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Alagille Syndrome
Conference Roundup

Liver transplant survival similar between patients with ALGS and BA

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No difference in the rate of patient or great survival was found between patients with Alagille syndrome (ALGS) and biliary atresia, despite higher rates of congenital heart defects, higher creatinine, and higher MELD/PELO scores at the time of transplant in patients with ALGS, according to a poster presented at the 2021 Annual SPLIT Meeting.

Data from 156 first-time liver transplant recipients with ALGS and 312 matched recipients with BA were analyzed.

Patients with ALGS had higher MELD/PELO scores, creatinine, albumin, and bilirubin levels at the time of transplant and were more likely to have underlying congenital heart disease than patients with BA. Catheter or surgical cardiac intervention was needed before or after transplant in 25.6% of patients with ALGS, with 22.2% requiring >1 intervention.

A longer length of stay was noted post-transplant in patients with ALGS. There was no difference in patient or graft survival between children with ALGS and those with BA.

Reference
Black K, et al. Pediatric Liver Transplant Outcomes in Alagille syndrome:  A Linked Database Analysis. Presented at: 2021 Annual SPLIT Meeting.

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