Biliary atresia.

Biliary atresia (BA) is a progressive inflammatory fibrosclerosing disease of the biliary system and a major cause of neonatal cholestasis. It affects 1:5,000-20,000 live births, with the highest incidence in Asia. The pathogenesis is still unknown, but emerging research suggests a role for ciliary dysfunction, redox stress and hypoxia. The study of the underlying mechanisms can be conceptualized along the likely prenatal timing of an initial insult and the distinction between the injury and prenatal and postnatal responses to injury. Although still speculative, these emerging concepts, new diagnostic tools and early diagnosis might enable neoadjuvant therapy (possibly aimed at oxidative stress) before a Kasai portoenterostomy (KPE). This is particularly important, as timely KPE restores bile flow in only 50-75% of patients of whom many subsequently develop cholangitis, portal hypertension and progressive fibrosis; 60-75% of patients require liver transplantation by the age of 18 years. Early diagnosis, multidisciplinary management, centralization of surgery and optimized interventions for complications after KPE lead to better survival. Postoperative corticosteroid use has shown benefits, whereas the role of other adjuvant therapies remains to be evaluated. Continued research to better understand disease mechanisms is necessary to develop innovative treatments, including adjuvant therapies targeting the immune response, regenerative medicine approaches and new clinical tests to improve patient outcomes.

A Retrospective Study of Long-Term Outcomes in 16 ABO-Incompatible Deceased Donor Pediatric Liver Transplants from a National Transplant Center at Helsinki University Hospital, Finland, 1987-2022.

BACKGROUND The use of ABO-incompatible liver transplants (ABO-ILTs) from deceased donors has become more common due to the shortage of available donor livers and increased transplant waiting times. This retrospective study from a national transplant center at Helsinki University Hospital, Finland, aimed to assess the long-term outcomes of ABO-incompatible deceased donor pediatric liver transplants between 1987 and 2022. MATERIAL AND METHODS Sixteen (9.5%) of the 169 pediatric liver transplantations were ABO-ILTs. The median age at transplantation was 5.0 (0.5-15.4) years. Reasons for ABO-ILTs were acute liver failure (18.75%), malignancy (12.5%), small body size and long waiting time (25%), and other reasons (43.75%). The median post-transplant follow-up time was 147 (0.72-353) months. Patient and graft survival and occurrence of surgical complications were compared to ABO-identical transplants, and anti-ABO antibody titers were analyzed. RESULTS The 1-, 3-, and 5-year patient survivals were comparable between the ABO-I and ABO-compatible groups, being 81.3%, 73.9%, and 73.9% (ABO-I) and 87.5%, 82.5%, 77.9% (ABO-compatible), respectively. Three patients with ABO-ILTs died of sepsis and multiorgan failure during the first 3 months after transplantation. The occurrence of biliary complications and early vascular thrombosis (<30 days after transplantation) did not differ significantly between recipients with an ABO-ILT vs ABO-compatible liver graft. CONCLUSIONS The findings from this study support findings from previous studies that outcomes after ABO-incompatible liver transplants in children were comparable to outcomes from ABO-identical liver transplants.