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We investigated the clinical manifestations, treatment, and prognoses of 177 patients with <jats:styled-content style=\"fixed-case\">UCD<\/jats:styled-content>s who were evaluated and treated from January 1999 to March 2009 in Japan, using a questionnaire survey. Among these 177 patients, 42 (seven with carbamoyl phosphate synthetase 1 deficiency, 27 with ornithine transcarbamylase deficiency, seven with argininosuccinate synthetase deficiency, and one with arginase 1 deficiency) underwent living\u2010donor <jats:styled-content style=\"fixed-case\">LT<\/jats:styled-content>. Although this study was retrospective and included limited neurodevelopmental information before and after <jats:styled-content style=\"fixed-case\">LT<\/jats:styled-content>, we evaluated whether <jats:styled-content style=\"fixed-case\">LT<\/jats:styled-content> could improve neurodevelopmental outcomes in patients with <jats:styled-content style=\"fixed-case\">UCD<\/jats:styled-content>s. The neurodevelopmental outcomes of patients with a <jats:styled-content style=\"fixed-case\">MAC<\/jats:styled-content> of &lt;300\u00a0\u03bcmol\/L at the time of onset were not significantly different between the <jats:styled-content style=\"fixed-case\">LT<\/jats:styled-content> and non\u2010<jats:styled-content style=\"fixed-case\">LT<\/jats:styled-content> groups (<jats:italic>P<\/jats:italic>=.222). <jats:styled-content style=\"fixed-case\">LT<\/jats:styled-content> may have prevented further neurodevelopmental complications in children with <jats:styled-content style=\"fixed-case\">MAC<\/jats:styled-content> \u2265300 \u03bcmol\/L (<jats:italic>P<\/jats:italic>=.008) compared with non\u2010transplant management. Therefore, Liver transplant should be considered in patients with <jats:styled-content style=\"fixed-case\">UCD<\/jats:styled-content> with a <jats:styled-content style=\"fixed-case\">MAC<\/jats:styled-content> of \u2265300\u00a0\u03bcmol\/L at the time of disease onset.<\/jats:p>","DOI":"10.1111\/petr.12987","type":"journal-article","created":{"date-parts":[[2017,6,13]],"date-time":"2017-06-13T04:02:45Z","timestamp":1497326565000},"source":"Crossref","is-referenced-by-count":28,"title":["Liver transplantation may prevent neurodevelopmental deterioration in high\u2010risk patients with urea cycle disorders"],"prefix":"10.1111","volume":"21","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1328-6696","authenticated-orcid":false,"given":"Jun","family":"Kido","sequence":"first","affiliation":[{"name":"Department of Pediatrics Graduate School of Medical Sciences Kumamoto University  Kumamoto 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