{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,28]],"date-time":"2026-08-28T14:34:51Z","timestamp":1787927691805,"version":"build-2784847793"},"reference-count":55,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2025,3,12]],"date-time":"2025-03-12T00:00:00Z","timestamp":1741737600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100005003","name":"Baskent \u00dcniversitesi","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100005003","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Transplantation"],"published-print":{"date-parts":[[2025,5]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Inborn errors of metabolism (IEMs) are inherited diseases causing significant morbidity and mortality, particularly in childhood. Liver transplantation (LT) can be curative or partially effective for these diseases. LT for IEMs has increased, making IEMs the second most common reason for pediatric LT after biliary atresia.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Patients and Methods<\/jats:title>\n                    <jats:p>Between 2001 and 2023, 50 pediatric patients with IEMs underwent LT at Ba\u015fkent University, Ankara Hospital. Data collected retrospectively included diagnosis, gender, age of diagnosis, age of LT, LT indication, donor data, graft type, rejection episodes, post\u2010transplant complications, and clinical findings of the IEMs before and after LT. Treatment methods, follow\u2010up duration, and survival time were also recorded.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Of the 332 pediatric LT patients, 50 (15.1%) had IEMs, with three requiring re\u2010transplantations. Diagnoses included glycogen storage diseases (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200911), tyrosinemia type 1 (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200910), primary hyperoxaluria (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20096), urea cycle disorders (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20096), homozygous familial hypercholesterolemia (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20094), propionic acidemia (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20094), deoxyguanosine kinase deficiency (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20093), maple syrup urine disease (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20092), methylmalonic acidemia (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20091), Niemann\u2010Pick disease type B (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20091), alkaptonuria with unknown neonatal cholestasis (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20091), and bile acid synthesis disorder (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20091). The parental consanguinity rate was 74%. Living\u2010related donors provided organs for 48 (90.5%) patients. The mean age at LT was 75.3\u2009\u00b1\u20098.2\u2009months (range: 5\u2013218), with a follow\u2010up period of 82.1\u2009\u00b1\u200910.2\u2009months (range:1\u2009day\u2013229\u2009months). Survival rates at 1, 5, 10, and 15\u2009years were 83.7%, 81%, 81%, and 70.9%, respectively.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>LT is an effective solution for children with IEM causing chronic organ failure and difficult to manage with medical treatment, showing a good long\u2010term prognosis.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/petr.70067","type":"journal-article","created":{"date-parts":[[2025,3,13]],"date-time":"2025-03-13T02:59:11Z","timestamp":1741834751000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Short and Long\u2010Term Outcomes of Liver Transplantation in Pediatric Patients With Inborn Errors of Metabolism: A Single\u2010Center Study"],"prefix":"10.1111","volume":"29","author":[{"given":"Hazel Delal","family":"Dara 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