期刊文献+

胆管闭锁:755例肝移植患儿的临床特点、危险因素以及预后情况

Biliary atresia: Clinical profiles, risk factors, and outcomes of 755 patients listed for liver transplantation
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摘要 Objectives: To test the hypothesis that risk analysis from the time of listing for liver transplantation (LT) focuses attention on areas where outcomes can be improved. Study design: Competing outcomes and multivariate models were used to determine significant risk factors for pretransplantation and posttransplantati on mortality and graft failure in patients with biliary atresia (BA) listed for LT and enrolled in the Studies of Pediatric Liver Transplantation (SPLIT) regist ry. Results: Of 755 patients, most were infants (age < 1 year). Significant wait ing list mortality risk factors included infancy and pediatric end-stage liver disease (PELD) score ≥20, whose components were also continuous risk factors. S urvival posttransplantation (n = 567) was 88%at 3 years. Most deaths were from infection (37%). Posttransplantation mortality risk factors included infant rec ipients, height/weight < -2 standard deviations (SD), use of cyclosporine versu s tacrolimus and retransplantation. Graft failure risks included height/weight < -2 SD, cadaveric partial donors,donor age ≤5 months, use of cyclosporine vers us tacrolimus, and rejection. Conclusions: Referral for LT should be anticipator y for infants with BA with failed portoenterostomies. Failing nutrition should p rompt aggressive support. Post-LT risk factors are mainly nonsurgical, includin g nutrition, the relative risk of infection over rejection, and the choice of im munosuppression. Objectives: To test the hypothesis that risk analysis from the time of listing for liver transplantation (LT) focuses attention on areas where outcomes can be improved. Study design: Competing outcomes and multivariate models were used to determine significant risk factors for pretransplantation and posttransplantati on mortality and graft failure in patients with biliary atresia (BA) listed for LT and enrolled in the Studies of Pediatric Liver Transplantation (SPLIT) regist ry. Results: Of 755 patients, most were infants (age < 1 year). Significant wait ing list mortality risk factors included infancy and pediatric end-stage liver disease (PELD) score ≥20, whose components were also continuous risk factors. S urvival posttransplantation (n = 567) was 88%at 3 years. Most deaths were from infection (37%). Posttransplantation mortality risk factors included infant rec ipients, height/weight < -2 standard deviations (SD), use of cyclosporine versu s tacrolimus and retransplantation. Graft failure risks included height/weight < -2 SD, cadaveric partial donors,donor age ≤5 months, use of cyclosporine vers us tacrolimus, and rejection. Conclusions: Referral for LT should be anticipator y for infants with BA with failed portoenterostomies. Failing nutrition should p rompt aggressive support. Post-LT risk factors are mainly nonsurgical, includin g nutrition, the relative risk of infection over rejection, and the choice of im munosuppression.
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