Characteristics of primary biliary cirrhosis in British Columbia's First Nations population

Primary biliary cirrhosis (PBC) is a rare, autoimmune liver disorder characterized by progressive destruction of intrahepatic bile ducts, that results in portal inflammation, scarring, cirrhosis and, eventually, liver failure. Although considered rare in Canadian populations, it is the leading indic...

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Bibliographic Details
Published in:Canadian Journal of Gastroenterology
Main Authors: Laura Arbour, Rosemarie Rupps, Leigh Field, Paul Ross, Anders Erikson, Harvey Henderson, Warren Hill, Eric M Yoshida
Format: Article in Journal/Newspaper
Language:English
Published: Hindawi Limited 2005
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Online Access:https://doi.org/10.1155/2005/203028
https://doaj.org/article/e4a5d6396a864c9494f8b752d36c1ba0
Description
Summary:Primary biliary cirrhosis (PBC) is a rare, autoimmune liver disorder characterized by progressive destruction of intrahepatic bile ducts, that results in portal inflammation, scarring, cirrhosis and, eventually, liver failure. Although considered rare in Canadian populations, it is the leading indication for referral for liver transplantation in British Columbia's First Nations population. Previously, an expanded review of all cases referred to the British Columbia Transplant Society for PBC was carried out comparing the demographics of those of First Nations descent with those not of First Nations descent. The review suggested that the rate of referral for transplantation was eight times higher for those of First Nations descent compared with those of other descent (P=0.0001), and a disproportionate number of the First Nations cases lived on Vancouver Island (48% of cases versus 18% expected, P<0.05). Additionally, the age of referral was significantly younger (45.9 versus 54.3 years) for those of First Nations descent and there are fewer First Nations men referred (1:34) than expected. For the purpose of the present report, 28 symptomatic cases were ascertained separately and reviewed in a clinical study to delineate the features of this population.