{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,31]],"date-time":"2025-10-31T14:30:08Z","timestamp":1761921008239,"version":"3.37.3"},"reference-count":8,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2022,3,17]],"date-time":"2022-03-17T00:00:00Z","timestamp":1647475200000},"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":[[2022,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Pulmonary veno\u2010occlusive disease (PVOD) and idiopathic\/heritable pulmonary arterial hypertension (I\/HPAH) cause progressive PH on the distinct genetic impact. A 29\u2010month\u2010old boy presented with a loss of consciousness. He had severe PH refractory to pulmonary vasodilators. Hypoxemia and ground\u2010glass opacity on the chest computed tomography were present, and significant pulmonary edema developed after the introduction of continuous intravenous prostaglandin I<jats:sub>2<\/jats:sub>. Based on the clinical diagnosis of PVOD, he underwent a single living\u2010donor lobar lung transplantation with the right lower lobe of his mother. The pathological findings of his explanted lung showed intimal thickening and luminal narrowing of the pulmonary vein. A genetic test revealed a novel heterozygous splice acceptor variant (c.77\u20102A&gt;C) in <jats:italic>BMPR2<\/jats:italic>, which is typically associated with I\/HPAH. This is the first pediatric case of PVOD with <jats:italic>BMPR2<\/jats:italic> variant, supporting the concept that I\/HPAH and PVOD are part of a spectrum of pulmonary vascular disease.<\/jats:p>","DOI":"10.1002\/ppul.25877","type":"journal-article","created":{"date-parts":[[2022,3,1]],"date-time":"2022-03-01T08:49:29Z","timestamp":1646124569000},"page":"1366-1369","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Pediatric pulmonary veno\u2010occlusive disease associated with a novel <i>BMPR2<\/i> variant"],"prefix":"10.1002","volume":"57","author":[{"given":"Wataru","family":"Takemori","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Graduate School of Medical Sciences Kyushu University  Fukuoka 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