Giorgina Mieli-Vergani1, Diego Vergani. 1. Paediatric Liver Centre, Institute of Liver Studies, King's College London School of Medicine at King's College Hospital, London, UK. giorgina.vergani@kcl.ac.uk
Abstract
PURPOSE OF REVIEW: To summarize publications on juvenile primary sclerosing cholangitis (PSC) published over the past 5 years. These studies contribute to the understanding of the clinical features, diagnostic pathways, genetic aspects, treatment and outcome of this condition. RECENT FINDINGS: Sclerosing cholangitis with strong autoimmune features is particularly frequent in paediatric age, where it is more common in girls, responds to immunosuppressive treatment and has a better prognosis than classical PSC. Modern-day magnetic resonance cholangiography allows accurate diagnosis of bile duct disease in most cases. Prolonged oral vancomycin treatment may be beneficial in difficult-to-treat PSC associated with inflammatory bowel disease. Juvenile PSC has a high recurrence rate after liver transplantation. PSC susceptibility and resistance are associated with both human leucocyte antigen-related and unrelated genetic factors. SUMMARY: Studies on large cohorts of children with PSC are needed for a better understanding of pathogenic mechanisms, response to treatment and outcome of this serious condition.
PURPOSE OF REVIEW: To summarize publications on juvenile primary sclerosing cholangitis (PSC) published over the past 5 years. These studies contribute to the understanding of the clinical features, diagnostic pathways, genetic aspects, treatment and outcome of this condition. RECENT FINDINGS:Sclerosing cholangitis with strong autoimmune features is particularly frequent in paediatric age, where it is more common in girls, responds to immunosuppressive treatment and has a better prognosis than classical PSC. Modern-day magnetic resonance cholangiography allows accurate diagnosis of bile duct disease in most cases. Prolonged oral vancomycin treatment may be beneficial in difficult-to-treat PSC associated with inflammatory bowel disease. Juvenile PSC has a high recurrence rate after liver transplantation. PSC susceptibility and resistance are associated with both human leucocyte antigen-related and unrelated genetic factors. SUMMARY: Studies on large cohorts of children with PSC are needed for a better understanding of pathogenic mechanisms, response to treatment and outcome of this serious condition.
Authors: David N Abarbanel; Scott M Seki; Yinka Davies; Natalie Marlen; Joseph A Benavides; Kathleen Cox; Kari C Nadeau; Kenneth L Cox Journal: J Clin Immunol Date: 2012-10-09 Impact factor: 8.317
Authors: Yinka K Davies; Cynthia J Tsay; Dario V Caccamo; Kathleen M Cox; Ricardo O Castillo; Kenneth L Cox Journal: Case Rep Transplant Date: 2013-02-24