{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,16]],"date-time":"2026-05-16T03:41:10Z","timestamp":1778902870399,"version":"3.51.4"},"reference-count":32,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2020,2,25]],"date-time":"2020-02-25T00:00:00Z","timestamp":1582588800000},"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":["J of Digest Diseases"],"published-print":{"date-parts":[[2020,3]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>To evaluate the efficacy of living\u2010donor liver transplantation (LDLT) in children with tyrosinemia type I.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Altogether 10 patients diagnosed with tyrosinemia type I underwent LDLT between June 2013 and April 2019. Cirrhosis was the indication for LDLT in all 10 patients, and hepatocellular carcinoma (HCC) was suspected in nine. Patients' outcomes, including liver function, restoration of metabolism, quality of life and physical development, were analyzed after LDLT.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>All recipients were alive with a normal liver function after a median follow\u2010up period of 49 months. Pathological examinations detected HCC in one patient, dysplasia in five and cirrhosis in all. Nine patients were found to have elevated alpha\u2010fetoprotein level, and their median alpha\u2010fetoprotein level dropped from 2520\u2009ng\/mL to a normal level after LDLT, with no recurrence of HCC detected during the follow\u2010up. Tyrosine metabolism was restored to its normal level with normalized plasma tyrosine and succinylacetone concentrations. Moreover, urinary succinylacetone excretion decreased significantly during the follow up. LDLT improved patients\u2019 renal tubular function, as evidenced by the normalized plasma phosphate concentration and improved glomerular filtration rate. Severe rickets symptoms, including spontaneous fractures and bone pain, were also ameliorated. Improved motor function was reported by all patients' parents during the follow\u2010up. Dietary restriction was no longer required, which was associated with a favorable catch\u2010up in growth and improved quality of life. Complete resolution of hypertrophic cardiomyopathy was observed one year after LDLT in one patient.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>LDLT is an effective treatment for patients with end\u2010stage liver disease resulting from tyrosinemia type I.<\/jats:p><\/jats:sec>","DOI":"10.1111\/1751-2980.12846","type":"journal-article","created":{"date-parts":[[2020,1,18]],"date-time":"2020-01-18T06:09:17Z","timestamp":1579327757000},"page":"189-194","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Living\u2010donor liver transplantation for children with tyrosinemia type I"],"prefix":"10.1111","volume":"21","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4033-1026","authenticated-orcid":false,"given":"Yuan","family":"Liu","sequence":"first","affiliation":[{"name":"Department of Liver Surgery Renji Hospital, School of Medicine, Shanghai Jiao Tong University  Shanghai 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