ArticleOrphanet journal of rare diseases2026
Living-donor liver transplantation in children with inherited metabolic and genetic cholestatic liver diseases: a single-center retrospective cohort study.
Article in Orphanet journal of rare diseases, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
backgroundLiving donor liver transplantation (LDLT) has become an important therapeutic option for children with selected inherited metabolic and genetic cholestatic liver diseases (IM-GCLDs).However, evidence on disease-specific outcomes across different diagnostic categories remains limited, and we therefore conducted a single-center retrospective study with contemporaneous non-IM-GCLD pediatric LDLT recipients as a comparator to better contextualize transplant-related outcomes and disease-specific benefits.
resultsAmong 21 children with IM-GCLDs, the median follow-up was 21 months; two patients died (one perioperatively from disseminated intravascular coagulation and one at 21 months from pneumonia-related multiorgan failure), and all others are alive with functioning grafts. 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 <10 years, mean weight- and height-for-age Z-scores increased from -0.48 to 0.43 and from -0.76 to -0.01; in children ≥10 years, mean height Z-scores increased from -1.49 to -0.53 while BMI Z-scores showed no significant change. Overall survival did not differ significantly between IM-GCLDs and non-IM-GCLD indications.
conclusionsLiving 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.
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