{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T08:53:46Z","timestamp":1791363226175,"version":"4.3.1"},"reference-count":23,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2025,9,19]],"date-time":"2025-09-19T00:00:00Z","timestamp":1758240000000},"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":[[2025,11]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Coats plus syndrome (CPS) is a rare telomere biology disorder (TBD) linked to mutations in the CTC1\u2010STN1\u2010TEN1 complex and characterized by cerebroretinal microangiopathy, bone marrow failure, and liver disease, often progressing to hepatopulmonary syndrome (HPS).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case Presentation<\/jats:title>\n                    <jats:p>\n                      The case is a 16\u2010year\u2010old boy clinically diagnosed with CPS at age 7. He developed exertional dyspnea at 14 with 93% of resting oxygen saturation (SpO\n                      <jats:sub>2<\/jats:sub>\n                      ), 70\u2009mmHg of partial pressure of oxygen (PaO\n                      <jats:sub>2<\/jats:sub>\n                      ), and 22.9\u2009mmHg of alveolar\u2013arterial oxygen difference (A\u2010aDO\n                      <jats:sub>2<\/jats:sub>\n                      ) under room air, prompting home oxygen therapy (HOT). Imaging and liver biopsy showed significant collateral circulation and portal vein fibrosis and dilation without cirrhosis or pulmonary fibrosis. HPS was diagnosed by positive microbubble contrast echocardiography and 15.7% of shunt fraction in pulmonary ventilation\u2013perfusion scintigraphy. Due to the rapid progression of HPS, he underwent living\u2010donor LT. The postoperative disease course was good, except for acute liver rejection, and the patient was discharged on postoperative day 40. One month after transplantation, echocardiography showed a negative bubble study, pulmonary scintigraphy revealed an improved shunt fraction of 10.2%, resting SpO\n                      <jats:sub>2<\/jats:sub>\n                      under room air was 100%, and he is doing well without the recurrence of HPS.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Historically, LT for HPS patients with TBDs was avoided because of uncertain prognosis and potential disease progression in other organs. However, early\u2010stage LT in TBDs may be preferable and reduce the complexity of LT. Before irreversible changes in intrapulmonary blood vessels occur, early hypoxemia monitoring and regular imaging are essential to ensure timely LT for HPS.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/petr.70184","type":"journal-article","created":{"date-parts":[[2025,9,20]],"date-time":"2025-09-20T04:29:41Z","timestamp":1758342581000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Successful Liver Transplantation for Coats Plus Syndrome With Hepatopulmonary Syndrome: A Case Report and Literature Review"],"prefix":"10.1111","volume":"29","author":[{"given":"Masato","family":"Kojima","sequence":"first","affiliation":[{"name":"Organ Transplantation Center National Center for Child Health and Development  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3031-7319","authenticated-orcid":false,"given":"Seisuke","family":"Sakamoto","sequence":"additional","affiliation":[{"name":"Organ Transplantation Center National Center for Child Health and Development  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2441-5158","authenticated-orcid":false,"given":"Hajime","family":"Uchida","sequence":"additional","affiliation":[{"name":"Organ Transplantation Center National Center for Child Health and Development  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3669-5548","authenticated-orcid":false,"given":"Yusuke","family":"Yanagi","sequence":"additional","affiliation":[{"name":"Organ Transplantation Center National Center for Child Health and Development  Tokyo 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Development  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4684-0446","authenticated-orcid":false,"given":"Akinari","family":"Fukuda","sequence":"additional","affiliation":[{"name":"Organ Transplantation Center National Center for Child Health and Development  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1651-0430","authenticated-orcid":false,"given":"Mureo","family":"Kasahara","sequence":"additional","affiliation":[{"name":"Organ Transplantation Center National Center for Child Health and Development  Tokyo 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