway are associated with primary immune defects and underlie the most common monogenic etiology of common variable immunodeficiency (CVID). However, little is known about how <jats:italic>NF\u03baB1<\/jats:italic> defects or primary immunodeficiency (PID) complicate pregnancy. We present a previously healthy 34-year-old patient who suffered from poor wound healing and sterile sepsis during the post-partum period of each of her three pregnancies. She was otherwise asymptomatic, but her daughter developed Evans Syndrome (ES) with hypogammaglobulinemia prompting expanded genetic testing which revealed a novel monoallelic variant in <jats:italic>NF\u03baB1<\/jats:italic>. This case highlights that pregnancy-related complications of PID can be difficult to recognize and may portend adverse patient outcomes. For these reasons, guidance regarding diagnosis and management of women of childbearing age with PID is warranted.<\/jats:p>","DOI":"10.3389\/fped.2021.648022","type":"journal-article","created":{"date-parts":[[2021,7,7]],"date-time":"2021-07-07T04:56:18Z","timestamp":1625633778000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":2,"title":["Case Report: Post-Partum Complications of NF\u03baB1 Deficiency Underscore a Need to Better Understand Primary Immunodeficiency Management During Pregnancy"],"prefix":"10.3389","volume":"9","author":[{"given":"Diem-Tran I.","family":"Nguyen","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Amanda","family":"Grimes","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Donald","family":"Mahoney","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sebastian","family":"Faro","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"William T.","family":"Shearer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Aaron L.","family":"Miller","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nicholas L.","family":"Rider","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2021,7,7]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"17023","DOI":"10.1038\/sigtrans.2017.23","article-title":"NF-\u03baB signaling in inflammation","volume":"2","author":"Liu","year":"2017","journal-title":"Signal Transduct Target Ther."},{"key":"B2","doi-asserted-by":"publisher","first-page":"6758","DOI":"10.1038\/sj.onc.1209943","article-title":"NF-kappaB and the immune response","volume":"25","author":"Hayden","year":"2006","journal-title":"Oncogene."},{"key":"B3","doi-asserted-by":"publisher","first-page":"37","DOI":"10.1016\/j.cell.2016.12.012","article-title":"30 years of NF-\u03baB: a blossoming of relevance to human pathobiology","volume":"168","author":"Zhang","year":"2017","journal-title":"Cell."},{"key":"B4","doi-asserted-by":"publisher","first-page":"1165","DOI":"10.1007\/s10875-013-9924-z","article-title":"Autosomal dominant anhidrotic ectodermal dysplasia with immunodeficiency caused by a novel NFKBIA mutation, p.Ser36Tyr, presents with mild ectodermal dysplasia and non-infectious systemic inflammation","volume":"33","author":"Yoshioka","year":"2013","journal-title":"J Clin Immunol."},{"key":"B5","doi-asserted-by":"publisher","first-page":"2046","DOI":"10.1182\/blood-2014-04-571265","article-title":"A nonsense mutation in IKBKB causes combined immunodeficiency","volume":"124","author":"Mousallem","year":"2014","journal-title":"Blood."},{"key":"B6","doi-asserted-by":"publisher","first-page":"2504","DOI":"10.1056\/NEJMoa1309199","article-title":"Deficiency of innate and acquired immunity caused by an IKBKB mutation","volume":"369","author":"Pannicke","year":"2013","journal-title":"N Engl J Med."},{"key":"B7","doi-asserted-by":"publisher","first-page":"277","DOI":"10.1038\/85837","article-title":"X-linked anhidrotic ectodermal dysplasia with immunodeficiency is caused by impaired NF-kappaB signaling","volume":"27","author":"D\u00f6ffinger","year":"2001","journal-title":"Nat Genet."},{"key":"B8","doi-asserted-by":"publisher","first-page":"1978","DOI":"10.3389\/fimmu.2017.01978","article-title":"Human NF-\u03baB1 haploinsufficiency and epstein\u2013barr virus-induced disease\u2014molecular mechanisms and consequences","volume":"8","author":"Hoeger","year":"2018","journal-title":"Front Immunol."},{"key":"B9","doi-asserted-by":"publisher","first-page":"1688","DOI":"10.1016\/j.jaci.2019.03.016","article-title":"NF-\u03baB pathway and the Goldilocks principle: lessons from human disorders of immunity and inflammation","volume":"143","author":"Scott","year":"2019","journal-title":"J Allergy Clin Immunol."},{"key":"B10","doi-asserted-by":"publisher","first-page":"1285","DOI":"10.1016\/j.jaci.2018.01.039","article-title":"Loss-of-function nuclear factor \u03baB subunit 1 (NFKB1) variants are the most common monogenic cause of common variable immunodeficiency in Europeans","volume":"142","author":"Tuijnenburg","year":"2018","journal-title":"J Allergy Clin Immunol."},{"key":"B11","doi-asserted-by":"publisher","first-page":"2618","DOI":"10.3389\/fimmu.2019.02618","article-title":"Late-onset antibody deficiency due to monoallelic alterations in NFKB1","volume":"10","author":"Schr\u00f6der","year":"2019","journal-title":"Front. 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