Primary biliary cirrhosis: when and why does the disease develop?
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.
Floreani
A
A
Department of Surgical and Gastroenterological Sciences, University of Padova, Padua, Italy. annarosa.floreani@unipd.it
Ostuni
P A
PA
Ferrara
F
F
Guido
M
M
D000293:Adolescent; D001323:Autoantibodies / Q000097:blood; D025065:Borrelia burgdorferi / Q000276:immunology; D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007075:Immunoglobulin M / Q000276:immunology; D008105:Liver Cirrhosis, Biliary / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology*; D008928:Mitochondria / Q000276:immunology; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
D001323:Autoantibodies; D002756:Cholagogues and Choleretics; D007075:Immunoglobulin M; D014580:Ursodeoxycholic Acid