{"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":1791228368778,"version":"4.1.0"},"reference-count":19,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"1","license":[{"start":{"date-parts":[[2013,11,29]],"date-time":"2013-11-29T00:00:00Z","timestamp":1385683200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2014,1,1]],"date-time":"2014-01-01T00:00:00Z","timestamp":1388534400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2014,1]]},"abstract":"<jats:sec>\n            <jats:title\/>\n            <jats:p>Decision making concerning liver transplantation is unique for children with urea cycle disorders (UCDs) and organic acidemias (OAs) because of their immediate high priority on the waiting list, which is not related to the severity of their disease. There are limited national outcome data on which recommendations about liver transplantation for UCDs or OAs can be based. This study was a retrospective analysis of United Network for Organ Sharing data for liver recipients who underwent transplantation at an age &lt; 18 years in 2002-2012. Repeat transplants were excluded. Among the pediatric liver transplants, 5.4% were liver-only for UCDs\/OAs. The proportion of transplants for UCDs\/OAs increased from 4.3% in 2002-2005 to 7.4% in 2010-2012 (<jats:italic toggle=\"yes\">P<\/jats:italic> &lt; 0.001). Ninety-six percent were deceased donor transplants, and 59% of these patients underwent transplantation at &lt;2 years of age. Graft survival improved as the age at transplant increased (<jats:italic toggle=\"yes\">P<\/jats:italic> = 0.04). Within 5 years after transplantation, the graft survival rate was 78% for children &lt; 2 years old at transplant and 88% for children \u2265 2 years old at transplant (<jats:italic toggle=\"yes\">P<\/jats:italic> = 0.06). Vascular thrombosis caused 44% of the graft losses, and 65% of these losses occurred in children &lt; 2 years old. Patient survival also improved as the age at transplant increased: the 5-year patient survival rate was 88% for children with UCDs\/OAs who were &lt;2 years old at transplant and 99% for children who were \u22652 years old at transplant (<jats:italic toggle=\"yes\">P<\/jats:italic> = 0.006). At the last-follow-up (54 \u00b1 34.4 months), children who underwent transplantation for UCDs\/OAs were more likely to have cognitive and motor delays than children who underwent transplantation for other indications. Cognitive and motor delays for children with UCDs\/OAs were associated with metabolic disorders, but they were not predicted by age or weight at transplant, sex, ethnicity, liver graft type (split versus whole), or hospitalization at transplant in univariate and multivariate analyses. In conclusion, most liver transplants for UCDs\/OAs occur in early childhood. Further research on the benefits of early transplantation for patients with UCDs\/OAs is needed because a younger age may increase posttransplant morbidity. <jats:italic toggle=\"yes\">Liver Transpl 20:89-99, 2014<\/jats:italic>. \u00a9 2013 AASLD.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1002\/lt.23765","type":"journal-article","created":{"date-parts":[[2013,10,17]],"date-time":"2013-10-17T18:47:13Z","timestamp":1382035633000},"page":"89-99","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":58,"title":["Pediatric liver transplantation for urea cycle disorders and organic acidemias: United Network for Organ Sharing data for 2002-2012"],"prefix":"10.1097","volume":"20","author":[{"given":"Emily R.","family":"Perito","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sue","family":"Rhee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John Paul","family":"Roberts","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philip","family":"Rosenthal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"issue":"suppl 1","key":"R4-12-20250124","first-page":"112118","article-title":"Liver transplantation for the treatment of urea cycle disorders.","volume":"21","author":"Whitington","year":"1998","journal-title":"J Inherit Metab Dis"},{"key":"R5-12-20250124","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1186\/1750-1172-7-32","article-title":"Suggested guidelines for the diagnosis and management of urea cycle disorders.","volume":"7","author":"Haberle","year":"2012","journal-title":"Orphanet J Rare Dis"},{"issue":"suppl 3","key":"R6-12-20250124","first-page":"S124S127","article-title":"Model for EndStage Liver Disease (MELD) exception for unusual metabolic liver diseases.","volume":"12","author":"McDiarmid","year":"2006","journal-title":"Liver Transpl"},{"key":"R7-12-20250124","doi-asserted-by":"crossref","first-page":"139144","DOI":"10.1016\/j.jpeds.2009.06.067","article-title":"An ethical and policy analysis of elective transplantation for metabolic conditions diagnosed by newborn screening.","volume":"156","author":"Ross","year":"2010","journal-title":"J Pediatr"},{"key":"R8-12-20250124","doi-asserted-by":"crossref","first-page":"796805","DOI":"10.1111\/j.1399-3046.2010.01339.x","article-title":"; for SPLIT Research Group. Liver transplantation in children with metabolic diseases: the Studies of Pediatric Liver Transplantation experience.","volume":"14","author":"Arnon","year":"2010","journal-title":"Pediatr