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Pediatric Primary Sclerosing Cholangitis

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Primary Sclerosing Cholangitis

Abstract

Primary sclerosing cholangitis (PSC) is a rare disorder of the hepatobiliary system characterized by chronic diffuse inflammation and obliterative fibrosis of the intrahepatic and/or extrahepatic bile ducts that subsequently progresses to liver cirrhosis and end-stage liver disease in the majority of patients. The estimated incidence and prevalence of pediatric PSC in the United States is 0.2 and 1.5 cases per 100,000 children. The majority of cases of pediatric PSC are symptomatic at presentation and common manifestations include fatigue, abdominal pain, anorexia, and pruritus. Approximately 30–40 % of pediatric PSC patients will suffer from consequences of chronic biliary disease, including significant pruritus, recurrent bacterial cholangitis, and complications of portal hypertension. Unique aspects of pediatric PSC include a high incidence of autoimmune sclerosing cholangitis (PSC-autoimmune hepatitis overlap syndrome) and small duct PSC. Cholangiocarcinoma is an extremely rare complication of pediatric PSC. There is no known therapy to prevent progression of pediatric PSC; however further research on the effectiveness of ursodeoxycholic acid and oral vancomycin therapies is warranted. Liver transplantation is a viable option for end-stage liver disease secondary to pediatric PSC and accounts for ~2 % of all pediatric liver transplants in the United States.

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Abbreviations

AIH:

Autoimmune hepatitis

ALP:

Alkaline phosphatase

ALT:

Alanine aminotransferase

ANA:

Antinuclear antibody

ASC:

Autoimmune sclerosing cholangitis

ASMA:

Anti-smooth muscle antibody

AST:

Aspartate aminotransferase

CCA:

Cholangiocarcinoma

CD:

Crohn’s disease

ERCP:

Endoscopic retrograde cholangiopan-creatography

GGTP:

Gamma-glutamyl transpeptidase

IBD:

Inflammatory bowel disease

LCH:

Langerhans cell histiocytosis

LKM:

Liver-kidney microsomal antibody

MRCP:

Magnetic resonance cholangiopan-creatography

OV:

Oral vancomycin

PSC:

Primary sclerosing cholangitis

SSC:

Secondary sclerosing cholangitis

UC:

Ulcerative colitis

UDCA:

Ursodeoxycholic acid

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Authors and Affiliations

  1. Section of Pediatric Gastroenterology, Hepatology and Nutrition, Children’s Hospital Colorado, University of Colorado School of Medicine, 13123 E. 16th Ave., B290, Aurora, CO, 80045, USA

    Dania Molla-Hosseini MD & Cara L. Mack MD

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  1. Dania Molla-Hosseini MD
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  2. Cara L. Mack MD
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Correspondence to Dania Molla-Hosseini MD .

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Editors and Affiliations

  1. Department of Gastroenterology-Hepatology, University of Colorado, Aurora, Colorado, USA

    Lisa M. Forman

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Molla-Hosseini, D., Mack, C.L. (2017). Pediatric Primary Sclerosing Cholangitis. In: Forman, L. (eds) Primary Sclerosing Cholangitis. Springer, Cham. https://doi.org/10.1007/978-3-319-40908-5_6

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  • DOI: https://doi.org/10.1007/978-3-319-40908-5_6

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