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Canadian Journal of Gastroenterology - Volume 19 2005, Issue 5, Pages 305-310

Original Article

Department of Genetics, University of British Columbia, Canada

British Columbia Transplant Society, Vancouver, Canada

Division of Medical Genetics, Department of Pathology, Victoria General Hospital, Victoria, Canada

Centre for Disease Control, Canada

Division of Gastroenterology, Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada

Copyright © 2005 Hindawi Publishing Corporation. This open-access article is distributed under the terms of the Creative Commons Attribution Non-Commercial License CC BY-NC, which permits reuse, distribution and reproduction of the article, provided that the original work is properly cited and the reuse is restricted to noncommercial purposes.


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.

RESULTS: Although available liver biopsy reports were consistent with PBC, not all cases were antimitochondrial antibody-positive 18% negative. There was a family history of PBC confirmed by medical records in 33% of cases. There were five multiplex families identified, one with seven affected individuals. Detailed family histories revealed a recurrence risk of 4% for PBC for all first-degree relatives older than 21 years of age, but 10% when considering only women. Other autoimmune conditions coexisted in PBC patients in 79% of all cases. Arthritis was most frequent 60%, with thyroid disease 16% and systemic lupus erythematosus 12% also present. Additionally, a history of autoimmune diseases arthritis, systemic lupus erythematosus and thyroid disease was present in 21% of first-degree relatives. A strong genetic predisposition to PBC and other autoimmune diseases, combined with common environmental factors, is postulated in this population. Further study is underway to identify these factors.

Autor: Laura Arbour, Rosemarie Rupps, Leigh Field, Paul Ross, Anders Erikson, Harvey Henderson, Warren Hill, and Eric M Yoshida



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