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Clin Liver Dis. 2016 Feb;20(1):99-111. doi: 10.1016/j.cld.2015.08.008. Epub 2015 Oct 6.

Sclerosing Cholangitis in Children and Adolescents.

Author information

1
Paediatric Liver, GI and Nutrition Centre, King's College Hospital, Denmark Hill, London SE5 9RS, UK. Electronic address: giorgina.vergani@kcl.ac.uk.
2
Institute of Liver Studies, King's College Hospital, Denmark Hill, London SE5 9RS, UK.

Abstract

Sclerosing cholangitis in pediatric age is a severe disease, often associated with inflammatory bowel disease. It recognizes different etiologies. Management and prognosis depend on the underlying cause. A high proportion of patients have autoimmune features similar to those of autoimmune hepatitis and respond biochemically to immunosuppression, although bile duct disease progresses in half of them leading to liver transplant. The disease can recur after transplant. Severity of liver disease and risk of recurrence after transplant are linked to the severity of bowel disease.

KEYWORDS:

Adolescents; Autoimmune hepatitis; Autoimmune sclerosing cholangitis; Children; Inflammatory bowel disease; Juvenile sclerosing cholangitis; Liver transplant; Primary sclerosing cholangitis

PMID:
26593293
DOI:
10.1016/j.cld.2015.08.008
[Indexed for MEDLINE]

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