The mystery of primary biliary cirrhosis in British Columbia's First Nations people.
OBJECTIVE
Primary biliary cirrhosis (PBC) is a rare chronic, progressive liver disorder leading to transplantation or death, with a known autoimmune basis. Although it has been estimated to have a prevalence of between 2-5 cases per 100,000 worldwide, it is not rare in British Columbia's (BC) First Nations (FN) peoples, where it is the leading indication for liver transplant in that population. A study of indications for liver transplant from 1989 to 1998 demonstrated that although just 3.9% of the population of BC is of FN descent, 25% of those requiring transplantation for PBC were of FN descent. From this, it can be calculated that PBC is about 8 times more common than in the non-native population.


METHODS
We studied the prevalence of PCB on Vancouver Island, where about 50% of the cases on the transplant Society List are reported.


CONCLUSIONS
As with many autoimmune diseases, PBC affects women predominantly. Genetic predisposition and environmental factors are thought to contribute to the development of PBC. A strong genetic component is postulated in the BC population where 6 affected individuals are all related through common great grandparents. Nonetheless, both genetic and environmental components are being explored in this unique population.
Arbour
Laura
L
University of British Columbia, Department of Medical Genetics, Vancouver, Canada. larbour@cw.bc.ca
Field
Leigh
L
Ross
Paul
P
Erikson
Anders
A
Yoshida
Eric
E
D001955:British Columbia / Q000453:epidemiology; D006801:Humans; D007198:Indians, North American / Q000706:statistics & numerical data*; D008105:Liver Cirrhosis, Biliary / Q000453:epidemiology* / Q000208:ethnology