RÉSUMÉ
<p><b>OBJECTIVE</b>To summarize the clinical efficacy of pediatric liver transplantation, and investigate the characters of pediatric liver transplantation in their indications, surgical procedures and postoperative management.</p><p><b>METHODS</b>From August 2000 to March 2007, 23 liver transplantations were performed on 20 children, aging from 6 months to 13 years old. The most common indications were biliary atresia, Wilson's disease, glycogen storage disease and urea cycle defects. Surgical procedures included 4 living donor liver transplantations, 1 Domino liver transplantation, 5 split grafts, 10 reduced liver grafts and 3 whole cadaveric grafts. The triple-drug (FK506, steroid and MMF) immunosuppressive regimen was used in 19 children, except one children using cyclosporine.</p><p><b>RESULTS</b>Three children died of primary non-function, heart failure and abdominal infections respectively during peri-operative period, and the mortality was 15.0%. Nine children showed different post-operative complications including 2 hepatic artery thrombosis, 1 portal vein thrombosis, 1 acute rejection, 3 biliary leakage, 2 biliary stricture, 2 intestinal fistula, 3 abdominal infection, 1 pulmonary infection and 1 heart failure. Cumulative patient survival rates at 6-month, 1-and 2-year were 80.0%, 73.9% and 73.9%, respectively.</p><p><b>CONCLUSIONS</b>Liver transplantation is an effective option to cure the liver disease of children with end-stage. Different surgical procedure could be chosen according to the children's age and body weight.</p>
Sujet(s)
Adolescent , Enfant , Enfant d'âge préscolaire , Femelle , Humains , Nourrisson , Mâle , Études de suivi , Immunosuppresseurs , Transplantation hépatique , Méthodes , Complications postopératoires , Thérapeutique , Études rétrospectives , Taux de survie , Résultat thérapeutiqueRÉSUMÉ
<p><b>BACKGROUND</b>Although liver transplantation has become a standard therapy for end-stage liver diseases, the experience of pediatric liver transplantation is limited in China. In this article we report our experience in pediatric liver transplantation, and summarize its characters in their indications, surgical techniques, and postoperative managements.</p><p><b>METHODS</b>Thirty-one children (< or = 18 years old) underwent liver transplantation in our centers. The mean age at transplantation was 12.4 years old (ranged from 5 months to 18 years) with 7 children being less than 4 years of age at transplantation. The most common diagnosis of patients who underwent liver transplantation were biliary atresia, Wilson's disease, primary biliary cirrhosis, glycogen storage disease, hepatoblastoma, urea cycle defects, fulminant hepatic failure, etc. The surgical procedures included 12 standard (without venovenous bypass), 6 pigyback, 6 reduced-size, 3 split, 3 living donor liver transplantation, and 1 Domino liver transplantation. The triple-drug (FK506, steroid, and mycophenolate mofetil) immunosuppressive regimen was used in most of patients. Patients were followed up for a mean of 21.8 months.</p><p><b>RESULTS</b>Five of the 31 patients died during perioperative time; mortality rate was 16.1%. The reasons of death were infections, primary non-function, heart failure, and hypovolemic shock. Postoperative complications in 10 patients included biliary leakage, acute rejection, abdominal infection, hepatitis B virus (HBV) or hepatitis C virus (HCV) infection, and pulmonary infection. Overall patient cumulative survival rate at 1-, 3-, and 5-year was 78.1%, 62.6%, 62.6%, respectively.</p><p><b>CONCLUSIONS</b>The most common indications of pediatric liver transplantation were congenital end-stage liver diseases. According to patients' age and body weight, standard, piggyback, reduced-size, split, or living donor liver transplantation should be performed. Pediatric liver transplantation especially requires higher surgical skills. The early postoperative management is the key to success. Postoperative bile leak was common, but most patients underwent liver transplantation had a better prognosis.</p>