{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,6]],"date-time":"2026-05-06T06:32:24Z","timestamp":1778049144611,"version":"3.51.4"},"reference-count":23,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2020,8,10]],"date-time":"2020-08-10T00:00:00Z","timestamp":1597017600000},"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 Transplantation"],"published-print":{"date-parts":[[2021,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>DC is caused by defects at the level of telomere maintenance, and cells from patients with this disease have abnormally short telomeres and show premature senescence. One consequence of DC is bone marrow failure. Thus, patients with DC often require HSCT. However, HSCT does not ameliorate other DC\u2010related manifestations. In fact, HSCT can accelerate organ dysfunction due to treatment\u2010related complications, and solid organ transplantation is required in some patients with DC. In this report, we describe the clinical course of a 5\u2010year\u2010old boy who was transferred to our hospital because of progressive dyspnea, 2\u00a0years after HSCT. At admission, he had tachypnea and hypoxemia. A liver biopsy was performed for suspected HPS caused by PH, and LT was considered. Eventually, his hypoxemia worsened, and he was transferred to a PICU and started on VA ECMO. He subsequently underwent a CLLT. ECMO was stopped on post\u2010operative day 12, extubation was achieved on post\u2010operative day 29, and the patient recovered well from the surgery. Our results show that CLLT could be a life\u2010saving treatment option for DC patients with very severe HPS in whom a poor outcome is expected after LT.<\/jats:p>","DOI":"10.1111\/petr.13802","type":"journal-article","created":{"date-parts":[[2020,8,10]],"date-time":"2020-08-10T23:19:43Z","timestamp":1597101583000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Combined lung and liver transplantation for noncirrhotic portal hypertension with severe hepatopulmonary syndrome in a patient with dyskeratosis congenita"],"prefix":"10.1111","volume":"25","author":[{"given":"Sohyun","family":"Shin","sequence":"first","affiliation":[{"name":"Department of Pediatrics Seoul National University Hospital Seoul Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7817-8728","authenticated-orcid":false,"given":"Dong In","family":"Suh","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Seoul National University Hospital Seoul Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0407-7828","authenticated-orcid":false,"given":"Jung Min","family":"Ko","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Seoul National University Hospital Seoul Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8113-1384","authenticated-orcid":false,"given":"June Dong","family":"Park","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Seoul National University Hospital Seoul Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7806-8759","authenticated-orcid":false,"given":"Jeong\u2010Moo","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Surgery Seoul National University Hospital Seoul Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5467-425X","authenticated-orcid":false,"given":"Nam\u2010Joon","family":"Yi","sequence":"additional","affiliation":[{"name":"Department of Surgery Seoul National University Hospital Seoul Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9006-4881","authenticated-orcid":false,"given":"Young Tae","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Thoracic and Cardiovascular Surgery Seoul National University Hospital Seoul Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9625-2672","authenticated-orcid":false,"given":"Samina","family":"Park","sequence":"additional","affiliation":[{"name":"Department of Thoracic and Cardiovascular Surgery Seoul National University Hospital Seoul 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