https://www.ncbi.nlm.nih.gov/pubmed/31410937 Biliary atresia
Pediatr Transplant. 2019 Aug 13:e13569. doi: 10.1111/petr.13569. [Epub ahead of print]
A paradigm shift in the intention-to-transplant children with biliary atresia: Outcomes of 101 cases and a review of the literature.
de Ville de Goyet Prof J1, Grimaldi C Dr2, Tuzzolino F3, di Francesco F Dr1.

Abstract

For children with BA who do not benefit from Kasai surgery, the only therapeutic option is liver replacement and transplantation. The very decision to proceed for transplantation is a crucial point in time because it is the first step toward the preparation for the transplantation. The former time point is defined in this analysis as "intent-to-transplant" care pathway. In the life of every BA candidate for liver replacement, this point in time varies and mostly depends on the decision of their primary caring teams-about when to switch from supportive care to transplant, and thus to refer to a transplant center. This intent-to-transplant analysis of a series of 101 consecutive infants that were referred to a single transplant team showed that excellent overall outcome (97% survival) has been achieved overall. However, three deaths occurred that were clearly related to a late referral. This analysis and recent observations from other centers strongly support that the timing for referring these children to a transplant center and/or deciding to list them on the waiting list is currently too late and should be anticipated to what it is currently. This paradigm shift in the intention-to-transplant children is likely necessary for giving a better chance to an increased number of children and impacts positively on the general outcome. Networking and defining new tools for a rapid recognition of the infants who need early transplantation are necessary; centralization of these children may be helpful to achieve better outcomes than currently observed.