Unique features of primary sclerosing cholangitis in children.

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.

authors

Mieli-Vergani G,Vergani D

doi

10.1097/MOG.0b013e3283388f5b

subject

Has Abstract

pub_date

2010-05-01 00:00:00

pages

265-8

issue

3

eissn

0267-1379

issn

1531-7056

pii

00001574-201005000-00014

journal_volume

26

pub_type

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