Defective Polymorphonuclear-Leukocyte Function and Chronic Granulomatous Disease in Two Female Children

In vitro studies of polymorphonuclear-leukocyte function in two female patients with clinical and pathological features of chronic granulomatous disease of childhood showed defective bactericidal capacity and abnormalities of metabolic response to phagocytosis. There was no clinical or laboratory evidence of leukocytic abnormalities in the family members of these girls. All patients with this syndrome previously described have been males. The syndrome in males is familial, and its transmission is X linked. The studies of leukocytic function in these girls demonstrate that defective bactericidal function and metabolic abnormalities of polymorphonuclear leukocytes associated with chronic granulomatous disease are not necessarily familial and are not limited to male patients with X-linked hereditary disease.