Hemostasis in the copper-laden Bedlington terrier: a possible model of Wilson's disease

A hemostatic survey was done on 14 Bedlington terriers, 13 of which have the recently discovered copper toxicosis. Their hepatic copper ranged from 109 to 9,888 microgram/g dry weight and their ages from 8 months to 8 years. Despite histologic evidence of hepatitis in younger dogs and cirrhosis in older ones, plasmatic coagulation factors were not depressed. In fact, the hemophilic factors VIII, IX and XI were above normal, more closely related to the age of the dog than to the hepatic copper. Furthermore, their platelet were unusually sensitive to adenosine diphosphate exposure. Offsprings of matings between Bedlington terriers and Beagles seem to be normal.