{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,3,30]],"date-time":"2025-03-30T04:16:50Z","timestamp":1743308210754,"version":"3.40.3"},"reference-count":5,"publisher":"Wiley","issue":"S1","license":[{"start":{"date-parts":[[2024,9,19]],"date-time":"2024-09-19T00:00:00Z","timestamp":1726704000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2025,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:label\/><jats:p>The lungs are integral to immune defense, and inborn errors of immunity (IEI) often manifest as lung disease. Lung complications of IEI can involve the airways, alveolar spaces, interstitium, vasculature, and pleura. Accurate identification of these lung disease patterns requires a thorough clinical history, physical examination, and high\u2010resolution computed tomography (HRCT), as lung imaging patterns guide further respiratory and immunological evaluations. Respiratory assessment may also include pulmonary function tests, bronchoscopy with bronchoalveolar lavage, and, in some cases, lung biopsy. Additionally, molecular diagnosis of underlying immune defects, typically through comprehensive clinical phenotyping, functional immune studies, and genetic testing, is crucial for informing patient management and guiding targeted therapies. Importantly, given the complexity of IEI, a multidisciplinary approach is necessary. Furthermore, ongoing research is required to refine therapies and improve outcomes for lung complications.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.27258","type":"journal-article","created":{"date-parts":[[2024,9,19]],"date-time":"2024-09-19T07:47:15Z","timestamp":1726732035000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["The lung in inborn errors of immunity"],"prefix":"10.1002","volume":"60","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3870-4102","authenticated-orcid":false,"given":"Sonia","family":"Restrepo\u2010Gualteros","sequence":"first","affiliation":[{"name":"School of Medicine Universidad Nacional de Colombia Bogot\u00e1 Colombia"},{"name":"Department of Pediatrics Fundaci\u00f3n Santa F\u00e9 de Bogot\u00e1 Bogot\u00e1 Colombia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8621-6109","authenticated-orcid":false,"given":"Gustavo","family":"Nino","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Sleep Medicine, Children's National Hospital George Washington University Washington District of Columbia USA"},{"name":"Center for Genetic Medicine Research Children's Research Institute Washington District of Columbia USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5494-5841","authenticated-orcid":false,"given":"Maria J.","family":"Gutierrez","sequence":"additional","affiliation":[{"name":"Division of Pediatric Allergy, Immunology and Rheumatology Johns Hopkins University School of Medicine Baltimore Maryland USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2024,9,19]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.2308511120"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2022.08.024"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-022-01289-3"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1080\/1744666X.2020.1801422"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1177\/11795565231196431"}],"container-title":["Pediatric Pulmonology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.27258","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,3,29]],"date-time":"2025-03-29T21:00:08Z","timestamp":1743282008000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ppul.27258"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,9,19]]},"references-count":5,"journal-issue":{"issue":"S1","published-print":{"date-parts":[[2025,3]]}},"alternative-id":["10.1002\/ppul.27258"],"URL":"https:\/\/doi.org\/10.1002\/ppul.27258","archive":["Portico"],"relation":{},"ISSN":["8755-6863","1099-0496"],"issn-type":[{"type":"print","value":"8755-6863"},{"type":"electronic","value":"1099-0496"}],"subject":[],"published":{"date-parts":[[2024,9,19]]},"assertion":[{"value":"2024-08-28","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-09-03","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-09-19","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}