About this Journal Submit a Manuscript Table of Contents
Case Reports in Pediatrics
Volume 2012 (2012), Article ID 937150, 3 pages
http://dx.doi.org/10.1155/2012/937150
Case Report

Twelve-Year-Old Girl with Primary Biliary Cirrhosis

Department of Gastroenterology and Hepatology, Mother and Child Health Care Institute, Radoja Dakica 6-8, Belgrade, 11070 New Belgrade, Serbia

Received 10 October 2012; Accepted 18 November 2012

Academic Editors: K. L. Chan, P. Czauderna, A. W. Kamps, and A. C. Lee

Copyright © 2012 Ivana Kitic et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Primary biliary cirrhosis (PBC) is a slowly progressive cholestatic liver disease of autoimmune etiology. The initial presentation of PBC is varies from asymptomatic, abnormal liver biochemical tests to overt cirrhosis. Unlike other autoimmune liver diseases, PBC has rarely been reported in childhood. We report a case of primary biliary cirrhosis in a 12-year-old girl. In addition to characteristic histology features, strongly positive antimitochondrial antibodies, increased liver enzyme levels, increased serum quantitative immunoglobulin M levels, and cholestasis were discovered. She had been treated with ursodeoxycholic acid. In the world literature, we found only few pediatric patients of primary biliary cirrhosis. Aetiology, pathogenesis, the long-term natural history, and prognosis remain obscure. Due to increased awareness of early-onset PBC, rather than typical older ones, further pediatric cases may be discovered.