Treatment
Treatment options include:
- phototherapy
- plasmapheresis
- is the most effective method for removing excess unconjugated bilirubin from the blood during severe hyperbilirubinaemia crisis (1)
- pharmacological treatment options include (2):
- enzyme-inducing agents (phenobarbital)
- bilirubin-binding agents (calcium phosphate, orlistat)
- choleretics (ursodeoxycholic acid),
- haeme-oxygenase inhibitors (Tin-protoporphyrin, Zinc-protoporphyrin)
- liver transplantation
- the only cure for Type 1 Crigler-Nijjar syndrome is a liver transplant
- in Type 2 Crigler-Nijjar syndrome the need for a liver transplant is infrequent (1)
Reference:
- Bhandari J, Thada PK, Shah M, et al. Crigler-Najjar Syndrome. [Updated 2024 Feb 12]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2026 Jan-.
- Tcaciuc E, Podurean M, Tcaciuc A. Management of Crigler-Najjar syndrome. Med Pharm Rep. 2021 Aug;94(Suppl No 1):S64-S67.
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