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Primary sclerosing cholangitis during childhood and adolescence
Tamir Miloh and Pinar Bulut, Clinical Liver Disease Special Issue: Liver Disease in Children Volume 2, Issue 5, pages 215–218, October 2013 Primary sclerosing cholangitis (PSC) is an idiopathic, chronic hepatobiliary disorder characterized by inflammation with progressive obliterative fibrosis and focal dilatation of the intrahepatic and/or extrahepatic bile ducts which leads to biliary cirrhosis and end-stage liver disease.[1] The clinical features of PSC in children and adolescents are summarized in Table 2. PSC is more common in Caucasian male patients and has a strong association with inflammatory bowel disease (IBD), especially ulcerative colitis. In children, secondary causes of sclerosing cholangitis include Langerhans cell histiocytosis, immunodeficiencies, and cystic fibrosis. The incidence of PSC in children is 0.23 cases per 100,000 person-years compared with 1.11/100,000 in adults.[2] . . . Please click here to access the full text of this article. There is also a video presentation and an interview with the authors Successful Treatment of Recurrent Primary Sclerosing Cholangitis after Orthotopic Liver Transplantation with Oral Vancomycin
Yinka K. Davies, Cynthia J. Tsay, Dario V. Caccamo, Kathleen M. Cox, Ricardo O. Castillo, and Kenneth L. Cox Case Reports in Transplantation Volume 2013 (2013), Article ID 314292, 5 pages http://dx.doi.org/10.1155/2013/314292 Abstract Primary sclerosing cholangitis (PSC) is a progressive, cholestatic disease of the liver that is marked by inflammation of the bile ducts and damage to the hepatic biliary tree. Approximately 60–70% of patients also have inflammatory bowel disease and progression of PSC can lead to ulcerative colitis and cirrhosis of the liver. Due to limited understanding of the etiology and mechanism of PSC, the only existing treatment option is orthotopic liver transplantation (OLT); however, recurrence of PSC, after OLT is estimated to be between 5% and 35%. We discuss the successful treatment of a pediatric patient, with recurrent PSC, after OLT with oral Vancomycin ... Please click here to access to the full text of this article Association between serum IgE level and adverse clinical endpoints in primary sclerosing cholangitis6/22/2014 Association between serum IgE level and adverse clinical endpoints in primary sclerosing cholangitis
James H. Tabibian, Felicity Enders, Mohamad H. Imam, Gururaj Kolar, Keith D. Lindor, Jayant A. Talwalkar. Annals of Hepatology, 2014, May-June, Vol. 13 No. 3, 2014: 384-389 Abstract Introduction: Primary sclerosing cholangitis (PSC) is an idiopathic hepatobiliary disorder associated with an increased risk for cholangiocarcinoma (CCA) and a median survival time of 12 years. Reliable predictors of CCA and other major adverse events in PSC are currently lacking. Recently, serum IgE was found to be as- sociated with CCA in a Japanese cohort of PSC patients. Our aim in this study was to determine whether IgE levels predict time to CCA, liver transplantation, or death in a Western (USA-based) cohort of PSC pa- tints. Material and methods: Thirty-eight patients with PSC and IgE levels were identified and categorized into low or high IgE groups based on the sample median. Groups were compared with respect to clinical characteristics and adverse endpoint-free survival . . . Please click here to be taken to the full text article Autoimmune Hepatitis and Primary Sclerosing Cholangitis in Children and Adolescents.
Claudia Patricia Rojas, Rajasekhar Bodicharla, German Campuzano-Zuluaga, Lina Hernandez, and Maria Matilde Rodriguez Posted online on April 22, 2014. (doi:10.3109/15513815.2014.898721 Abstract Clinical presentation and histopathology of autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) overlap syndrome (OS) are similar, but their management is different. We conducted a pediatric retrospective cross-sectional study of 34 patients with AIH and PSC. AIH had female predominance (74%) and was lower in PSC (45%). Read More by clicking here: http://informahealthcare.com/doi/abs/10.3109/15513815.2014.898721 Posted online on April 22, 2014. (doi:10.3109/15513815.2014.898721) |
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December 2016
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