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Alagille syndrome
Guidelines
Key sources
The following summarized guidelines for the evaluation and management of Alagille syndrome are prepared by our editorial team based on guidelines from the European Association for the Study of the Liver (EASL 2024), the Italian Society of Pediatric Gastroenterology, Hepatology and Nutrition (SIGENP 2022), and the American Society of Transplantation (AST/NASPGHAN/AASLD 2014).
1
2
3
Screening and diagnosis
Diagnostic procedures
Medical management
Ileal bile acid transporter inhibitors: as per EASL 2024 guidelines, offer ileal bile acid transporter inhibitors, when available, in patients with Alagille syndrome and cholestatic pruritus.
B
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Statins
Surgical interventions
Liver donation
As per AASLD/AST/NASPGHAN 2014 guidelines:
Consider using living donation from a first-degree family member for patients with Alagille syndrome, but obtain a careful donor evaluation with liver biopsy and/or cholangiography to rule out bile duct hypoplasia.
C
Obtain a careful evaluation of the donor for bile duct hypoplasia and vascular anomalies if the potential donor and recipient share the same mutant JAG1 or NOTCH2 allele, but do not perform living-related liver transplantation in most circumstances.
B