The challenges of liver transplantation in children with primary sclerosing cholangitis
Primary sclerosing cholangitis (PSC) in children can progress to end-stage liver disease requiring liver transplantation. PSC poses many challenges beginning with evaluation and classification of ductal involvement and overlap syndromes, few options for medical management and unique risks in the pos...
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Published in | Expert review of gastroenterology & hepatology Vol. 9; no. 3; p. 289 |
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Main Authors | , , |
Format | Journal Article |
Language | English |
Published |
England
01.03.2015
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Abstract | Primary sclerosing cholangitis (PSC) in children can progress to end-stage liver disease requiring liver transplantation. PSC poses many challenges beginning with evaluation and classification of ductal involvement and overlap syndromes, few options for medical management and unique risks in the post-transplant period. The construct that PSC may be an autoimmune disease is based on positive autoantibodies, association with inflammatory bowel disease, linkage to HLA type and overlap/autoimmune sclerosing cholangitis; however, PSC is not responsive to standard immunosuppression. Study of PSC and post-transplant outcomes in children may provide a unique background in which to study this challenging disease. This is particularly intriguing in the subset of patients diagnosed in the first decade of life, suggesting a strong link to predisposing genetic susceptibility and immune dysregulation. Long-term, multicenter effort is likely to be the only mechanism to study this rare disease in children and to improve outcomes in the future. |
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AbstractList | Primary sclerosing cholangitis (PSC) in children can progress to end-stage liver disease requiring liver transplantation. PSC poses many challenges beginning with evaluation and classification of ductal involvement and overlap syndromes, few options for medical management and unique risks in the post-transplant period. The construct that PSC may be an autoimmune disease is based on positive autoantibodies, association with inflammatory bowel disease, linkage to HLA type and overlap/autoimmune sclerosing cholangitis; however, PSC is not responsive to standard immunosuppression. Study of PSC and post-transplant outcomes in children may provide a unique background in which to study this challenging disease. This is particularly intriguing in the subset of patients diagnosed in the first decade of life, suggesting a strong link to predisposing genetic susceptibility and immune dysregulation. Long-term, multicenter effort is likely to be the only mechanism to study this rare disease in children and to improve outcomes in the future. |
Author | Venkat, Veena L Sindhi, Rakesh Ranganathan, Sarangarajan |
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SubjectTerms | Autoimmune Diseases - complications Autoimmune Diseases - genetics Autoimmune Diseases - immunology Autoimmune Diseases - surgery Child Child, Preschool Cholangitis, Sclerosing - complications Cholangitis, Sclerosing - genetics Cholangitis, Sclerosing - immunology Cholangitis, Sclerosing - surgery End Stage Liver Disease - etiology End Stage Liver Disease - surgery Genetic Predisposition to Disease Hepatitis, Autoimmune - complications Humans Infant Inflammatory Bowel Diseases - complications Liver Transplantation Recurrence Treatment Outcome |
Title | The challenges of liver transplantation in children with primary sclerosing cholangitis |
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