Abstract:
Background: The natural history of pediatric-onset primary sclerosing cholangitis (PSC) and overlap with autoimmune hepatitis (PSC/AIH) is poorly known.
Objective: The aim of this study was to evaluate the clinical outcome of patients with pediatric-onset disease in a tertiary referral center.
Methods: We traced 33 patients (median age at diagnosis 16 years), with PSC or PSC/AIH in cholangiography and liver histology diagnosed between December 1993 and 2011, at Helsinki University Hospital. Diagnostic procedures and long-term follow-up were reassessed until the end of December 2013.
Results: PSC was confirmed in all 33 patients; 19 of them had an overlap with AIH. At diagnosis, three of 33 had cirrhosis. Inflammatory bowel disease (IBD) was associated in 76% of the patients, mostly ulcerative colitis (70%); treatment of IBD being a minor determinant of the clinical outcome of liver disease ... please click here for the remainder of the article which is freely available..
Andrea Tenca, Martti Fa ̈rkkila ̈, Johanna Arola, Tytti Jaakkola, Roberto Penagini and Kaija-Leena Kolho
United European Gastroenterology JournalDOI: 10.1177/2050640615616012
Background: The natural history of pediatric-onset primary sclerosing cholangitis (PSC) and overlap with autoimmune hepatitis (PSC/AIH) is poorly known.
Objective: The aim of this study was to evaluate the clinical outcome of patients with pediatric-onset disease in a tertiary referral center.
Methods: We traced 33 patients (median age at diagnosis 16 years), with PSC or PSC/AIH in cholangiography and liver histology diagnosed between December 1993 and 2011, at Helsinki University Hospital. Diagnostic procedures and long-term follow-up were reassessed until the end of December 2013.
Results: PSC was confirmed in all 33 patients; 19 of them had an overlap with AIH. At diagnosis, three of 33 had cirrhosis. Inflammatory bowel disease (IBD) was associated in 76% of the patients, mostly ulcerative colitis (70%); treatment of IBD being a minor determinant of the clinical outcome of liver disease ... please click here for the remainder of the article which is freely available..
Andrea Tenca, Martti Fa ̈rkkila ̈, Johanna Arola, Tytti Jaakkola, Roberto Penagini and Kaija-Leena Kolho
United European Gastroenterology JournalDOI: 10.1177/2050640615616012
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