{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,26]],"date-time":"2026-06-26T11:49:18Z","timestamp":1782474558726,"version":"3.54.5"},"reference-count":40,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2026,6,15]],"date-time":"2026-06-15T00:00:00Z","timestamp":1781481600000},"content-version":"vor","delay-in-days":14,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,6,1]],"date-time":"2026-06-01T00:00:00Z","timestamp":1780272000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2026,6]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>\n                      Lung disease due to\n                      <jats:italic>ABCA3<\/jats:italic>\n                      variants and resultant surfactant dysfunction is an autosomal recessive disease. However, monoallelic\n                      <jats:italic>ABCA3<\/jats:italic>\n                      variants are overrepresented in term or late\u2010preterm infants with non\u2010fatal neonatal respiratory distress syndrome (RDS). The childhood respiratory outcomes of infants with monoallelic\n                      <jats:italic>ABCA3<\/jats:italic>\n                      variants who present with neonatal RDS are unknown. In this study, we report the respiratory outcomes beyond neonatal hospitalization (median follow\u2010up 1 year [range 1\u20139.4 years]) for infants with monoallelic\n                      <jats:italic>ABCA3<\/jats:italic>\n                      variants and neonatal RDS.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      Participants with monoallelic\n                      <jats:italic>ABCA3<\/jats:italic>\n                      variants classified as pathogenic, likely pathogenic, or of uncertain significance with neonatal RDS identified in 3 cohorts (ChILD Registry\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20099, disease\u2010based cohort\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200919, and nested case\u2010control\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200920) were included in this retrospective, descriptive study. The primary outcome was duration of supplemental oxygen use. Secondary outcomes included respiratory morbidities after NICU discharge.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Of 48 infants who met inclusion criteria, 8 (17%) were discharged home on supplemental oxygen, including two who required home invasive mechanical ventilation. The\n                      <jats:italic>ABCA3<\/jats:italic>\n                      pathogenic variant p.Glu292Val was the most common variant identified in the infants who required oxygen beyond NICU discharge (6 of 8). Oxygen was discontinued by 10 months of age in 50% of infants discharged from birth hospitalization on oxygen. Respiratory morbidities, including hospitalizations (62% vs. 8%,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.005) and emergency department (ED) visits (71% vs. 35%,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.01), were more common among participants discharged home on oxygen compared to those discharged home without oxygen.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>\n                      These findings suggest that infants with monoallelic\n                      <jats:italic>ABCA3<\/jats:italic>\n                      variants and neonatal RDS may require prolonged home oxygen or mechanical ventilation and experience increased risk for respiratory morbidities, including subsequent hospitalization and ED visits.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.71695","type":"journal-article","created":{"date-parts":[[2026,6,15]],"date-time":"2026-06-15T13:20:27Z","timestamp":1781529627000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Respiratory Outcomes in Children With Neonatal Respiratory Distress Syndrome and Monoallelic\n                    <i>ABCA3<\/i>\n                    Variants"],"prefix":"10.1002","volume":"61","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5134-3845","authenticated-orcid":false,"given":"Velda Ocasio","family":"Ram\u00edrez","sequence":"first","affiliation":[{"name":"Eudowood Division of Pediatric Respiratory Sciences Johns Hopkins University School of Medicine Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer A.","family":"Wambach","sequence":"additional","affiliation":[{"name":"Edward Mallinckrodt Department of Pediatrics Washington University School of Medicine, St. Louis Children's Hospital Saint Louis Missouri USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rebekah J.","family":"Nevel","sequence":"additional","affiliation":[{"name":"Child Health, Division of Pediatric Pulmonary Medicine University of Missouri Columbia Missouri USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Voss","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Pulmonary and Sleep Medicine, Children's Hospital of Philadelphia University of Pennsylvania Perelman School of Medicine Philadelphia Pennsylvania USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0009-0194-3207","authenticated-orcid":false,"given":"Daniel","family":"Craven","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonology, Rainbow Babies and Children's Hospital, Cleveland Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alicia","family":"Casey","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Medicine, Boston Children's Hospital Harvard Medical School, Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Devaney","family":"Camburn","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Pulmonary and Sleep Medicine, Children's Hospital of Philadelphia University of Pennsylvania Perelman School of Medicine Philadelphia Pennsylvania USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven K.","family":"Brennan","sequence":"additional","affiliation":[{"name":"Edward Mallinckrodt Department of Pediatrics Washington University School of Medicine, St. Louis Children's Hospital Saint Louis Missouri USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Whitney Bour","family":"Eldridge","sequence":"additional","affiliation":[{"name":"Edward Mallinckrodt Department of Pediatrics Washington University School of Medicine, St. Louis Children's Hospital Saint Louis Missouri USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F. Sessions","family":"Cole","sequence":"additional","affiliation":[{"name":"Edward Mallinckrodt Department of Pediatrics Washington University School of Medicine, St. Louis Children's Hospital Saint Louis Missouri USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gail H.","family":"Deutsch","sequence":"additional","affiliation":[{"name":"Pathology, Seattle Children's Hospital Seattle Washington USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael W.","family":"Kuzniewicz","sequence":"additional","affiliation":[{"name":"Division of Research, Kaiser Permanente Northern California Oakland California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3297-8532","authenticated-orcid":false,"given":"Lisa R.","family":"Young","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Pulmonary and Sleep Medicine, Children's Hospital of Philadelphia University of Pennsylvania Perelman School of Medicine Philadelphia Pennsylvania USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0540-8083","authenticated-orcid":false,"given":"Lawrence","family":"Nogee","sequence":"additional","affiliation":[{"name":"Eudowood Neonatal Pulmonary Division, Department of Pediatrics Johns Hopkins University School of Medicine Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9167-6994","authenticated-orcid":false,"given":"S. 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