Background <p>Pediatric liver transplantation is the definitive treatment for end-stage liver disease, including cirrhosis secondary to biliary atresia (BA). Understanding long-term outcomes and factors influencing survival is essential for optimizing care.</p> Methods <p>We performed a retrospective, single-center analysis of pediatric patients (&lt; 18 years) who underwent liver transplantation for biliary atresia between March 2000 and June 2023 at Abu-Ali Sina Hospital, Shiraz University of Medical Sciences. Clinical and laboratory data of patients were retrieved from electronic medical records.</p> Results <p>A total of 160 patients were included, with a mean age at transplantation of 4.5 ± 0.8 years. The cumulative 20-year graft survival rate was 63.8%. Patient survival was 80% at 6 months and 64.4% at 5 years post-transplantation. Mortality was predominantly concentrated within the first 90 days following transplantation, with sepsis, pneumonia, and hemorrhage representing the leading causes of early death. Chronic rejection, although less frequent, remained a significant contributor to late graft loss. Notably, the decline in overall survival was largely driven by early post-transplant mortality, with survival curves demonstrating relative stabilization beyond the initial high-risk period. After adjustment for graft type and recipient sex, no statistically significant differences in graft or patient survival were observed. These findings are consistent with accumulating evidence that, in experienced centers, living donor liver transplantation (LDLT) and deceased donor liver transplantation (DDLT) yield comparable long-term outcomes.</p> Conclusions <p>Pediatric liver transplantation offers durable long-term outcomes, with early postoperative care being critical. Both LDLT and DDLT showed similar trends in outcomes in this cohort, although no definitive differences could be established. Prospective, multicenter studies are warranted to further clarify factors influencing long-term outcomes.</p>

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Long-term outcomes of liver transplantation in biliary atresia: a 20-year single-center study

  • Zahra Beyzaei,
  • Alireza Shamsaeefar,
  • Kiana Ghatei,
  • Kurosh Kazemi,
  • Saman Nikeghbalian,
  • Masoud Dehghani,
  • Seyed-Ali Malekhosseini,
  • Maryam Jalali,
  • Bita Geramizadeh

摘要

Background

Pediatric liver transplantation is the definitive treatment for end-stage liver disease, including cirrhosis secondary to biliary atresia (BA). Understanding long-term outcomes and factors influencing survival is essential for optimizing care.

Methods

We performed a retrospective, single-center analysis of pediatric patients (< 18 years) who underwent liver transplantation for biliary atresia between March 2000 and June 2023 at Abu-Ali Sina Hospital, Shiraz University of Medical Sciences. Clinical and laboratory data of patients were retrieved from electronic medical records.

Results

A total of 160 patients were included, with a mean age at transplantation of 4.5 ± 0.8 years. The cumulative 20-year graft survival rate was 63.8%. Patient survival was 80% at 6 months and 64.4% at 5 years post-transplantation. Mortality was predominantly concentrated within the first 90 days following transplantation, with sepsis, pneumonia, and hemorrhage representing the leading causes of early death. Chronic rejection, although less frequent, remained a significant contributor to late graft loss. Notably, the decline in overall survival was largely driven by early post-transplant mortality, with survival curves demonstrating relative stabilization beyond the initial high-risk period. After adjustment for graft type and recipient sex, no statistically significant differences in graft or patient survival were observed. These findings are consistent with accumulating evidence that, in experienced centers, living donor liver transplantation (LDLT) and deceased donor liver transplantation (DDLT) yield comparable long-term outcomes.

Conclusions

Pediatric liver transplantation offers durable long-term outcomes, with early postoperative care being critical. Both LDLT and DDLT showed similar trends in outcomes in this cohort, although no definitive differences could be established. Prospective, multicenter studies are warranted to further clarify factors influencing long-term outcomes.