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Alagille Syndrome
Conference Roundup

Liver transplantation is safe in children with Alagille syndrome

Posted on May 23, 2021

Liver transplantation is safe in children with Alagille syndrome (ALGS) with most catching-up in growth after the procedure, according to a presentation at the 6th World Congress of Paediatric Gastroenterology, Hepatology and Nutrition.

In this retrospective study, clinical data from 89 patients with ALGS treated between 1995 and 2019 was collected pre-and post-liver transplant. Overall, 20% of patients (n = 18) underwent liver transplantation and 2 are currently on the waiting list. Malnutrition and/or growth retardation, severe pruritus, synthetic liver failure, and recurrent bone fractures were indications for transplantation. The median age at the time of transplant was 5.8 years (range 1.5-11.8).

All patients who underwent transplantation developed severe cholestasis in the first months of life, 27.7% had a history of Kasai hepatoportoenterostomy for misdiagnosed biliary atresia, 22% underwent partial external biliary, and 5.5% underwent ileal bypass. Most patients (72%) had cardiovascular anomalies, with pulmonary stenosis (55%) being the most common.

Transplantations included 12 from living donors and 6 from deceased donors. Portal vein stenosis and hepatic artery thrombosis occurred in the early post-transplant period in 2 patients each, respectively. Half of the patients developed acute cellular rejection and 1 patient died as a result of intra-abdominal hemorrhage. Overall patient survival was 94% over a median post-transplant follow-up was 9.2 years (1.9-13.4). Significant catch-up growth and weight gain were noted in this group of patients.

The authors said the at the time of the presentation, all patients present with normal graft function and no other significant issues, with stable renal function. No patients developed

chronic kidney disease or arterial hypertension.

A biopsy 3-8 years after transplant in 13 patients showed normal or mild fibrosis in 77% and moderate fibrosis in 23%.

Reference
Czubkowski P, et al. Outcomes of liver transplantation in children with Alagille syndrome. Presented at: 6th World Congress of Paediatric Gastroenterology, Hepatology and Nutrition.

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