Alagille Syndrome
Conference Roundup

Patients with ALGS have poorer survival following LDLT than those with biliary atresia

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Patients with Alagille syndrome (ALGS) and biliary atresia (BA) have different experiences when it comes to undergoing living donor liver transplantation (LDLT), including different wait times and graft event-free survival in the first 6 months after transplantation, according to a poster at 2021 SPLIT Annual Meeting.

According to the presentation, LT is common in patients with these 2 rare diseases. Only 24%-41% of patients with ALGS reach adulthood without needing LT whereas only 23% of patients with BA survive to the age of 20 years with their native liver. BA is considered the leading indication for LT in children.

In this retrospective analysis to evaluate outcomes in with ALGS compared with children with BA, 117 patients with ALGS undergoing first-time pediatric LT were compared with 936 patients with BA were included. There were 5 patients that had both BA and ALGS, which were included in the ALGS cohort.

Patients with ALGS were more likely than those with BA to be older at the time of listing (3.55 years vs 1.9 years; P <0.0001) and older at the time of transplants (4.21 years vs 2.45; P <0.0001)

Patients with ALGS spent more time on the transplant waitlist (127 days vs 88 days) and were less likely to utilize LDLT than patients with BA (12% vs 22%).

Overall, 7 patients with ALGS and 33 with BA died, with all deaths in patients with ALGS who underwent LDLT occurring in the first 6 months post-transplant

“It was unclear as to why patients with ALGS had poorer survival following LDLT than those with BA, as there were no clear trends in causes of death and graft failure in the ALGS population,” the authors concluded.

Miloh T, et al. Presented at: The Society of Pediatric Liver Transplantation (SPLIT) Annual Meeting; September 30–October 1, 2021.