Abstract
Several therapeutic modalities have been introduced to deal with the graft shortage in pediatric liver transplantation. We have performed thrity living related liver transplantation (LRLT) procedures over the past 19 months in 30 patients who were 8 months to 15 years of age; 23 had biliary atresia, 2 liver cirrhosis, 2 Budd-Chiari syndrome, 1 Protoporphyria and 2 progressive intrahepatic cholestasis. Sixteen lateral segmentectomies, 13 left lobectomies and one right lobectomy were performed for graft harvesting. A partial orthotopic live transplant with an intact inferior vena cava was performed. Our standard immunosuppression consists of FK 506 and low dose steroids. Postoperative management has been done on Redox theory. Arterial ketone body ratio (AKBR) was serially measured every 12 hours over the first postopertaive week and once a day later as an indicator for evaluating the viabilitiy of graft liver until the graft survival was obtained. Twenty four recipients are alive and well with the original graft and normal live function. Patient survival rate was 88% (22/25) in elective cases. 6 patients died with functioning graft due to the extrahepatic complications; one aspiration asphyxia, two cardiac insufficiency, one candida infection and one lymphoproliferative disorder. Graft complications were encountered in 5 recipients. Two patients had biliary stenosis, one portal vein thrombosis, one arterial thrombosis and two hepatic vein stenosis. Three rejection episodes were obsebed in 7 ABO compatible cases and one episode in 3 incompatible cases. In all 30 cases, AKBR increased above 1.0 within 2 days after transplantation. However, the increased AKBR above 1.0 decreased again in 5 cases. The causes of the AKBR redecrease were determined to be hepatic artery thrombosis, portal vein thrombosis, massive intraabdominal hematoma, hepatic stenosis and lymphoproliferative infiltration into the liver, respectively. The decreased AKBR recovered promptly following successful surgical interventions in 4 cases. All donors have perfectly normal hepatic function and came back to the normal social lives. Our results suggested that LRLT is a very important option toward resolving the graft shortage in pediatric liver transplantation and post operative management based on the Redox theory is crucial for outcome.