BACKGROUND:Biliary atresia(BA) is a major cause of chronic cholestasis,a fatal disorder in infants.This study was undertaken to evaluate the safety and effectiveness of primary living donor liver transplantation(LDLT)...BACKGROUND:Biliary atresia(BA) is a major cause of chronic cholestasis,a fatal disorder in infants.This study was undertaken to evaluate the safety and effectiveness of primary living donor liver transplantation(LDLT) in comparison with the traditional first-line treatment,the Kasai procedure.METHODS:We assessed 28 children with BA at age of less than two years(3-21.3 months) who had undergone LDLT in two hospitals in Southwest China during the period of 2008-2011.Eighteen children who had had primary LDLT were included in a primary LDLT group,and ten children who had undergone the Kasai operation in a pre-Kasai group.All patients were followed up after discharge from the hospital.The records of the BA patients and donors were reviewed.RESULTS:The time of follow-up ranged 12-44.5 months with a median of 31 months.The 30-day and 1-year survival rates were 85.7% and 78.6%,respectively.There was no significant difference in the 30-day or 1-year survival between the two groups(83.3% vs 90% and 77.8% vs 80%,P】0.05).The main cause of death was hepatic artery thrombosis.There were more patients with complications who required intensive medical care or re-operation in the pre-Kasai group(8,80%) than in the primary LDLT group(9,50%)(P=0.226).But no significant differences were observed in operating time(9.3 vs 8.9 hours,P=0.77),intraoperative blood loss(208.6 vs 197.0 mL,P=0.84) and blood transfusion(105.6 vs 100.0 mL,P=0.91) between the two groups.The durations of ICU and hospital stay in the primary LDLT group and pre-Kasai group were 180.4 vs 157.7 hours(P=0.18) and 27 vs 29 days(P=0.29),respectively.CONCLUSIONS:Primary LDLT is a safe and efficient management for young pediatric patients with BA.Compared with the outcome of LDLT for patients receiving a previous Kasai operation,a similar survival rate and a low rate of re-operation and intensive medical care for patients with BA can be obtained.展开更多
Biliary atresia(BA), a chronic progressive cholestatic disease of infants, is the leading cause for liver transplant in children, especially in patients under two years of age. BA can be successfully treated with the ...Biliary atresia(BA), a chronic progressive cholestatic disease of infants, is the leading cause for liver transplant in children, especially in patients under two years of age. BA can be successfully treated with the Kasai portoenterostomy; however most patients still require a liver transplant, with up to one half of BA children needing a transplant by age two. In the current pediatric end-stage liver disease system, children with BA face the risk of not receiving a liver in a safe and timely manner. In this review, we discuss a number of possible solutions to help these children. We focus on two general approaches:(1) preventing/delaying need for transplantation, by optimizing the success of the Kasai operation; and(2) expediting transplantation when needed, by performing techniques other than the standard deceased-donor, whole, ABO-matched organ transplant.展开更多
基金supported by a grant from the National Science and Technology Major Project of China (2008ZX10002-025& 2008ZX10002-026)
文摘BACKGROUND:Biliary atresia(BA) is a major cause of chronic cholestasis,a fatal disorder in infants.This study was undertaken to evaluate the safety and effectiveness of primary living donor liver transplantation(LDLT) in comparison with the traditional first-line treatment,the Kasai procedure.METHODS:We assessed 28 children with BA at age of less than two years(3-21.3 months) who had undergone LDLT in two hospitals in Southwest China during the period of 2008-2011.Eighteen children who had had primary LDLT were included in a primary LDLT group,and ten children who had undergone the Kasai operation in a pre-Kasai group.All patients were followed up after discharge from the hospital.The records of the BA patients and donors were reviewed.RESULTS:The time of follow-up ranged 12-44.5 months with a median of 31 months.The 30-day and 1-year survival rates were 85.7% and 78.6%,respectively.There was no significant difference in the 30-day or 1-year survival between the two groups(83.3% vs 90% and 77.8% vs 80%,P】0.05).The main cause of death was hepatic artery thrombosis.There were more patients with complications who required intensive medical care or re-operation in the pre-Kasai group(8,80%) than in the primary LDLT group(9,50%)(P=0.226).But no significant differences were observed in operating time(9.3 vs 8.9 hours,P=0.77),intraoperative blood loss(208.6 vs 197.0 mL,P=0.84) and blood transfusion(105.6 vs 100.0 mL,P=0.91) between the two groups.The durations of ICU and hospital stay in the primary LDLT group and pre-Kasai group were 180.4 vs 157.7 hours(P=0.18) and 27 vs 29 days(P=0.29),respectively.CONCLUSIONS:Primary LDLT is a safe and efficient management for young pediatric patients with BA.Compared with the outcome of LDLT for patients receiving a previous Kasai operation,a similar survival rate and a low rate of re-operation and intensive medical care for patients with BA can be obtained.
文摘Biliary atresia(BA), a chronic progressive cholestatic disease of infants, is the leading cause for liver transplant in children, especially in patients under two years of age. BA can be successfully treated with the Kasai portoenterostomy; however most patients still require a liver transplant, with up to one half of BA children needing a transplant by age two. In the current pediatric end-stage liver disease system, children with BA face the risk of not receiving a liver in a safe and timely manner. In this review, we discuss a number of possible solutions to help these children. We focus on two general approaches:(1) preventing/delaying need for transplantation, by optimizing the success of the Kasai operation; and(2) expediting transplantation when needed, by performing techniques other than the standard deceased-donor, whole, ABO-matched organ transplant.
