An overview of paediatric autoimmune and genetic cholestatic liver disease for the adult physician

Paediatric chronic liver diseases are rare, but are increasingly encountered in adult practice as survival improves and more adolescents transition to adult services. Adult physicians must be familiar with these conditions, recognise complications and implement high-quality management of these conditions to provide safe, effective care. Autoimmune liver disease in children includes autoimmune hepatitis, autoimmune sclerosing cholangitis and primary sclerosing cholangitis, with distinct serological and clinical profiles. Cholestatic disorders such as progressive familial intrahepatic cholestasis and Alagille syndrome present additional challenges, often impacting multiple organs and requiring multidisciplinary care. Many patients will enter adulthood having undergone liver transplant, requiring long-term immunosuppression and, where relevant, family planning advice. Recognition of extra-hepatic manifestations, metabolic complications and mental health issues is essential to holistic management. This article outlines the key paediatric liver diseases relevant to adult practice, highlighting key elements of care and long-term considerations for this unique and often complex patient population.