Abstract
This report describes three children, two boys and one girl, with the clinical manifestations of chronic granulomatous disease of childhood (CGD), a disease thought to be transmitted as an X-linked recessive trait. In vitro studies of neutrophils from the patients showed a defect characteristic of CGD, marked impairment of the ability to kill opsonized bacteria. The ability of the neutrophils to ingest bacteria was normal, and no structural or morphologic abnormalities could be discerned by electron microscopy. Heterozygosity for the neutrophil defect was demonstrated in the mother of one patient; however, this boy had a deficiency of serum IgA. A heterozygous state was not demonstrable in the parents or first-degree relatives of the other boy or the girl. The findings in these patients suggest that several types of neutrophil dysfunction syndromes exist. A proposed classification of neutrophil dysfunction syndromes, based on the present cases and other cases reported in the literature, is presented.
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