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Urgent liver transplantation for biliary atresia: the experience in Bicêtre
F Gauthier1, C Chardot, S Branchereau
1Service de Chirurgie Pédiatrique, Centre Hospitalier Universitaire Bicêtre, Faculté de Médecine Paris Sud, Université Paris 11, Le Kremlin Bicêtre, France.
Insights
Children with biliary atresia (BA) and acute liver necrosis (ALN) benefit from urgent liver transplantation (LT) registration. This strategy significantly improves survival rates for these critically ill children, offering them a crucial chance to receive a life-saving transplant.
Area of Science:
- Pediatric Surgery
- Hepatology
- Transplantation Medicine
Background:
- Biliary atresia (BA) is a severe liver disease in children.
- Acute liver necrosis (ALN) complicates BA, necessitating urgent intervention.
- Liver transplantation (LT) is a critical treatment for end-stage liver disease in children.
Purpose of the Study:
- To evaluate the impact of an urgent registration code for children with BA and ALN on LT waiting lists.
- To assess the outcomes of pediatric liver transplantation for BA with ALN.
- To determine if this allocation strategy affects graft survival and patient outcomes.
Main Methods:
- Retrospective analysis of 115 pediatric LT cases over 7 years.
- Comparison of outcomes between elective and urgent LT for BA.
- Analysis of patient and graft survival rates at 1, 12, and 48 months post-transplant.
Main Results:
- Urgent LT accounted for 25% of BA cases (0-2 years) and 67% (under 1 year).
- Patient survival at 48 months was 60% for urgent LT vs. 85% for elective LT.
- Graft survival at 48 months was 53% for urgent LT vs. 76% for elective LT (p < 0.05).
- Outcomes for BA with ALN were comparable to other urgent LT indications, excluding retransplants.
Conclusions:
- An urgent registration code for children with BA and ALN significantly increases their chance of receiving a life-saving LT.
- This allocation strategy improves survival rates for critically ill children without compromising graft viability.
- The French organ sharing rules effectively prioritize children with BA and ALN, offering them a >50% chance of survival.
Abstract:
According to French rules for cadaver organ sharing, children with biliary atresia (BA) complicated with acute necrosis (ALN) can be registered on the waiting list for liver transplantation (LT) in a special intermediate grade urgent code. Over a 7 years period, 100 children have been submitted to elective LT for BA and 15 to urgent LT. Urgent procedures accounted for 25% of LT for BA in patients aged 0-2 years and 67% (8/12) in patients under 1 year of age. Children actuarial survival at 1, 12 and 48 months was respectively 66%, 60% and 60% versus 92%, 86% and 85%, deaths occurring earlier in the urgent group. Graft actuarial survival at 1, 12 and 48 months were 60%, 53% and 53% versus 85%, 77% and 76% (p < 0.05), respectively. Outcome of children and grafts after LT is not significantly different in BA cases and in other urgent indications, excluding retransplantations. In a LT program based on cadaver organ donation, allocation of in an urgent registration code to children with BA and ALN offers them more than 50% chance to escape death and does not result in wasting of grafts.