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Primary biliary cirrhosis: when and why does the disease develop?
A Floreani1, P A Ostuni, F Ferrara
1Department of Surgical and Gastroenterological Sciences, University of Padova, Padua, Italy. annarosa.floreani@unipd.it
Abstract:
Primary biliary cirrhosis is a chronic cholestatic liver disease with an autoimmune pathogenesis, that generally develops in adult life, often in perimenopausal age. The clinical features are heterogeneous, ranging from an asymptomatic presentation to end-stage liver disease. Primary biliary cirrhosis is unknown in children and its natural history has yet to be elucidated. Following a Canadian report of primary biliary cirrhosis in two girls (16 and 15 years old), we describe a clinical case developing at 17 years of age. A temporal association between Borrelia Burgdorferi infection and diagnosis of primary biliary cirrhosis was observed.
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