Abstracts Poster Abstracts - Dr Falk

103A 5-years single center experience in liver transplantation forpaediatric end-stage cholestatic diseasesSonzogni A., Torre G., Colledan M., Spada M., Lucianetti A., Melzi M.l., Stroppa P.,Riva S., Gridelli B.Liver Transplantation Center, Ospedali Riuniti, I-24128 Bergamo, Italy,E-mail: asonzogni@ospedaliriuniti.bergamo.itChronic cholestatic liver diseases are the leading causes of cirrhosis and hepatic failurein pediatric groups. Orthotopic liver transplantation (OLTx) is now a safe curativeprocedure in these diseases. We present a 5-years single center experience withencouraging long-term results.Materials and Methods: Between October 1997 and August 2003, 206 pediatricpatients (median age at OLTx: 1.4 yrs - range 0.1-17 yrs; median weight: 9 kg - range2-65 kg) underwent 229 OLTx for end-stage liver disease in our center. Most of them(73%) received a split liver graft and an entero-biliary anastomosis were performed inall of them.161 were transplanted for end-stage cholestatic liver disease (123 extrahepatic biliaryatresia, 17 Alagille syndrome, 12 familial progressive intrahepatic cholestasis, 3Crigler-Najjar syndrome) after a median time of 40 days period in waiting list. Pre-OLTxLFT's values were very high levels of total bilirubin (mean 18.5 mg/dl; range 0.8-46),cholesterol (mean 215 mg/dl; range 22-1120) and other laboratory findings of liverfailure. Most of them (85%) suffered from severe malnutrition and were above 5 thpercentile at OLTx (mean weight 8 kg; range 4-44). Incidental HCC was discovered inexplanted liver in 3/161 and chemotherapy was performed; no tumor relapse wasexperience in a medium time follow-up of 3 yrs. Patients were discharged from IntensiveUnit in a median time of 3 days and from Pediatric Department in a median time of 3weeks. Growth indexes come back to normal values at a mean time of 0.8 yrs fromsurgery. Post-OLTx immunosuppression was carried on drugs combination in first 8months (steroid and cyclosporin or tacrolimus) and on cyclosporin or tacrolimus basisin long term follow-up. Follow-up was realized in all children as outpatients with apreviously definite schedule; access to Liver Unit for sudden matters was anywaywarranted.Results: Patients' survival rate was 87% at one year from surgery, 85% at 3 years and83% at 5 years from surgery. Main post-OLTx complications were acute cellularrejection steroid-sensitive (24%), vascular thrombosis (11%) and mechanicalcholestasis due to anastomotic stricture (43 pts, 27%); this latter complication wassuccessfully treated by PTC procedure and long-term administration of ursodeoxycholicacid at maximal dosage in 38 pts. PTLD-EBV related was observed in 4 children (2 caseof polymorphic PTLD, 2 cases of large cell B lymphoma) and were successfully treatedby stopping immunosuppressive drugs. Re-transplantation rate was 10% (16 pts) andmortality rate during and around surgery was 8%.