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 inExpert review of gastroenterology & hepatology Vol. 9; no. 3; p. 289
Main Authors Venkat, Veena L, Ranganathan, Sarangarajan, Sindhi, Rakesh
Format Journal Article
LanguageEnglish
Published England 01.03.2015
Subjects
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
PSC
transplant outcome
autoimmune liver disease
children
liver transplant
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Summary: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.
ISSN:1747-4132
DOI:10.1586/17474124.2015.1002085