{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,16]],"date-time":"2026-05-16T03:41:10Z","timestamp":1778902870435,"version":"3.51.4"},"reference-count":17,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2017,6,14]],"date-time":"2017-06-14T00:00:00Z","timestamp":1497398400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Practical Research Project for Rare\/Intractable Diseases"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Transplantation"],"published-print":{"date-parts":[[2017,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Classical <jats:styled-content style=\"fixed-case\">MSUD<\/jats:styled-content> is often fatal without appropriate medical interventions because of metabolic crisis. There are numerous reports suggesting the therapeutic potential of deceased donor liver transplantation for <jats:styled-content style=\"fixed-case\">MSUD<\/jats:styled-content>. However, the usefulness of <jats:styled-content style=\"fixed-case\">LDLT<\/jats:styled-content> for <jats:styled-content style=\"fixed-case\">MSUD<\/jats:styled-content> is unknown. We report a case of classical <jats:styled-content style=\"fixed-case\">MSUD<\/jats:styled-content>, which was successfully managed by <jats:styled-content style=\"fixed-case\">LDLT<\/jats:styled-content> from the patient's father at 1\u00a0year of age. Abnormal brain findings, which were cured with effective treatment, gradually disappeared after <jats:styled-content style=\"fixed-case\">LDLT<\/jats:styled-content>. The patient then developed normally. Findings from this case suggest the importance of <jats:styled-content style=\"fixed-case\">LDLT<\/jats:styled-content> for maintaining low leucine levels and subsequent normal neurological development. Although <jats:styled-content style=\"fixed-case\">LDLT<\/jats:styled-content> involves a modest surgical insult, <jats:styled-content style=\"fixed-case\">LDLT<\/jats:styled-content> with a related donor achieves acceptable leucine levels for life.<\/jats:p>","DOI":"10.1111\/petr.12948","type":"journal-article","created":{"date-parts":[[2017,6,14]],"date-time":"2017-06-14T08:26:42Z","timestamp":1497428802000},"source":"Crossref","is-referenced-by-count":8,"title":["A case of classical maple syrup urine disease that was successfully managed by living donor liver transplantation"],"prefix":"10.1111","volume":"21","author":[{"given":"Chika","family":"Takano","sequence":"first","affiliation":[{"name":"Department of Pediatrics and Child Health Nihon University School of Medicine  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1324-0321","authenticated-orcid":false,"given":"Mika","family":"Ishige","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Child Health Nihon University School of Medicine  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Erika","family":"Ogawa","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Child Health Nihon University School of Medicine  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiromi","family":"Usui","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Child Health Nihon University School of Medicine  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Reiko","family":"Kagawa","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Hiroshima University Graduate School of Biomedical &amp; Health Sciences  Hiroshima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Go","family":"Tajima","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Hiroshima University Graduate School of Biomedical &amp; Health Sciences  Hiroshima Japan"},{"name":"Division of Neonatal Screening, Research Institute National Center for Child Health and Development  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ryoji","family":"Fujiki","sequence":"additional","affiliation":[{"name":"Department of Technology Development Kazusa DNA Research Institute  Chiba Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Toshiyuki","family":"Fukao","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Graduate School of Medicine Gifu University  Gifu Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Koichi","family":"Mizuta","sequence":"additional","affiliation":[{"name":"Transplant Surgery Division Department of Surgery Jichi Medical University  Tochigi Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tatsuo","family":"Fuchigami","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Child Health Nihon University School of Medicine  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shori","family":"Takahashi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Child Health Nihon University School of Medicine  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2017,6,14]]},"reference":[{"key":"e_1_2_7_2_1","unstructured":"DavidT ChuangVES.Maple Syrup Urine Disease (Branched\u2010Chain Ketoaciduria).The Online Metabolic&Molecular Bases of Inherited Disease: Part 8: AMINO ACIDS:1\u201074."},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-6143.2005.01209.x"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-3046.2010.01411.x"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2011.06.033"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000000469"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.transproceed.2012.04.031"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/petr.12446"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1590\/1414-431X20143830"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/petr.12447"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/petr.12738"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/petr.12736"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.rct.0000164667.65648.72"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/8904_2016_532"},{"key":"e_1_2_7_15_1","volume-title":"GeneReviews(R)","author":"Strauss KA","year":"1993"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.4103\/2249-4847.116411"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/lt.20800"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2016.01.005"}],"container-title":["Pediatric Transplantation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fpetr.12948","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/petr.12948","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,1]],"date-time":"2023-10-01T22:57:18Z","timestamp":1696201038000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/petr.12948"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,6,14]]},"references-count":17,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2017,8]]}},"alternative-id":["10.1111\/petr.12948"],"URL":"https:\/\/doi.org\/10.1111\/petr.12948","archive":["Portico"],"relation":{},"ISSN":["1397-3142","1399-3046"],"issn-type":[{"value":"1397-3142","type":"print"},{"value":"1399-3046","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,6,14]]},"article-number":"e12948"}}