@article{article_979822, title={Pediatric Liver Transplantation For Hereditary Metabolic Disorders With Structural Liver Damage}, journal={Türkiye Çocuk Hastalıkları Dergisi}, volume={15}, pages={408–414}, year={2021}, DOI={10.12956/tchd.979822}, author={Çelik, Neslihan and Emiroglu, Remzi}, keywords={liver transplantation, metabolic disease, Child}, abstract={<p> <b>Objective: </b> Variety of inherited metabolic diseases with or without liver parenchymal injury can be cured by liver transplantation and this process is actually a surgical enzyme replacement therapy for the defective protein. The aim of this single center study was to analyze the liver transplantation indications, post-transplantation outcomes with benefits of the procedure, and also identification of the problems encountered during the process to improve the success rate in pediatric metabolic diseases with liver parenchymal damage. </p> <p> <b>
Material and Methods: </b> We retrospectively reviewed the records of children who underwent liver transplantation because of metabolic disorders with liver parenchymal damage from January 2015 to June 2021. Data collected included patient and donor demographics, operative techniques, patient and graft survivals, post-transplant surgical and medical complications, and immunosuppressive protocols. </p> <p> <b>Results: </b> Fourteen children with progressive familial intrahepatic cholestasis (n = 8), Alagille syndrome (n = 3), tyrosinemia type-I (n=1), familial neonatal hepatitis (FNH, n=1) and glycogen storage disease type-I (GSD-I, n = 1) received left lateral segment (n=10), right lobe (n=2), reduced size left lateral segment (n=1), and mono-segment (n=1) allografts from living donors. The median age of 5 boys and 9 girls at time of transplantation was 42.3 months (range 6.9-215.5 months). One patient with FNH was lost to follow and excluded from the study. The median follow-up time was 12.7 months (range 0.4-53.4 months) and we lost four patients because of sepsis. Most common post-transplant complications were acute cellular rejection and infections. </p> <p> <b>
Conclusion: </b>Liver transplantation is a lifesaving treatment for patients with metabolic disorders comprising liver failure, malignancies, and associated complications despite several medical and surgical treatment modalities. </p>}, number={5}, publisher={T.C. Sağlık Bakanlığı Ankara Şehir Hastanesi}