Transplant"},{"key":"R9-12-20250124","doi-asserted-by":"crossref","first-page":"203205","DOI":"10.1016\/j.ymgme.2013.01.013","article-title":"Development of clinical guidelines for inborn errors of metabolism: commentary.","volume":"108","author":"Vockley","year":"2013","journal-title":"Mol Genet Metab"},{"key":"R10-12-20250124","doi-asserted-by":"crossref","first-page":"13321342","DOI":"10.1002\/lt.20587","article-title":"Current role of liver transplantation for the treatment of urea cycle disorders: a review of the worldwide English literature and 13 cases at Kyoto University.","volume":"11","author":"Morioka","year":"2005","journal-title":"Liver Transpl"},{"key":"R11-12-20250124","doi-asserted-by":"crossref","first-page":"334339","DOI":"10.1034\/j.1600-6143.2003.00058.x","article-title":"Liver transplantation in children with metabolic disorders in the United States.","volume":"3","author":"Kayler","year":"2003","journal-title":"Am J Transplant"},{"key":"R12-12-20250124","doi-asserted-by":"crossref","first-page":"27542763","DOI":"10.1111\/j.1600-6143.2005.01084.x","article-title":"Living donor liver transplantation for pediatric patients with inheritable metabolic disorders.","volume":"5","author":"Morioka","year":"2005","journal-title":"Am J Transplant"},{"key":"R13-12-20250124","doi-asserted-by":"crossref","first-page":"623628","DOI":"10.1097\/01.tp.0000167995.46778.72","article-title":"Living donor liver transplantation for noncirrhotic inheritable metabolic liver diseases: impact of the use of heterozygous donors.","volume":"80","author":"Morioka","year":"2005","journal-title":"Transplantation"},{"key":"R14-12-20250124","doi-asserted-by":"crossref","first-page":"390395","DOI":"10.1111\/j.1399-3046.2011.01494.x","article-title":"Living donor liver transplantation for ornithine transcarbamylase deficiency.","volume":"15","author":"Wakiya","year":"2011","journal-title":"Pediatr Transplant"},{"key":"R15-12-20250124","doi-asserted-by":"crossref","first-page":"E196E200","DOI":"10.1111\/j.1399-3046.2012.01716.x","article-title":"Living donor liver transplantation from an asymptomatic mother who was a carrier for ornithine transcarbamylase deficiency.","volume":"16","author":"Wakiya","year":"2012","journal-title":"Pediatr Transplant"},{"key":"R16-12-20250124","doi-asserted-by":"crossref","first-page":"721729","DOI":"10.1002\/lt.23667","article-title":"Deceased donor liver transplantation in infants and small children: are partial grafts riskier than whole organs?","volume":"19","author":"Cauley","year":"2013","journal-title":"Liver Transpl"},{"issue":"suppl 1","key":"R17-12-20250124","first-page":"S84S87","article-title":"Early orthotopic liver transplantation in urea cycle defects: follow up of a developmental outcome study.","volume":"100","author":"Campeau","year":"2010","journal-title":"Mol Genet Metab"},{"key":"R18-12-20250124","doi-asserted-by":"crossref","first-page":"e523e526","DOI":"10.1542\/peds.2004-0198","article-title":"Developmental outcomes with early orthotopic liver transplantation for infants with neonatalonset urea cycle defects and a female patient with lateonset ornithine transcarbamylase deficiency.","volume":"114","author":"McBride","year":"2004","journal-title":"Pediatrics"},{"key":"R19-12-20250124","first-page":"194203","article-title":"Paediatric liver transplantation for metabolic disorders. Part 1: liverbased metabolic disorders without liver lesions.","volume":"35","author":"Darwish","year":"2011","journal-title":"Clin Res Hepatol Gastroenterol"},{"key":"R20-12-20250124","doi-asserted-by":"crossref","first-page":"158167","DOI":"10.1111\/petr.12041","article-title":"Liver transplantation for urea cycle disorders in pediatric patients: a singlecenter experience.","volume":"17","author":"Kim","year":"2013","journal-title":"Pediatr Transplant"},{"key":"R21-12-20250124","doi-asserted-by":"crossref","first-page":"116121.e1.e1","DOI":"10.1016\/j.jpeds.2011.06.033","article-title":"Liver transplantation for classical maple syrup urine disease: longterm followup in 37 patients and comparative United Network for Organ Sharing experience.","volume":"160","author":"Mazariegos","year":"2012","journal-title":"J Pediatr"},{"key":"R22-12-20250124","first-page":"412422","article-title":"; for Studies of Pediatric Liver Transplantation (SPLIT) Research Group. The SPLIT research agenda 2013.","volume":"17","author":"Alonso","year":"2013","journal-title":"Pediatr Transplant"}],"container-title":["Liver Transplantation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Flt.23765","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/lt.23765","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/lt.23765","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.lww.com\/01445473-201401000-00012","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,1,24]],"date-time":"2025-01-24T17:27:59Z","timestamp":1737739679000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.lww.com\/01445473-201401000-00012"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,1]]},"references-count":19,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2014]]}},"URL":"https:\/\/doi.org\/10.1002\/lt.23765","relation":{},"ISSN":["1527-6465"],"issn-type":[{"value":"1527-6465","type":"print"}],"subject":[],"published":{"date-parts":[[2014,1]]}}}