Genetic Cholestasis Syndromes.

This article focuses on common genetic cholestatic liver syndromes, looking at genotype-phenotype correlations in conditions such as progressive familial intrahepatic cholestasis (PFIC), bile acid synthesis disorders and cholangiopathies. It discusses how molecular classification informs prognosis, malignancy risk and therapeutic response. This review also discusses current and emerging therapies, notably ileal bile acid transport inhibitors and their use in PFIC and Alagille syndrome.