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Disease-specific manifestations, including neuropsychiatric symptoms, portal hypertension, metabolic crises, cholestasis, hyperbilirubinemia, and hyperammonemia, improved or resolved in almost all survivors.At 6 months after LDLT, in children\u2009&lt;10 years, mean weight- and height-for-age Z-scores increased from \u22120.48 to 0.43 and from \u22120.76 to \u22120.01; in children\u2009\u226510 years, mean height Z-scores increased from \u22121.49 to \u22120.53 while BMI Z-scores showed no significant change. Overall survival did not differ significantly between IM-GCLDs and non-IM-GCLD indications.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Living donor liver transplantation in children with IM-GCLDs not only improves survival but also confers disease-specific benefits, including recovery of neurologic function, metabolic stabilization, relief of portal hypertension and cholestasis, and catch-up growth. These findings support LDLT as an important therapeutic option for IM-GCLDs, while diagnosis-tailored perioperative assessment and long-term management remain essential given the phenotypic heterogeneity.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/s13023-026-04369-4","type":"journal-article","created":{"date-parts":[[2026,4,30]],"date-time":"2026-04-30T13:08:27Z","timestamp":1777554507000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Living-donor liver transplantation in children with inherited metabolic and genetic cholestatic liver diseases: a single-center retrospective cohort study"],"prefix":"10.1186","volume":"21","author":[{"given":"Hui","family":"Liu","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zhida","family":"Chen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2868-0389","authenticated-orcid":false,"given":"Wanfu","family":"Li","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Halimulati","family":"Huxitaer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gulimiremu","family":"Maimaiti","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ayiguzaili","family":"Maimaijiang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yeliaman","family":"Jiayilawu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Aerxin","family":"Habuding","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Runqi","family":"Xi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Haoyu","family":"Wang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"FangJuan","family":"Song","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2026,4,30]]},"reference":[{"key":"4369_CR1","unstructured":"Qiang X. 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This study was reviewed and approved by the Ethics Committee of the First Affiliated Hospital of Xinjiang Medical University (This study was approved by the Ethics Committee of the First Affiliated Hospital of Xinjiang Medical University (approval No. K202602-46). The requirement for informed consent was waived due to the retrospective nature of the study). All procedures involving human participants were performed in accordance with the Declaration of Helsinki and the ethical standards of national and institutional research committees.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Institutional review board"}},{"value":"Informed consent was obtained from all subjects involved in the study.Written informed consent has been obtained from the patient(s) to publish this paper\u201d if applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Informed consent"}},{"value":"None.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Patents"}},{"value":"The authors declare no conflicts of interest.","order":5,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflicts of interest"}}],"article-number":"183"}}