Sclerosing cholangitis in the paediatric patient

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    36 Citations (Scopus)

    Abstract

    Sclerosing cholangitis in childhood is a heterogeneous condition, which has different aetiologies. Sclerosing cholangitis may be inherited and diagnosed in the neonatal period (neonatal sclerosing cholangitis); it may present later with features of autoimmunity (autoimmune sclerosing cholangitis); or it may be associated with a variety of disorders, including Langerhans cell histiocytosis, immunodeficiency, psoriasis, cystic fibrosis, reticulum cell sarcoma and sickle cell anaemia. In contrast to the experience in adult patients, sclerosing cholangitis occurring as an individual disease (primary sclerosing cholangitis) is rare. The initiating events and possible pathogenic mechanisms differ in the various forms of sclerosing cholangitis and are still obscure. Treatment and prognosis depend on the type of sclerosing cholangitis present.
    Original languageEnglish
    Pages (from-to)681 - 690
    Number of pages10
    JournalBEST PRACTICE AND RESEARCH CLINICAL GASTROENTEROLOGY
    Volume15
    Issue number4
    DOIs
    Publication statusPublished - 2001

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