CANINE GRANULOCYTOPATHY SYNDROME - INHERITED DISORDER OF LEUKOCYTE FUNCTION

  • 1 January 1979
    • journal article
    • research article
    • Vol. 95  (3) , 731-744
Abstract
A disease closely resembling the human neutrophil dysfunction syndromes was identified in a colony of dogs. The syndrome, referred to as the canine granulocytopathy syndrome, was characterized by recurrent life-threatening bacterial infections and a greatly shortened life span. The disease was genetically determined, being transmitted as an autosomal recessive trait. The increased susceptibility to pyrogenic infections and shortened life span was related to an impairment of leukocyte function at the cellular level. Preparations of neutrophils from affected animals exhibit impaired in vitro killing of Escherichia coli. The defect in bactericidal activity was associated with reduced glucose oxidation by the hexose monophosphate shunt and an increased capacity to reduce nitroblue tetrazolium dye. The canine granulocytopathy syndrome will be of considerable value as a model for the study of granulocytopathy syndromes in man.