{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T15:07:17Z","timestamp":1780412837376,"version":"3.54.1"},"reference-count":26,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2018,5,3]],"date-time":"2018-05-03T00:00:00Z","timestamp":1525305600000},"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":[[2018,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Citrullinemia or ASS deficiency in its classical form presents in the neonatal period with poor feeding, hyperammonemia, encephalopathy, seizures, and if untreated can be fatal. Despite advances in medical therapy, neurocognitive outcomes remain suboptimal. LT has emerged as a potential management option. A retrospective single\u2010center review identified 7 children with a median age of 1.1\u00a0years (range, 0.6\u20105.8) at referral. Five children presented clinically, and 2 were treated prospectively from birth due to positive family history. All patients received standard medical and dietary therapy prior to <jats:styled-content style=\"fixed-case\">LT<\/jats:styled-content>. The indications for <jats:styled-content style=\"fixed-case\">LT<\/jats:styled-content> were frequent metabolic decompensations in 4, elective in 2, and ALF in 1. The median age at <jats:styled-content style=\"fixed-case\">LT<\/jats:styled-content> was 2.4\u00a0years (range, 1.3\u20106.5). Five patients received 6 left lateral segment grafts, one a live unrelated donor left lateral segment as an APOLT graft, and one a cadaveric whole liver graft as <jats:styled-content style=\"fixed-case\">APOLT<\/jats:styled-content>. One child required retransplantation due to hepatic artery thrombosis. Graft and patient survival were 86% and 100%, respectively. Median follow\u2010up is 3.1\u00a0years (range, 0.1\u20104.1), and the median age at follow\u2010up is 5.5\u00a0years (range, 4.0\u20109.8). There have been no metabolic decompensations in 6 children, while 1 patient (with <jats:styled-content style=\"fixed-case\">APOLT<\/jats:styled-content>) developed asymptomatic hyperammonemia with no clinical or histological signs of liver injury, requiring additional medical therapy. Our medium\u2010term experience following <jats:styled-content style=\"fixed-case\">LT<\/jats:styled-content> in citrullinemia is favorable, demonstrating a positive transformation of the clinical phenotype.<\/jats:p>","DOI":"10.1111\/petr.13191","type":"journal-article","created":{"date-parts":[[2018,5,4]],"date-time":"2018-05-04T06:25:13Z","timestamp":1525415113000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Liver transplantation for neonatal\u2010onset citrullinemia"],"prefix":"10.1111","volume":"22","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4007-6558","authenticated-orcid":false,"given":"Roshni","family":"Vara","sequence":"first","affiliation":[{"name":"Department of Inherited Metabolic Disease Evelina London Children's Hospital  London UK"},{"name":"Paediatric Liver, GI and Nutrition Centre King's College Hospital  London 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