{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,10]],"date-time":"2026-04-10T06:23:51Z","timestamp":1775802231292,"version":"3.50.1"},"reference-count":26,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2021,10,23]],"date-time":"2021-10-23T00:00:00Z","timestamp":1634947200000},"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":[[2022,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>The native liver of patients with maple syrup urine disease (MSUD) (1st recipients) can be used as a graft for non\u2010MSUD patients with end\u2010stage liver disease (2nd recipients). This study aimed to demonstrate the optimal operational procedures and the long\u2010term outcomes of 2nd recipients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Six 2nd recipients of living donor domino liver transplantation (LD\u2010DLT) (age: 42.5 [22\u2013169] months at DLT) received a native liver as a graft from an MSUD patient at our hospital between June 2014 and April 2020. We reviewed the operational procedures and outcomes of 2nd recipients after LD\u2010DLT.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The 2nd recipients' original diseases included biliary atresia, congenital hepatic fibrosis, congenital protein C deficiency, familial hypercholesterolemia, hepatoblastoma, and mitochondrial hepatopathy. Five of the six recipients had a whole liver and one had a right lobe graft. The site at which the vessels of the MSUD liver were dissected prioritized the safety of the 1st recipient. At the end of follow\u2010up, all recipients were doing well without surgical complications. The mean serum amino acid values of the 2nd recipients did not exceed the upper limit of the reference values during the long\u2010term observation period. All patients showed normal growth while maintaining the same z\u2010score of height and weight after LD\u2010DLT as the preoperative level.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The liver of patients with MSUD can be used safely without concern regarding long\u2010term complications or <jats:italic>de novo<\/jats:italic> MSUD development. LD\u2010DLT using the MSUD liver can expand the donor pool as an alternative graft in pediatric LT.<\/jats:p><\/jats:sec>","DOI":"10.1111\/petr.14174","type":"journal-article","created":{"date-parts":[[2021,10,23]],"date-time":"2021-10-23T15:56:03Z","timestamp":1635004563000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Surgical technique and the long\u2010term outcomes of pediatric living donor domino liver transplantation from patients with maple syrup urine disease"],"prefix":"10.1111","volume":"26","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6985-945X","authenticated-orcid":false,"given":"Seiichi","family":"Shimizu","sequence":"first","affiliation":[{"name":"Organ Transplantation Center National Center for Child Health and Development Tokyo 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