{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T19:26:08Z","timestamp":1791228368728,"version":"4.1.0"},"reference-count":22,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2023,5,4]],"date-time":"2023-05-04T00:00:00Z","timestamp":1683158400000},"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 Anesthesia"],"published-print":{"date-parts":[[2023,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Ornithine transcarbamylase deficiency is an X\u2010linked genetic disorder that induces accumulation of ammonia in the liver and is the most common urea cycle disorder. The clinical manifestation of ornithine transcarbamylase deficiency is hyperammonemia that causes irreversible neurological damage. Liver transplantation is a curative therapy for ornithine transcarbamylase deficiency. The aim of this study is to suggest, from our previous experience, an anesthesia management protocol of liver transplantation for ornithine transcarbamylase deficiency, particularly focused on liver transplantation for cases with uncontrolled hyperammonemia.<\/jats:p><\/jats:sec><jats:sec><jats:title>Method<\/jats:title><jats:p>We retrospectively reviewed our anesthesia\u2010related experience in all cases of liver transplantation for ornithine transcarbamylase deficiency in our center.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Twenty\u2010nine liver transplantation cases for ornithine transcarbamylase deficiency were found between November 2005 and March 2021 in our center.<\/jats:p><jats:p>Of these, 25 cases were stable through the perioperative period. However, 2 cases with carrier donor graft had hyperammonemia after liver transplantation. Another two cases had uncontrolled hyperammonemia before liver transplantation, even with continuous hemodialysis. They underwent life\u2010saving liver transplantation. Their metabolic status stabilized after the anhepatic phase.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Liver transplantation for cases with uncontrolled hyperammonemia can be performed with proper management. Second, liver transplantation with carrier donors should be avoided because of the risk of postoperative recurrence.<\/jats:p><\/jats:sec>","DOI":"10.1111\/pan.14691","type":"journal-article","created":{"date-parts":[[2023,5,4]],"date-time":"2023-05-04T07:29:41Z","timestamp":1683185381000},"page":"620-630","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Anesthesia management protocol for liver transplantation as treatment for ornithine transcarbamylase deficiency"],"prefix":"10.1111","volume":"33","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8165-7068","authenticated-orcid":false,"given":"Chiaki","family":"Baba","sequence":"first","affiliation":[{"name":"Division of Anesthesia, Department of Critical Care and Anesthesia National Center for Child Health and Development Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sho","family":"Yukimasa","sequence":"additional","affiliation":[{"name":"Division of Anesthesia, Department of Critical Care and Anesthesia National Center for Child Health and Development Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Risa","family":"Yasuno","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology Tokyo Women's Medical University Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroki","family":"Ichiyanagi","sequence":"additional","affiliation":[{"name":"Division of Anesthesia, Department of Critical Care and Anesthesia National Center for Child Health and Development Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jun","family":"Ninagawa","sequence":"additional","affiliation":[{"name":"Division of Anesthesia, Department of Critical Care and Anesthesia National Center for Child Health and Development Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shugo","family":"Kasuya","sequence":"additional","affiliation":[{"name":"Division of Anesthesia, Department of Critical Care and Anesthesia National Center for Child Health and Development Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mureo","family":"Kasahara","sequence":"additional","affiliation":[{"name":"Center for Organ Transplantation, National Center for Child Health and Development Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Reiko","family":"Horikawa","sequence":"additional","affiliation":[{"name":"Division of Endocrinology and Metabolism National Center for Child Health and Development Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasuko","family":"Nagasaka","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology Tokyo Women's Medical University Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasuyuki","family":"Suzuki","sequence":"additional","affiliation":[{"name":"Division of Anesthesia, Department of Critical Care and Anesthesia National Center for Child Health and Development Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,5,4]]},"reference":[{"key":"e_1_2_9_2_1","volume-title":"Ornithine transcarbamylase deficiency. In Stat Pearls","author":"Donovan K","year":"2021"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/1096-8628(20000814)93:4<313::AID-AJMG11>3.0.CO;2-M"},{"key":"e_1_2_9_4_1","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1016\/S0065-3101(24)00072-0","article-title":"Urea cycle disorders: diagnosis, pathophysiology, and therapy","volume":"43","author":"Brusilow SW","year":"1996","journal-title":"Adv Pediatr"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11011-018-0259-6"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/jhg.2015.54"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199609193351204"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.gene.2018.09.026"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1067\/mpd.2001.111837"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/petr.12196"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/petr.12741"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1460-9592.2010.03322.x"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1460-9592.2005.01695.x"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/lt.20587"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-3046.2011.01494.x"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/petr.12848"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1034\/j.1399-0004.1998.5440415.x"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-3046.2012.01716.x"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1002\/jimd.12384"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/ped.12439"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1242\/dmm.047134"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/0065-2571(66)90025-2"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1053\/jhep.2003.50052"}],"container-title":["Pediatric Anesthesia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pan.14691","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,10,19]],"date-time":"2024-10-19T19:28:12Z","timestamp":1729366092000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/pan.14691"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,5,4]]},"references-count":22,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2023,8]]}},"alternative-id":["10.1111\/pan.14691"],"URL":"https:\/\/doi.org\/10.1111\/pan.14691","archive":["Portico"],"relation":{},"ISSN":["1155-5645","1460-9592"],"issn-type":[{"value":"1155-5645","type":"print"},{"value":"1460-9592","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,5,4]]},"assertion":[{"value":"2021-12-14","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-04-20","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-05-04","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}