DEFECTIVE INITIATION OF METABOLIC STIMULATION IN PHAGOCYTIZING GRANULOCYTES - NEW CONGENITAL DEFECT

  • 1 January 1976
    • journal article
    • research article
    • Vol. 88  (5) , 757-768
Abstract
A boy and a girl suffering from recurrent bacterial infections, from 1 family with apparently healthy parents, were studied. The granulocytes of these patients were capable of normal ingestion of latex particles and DNA-anti-DNA immune complexes. When the metabolic changes in these granulocytes during phagocytosis of latex particles were studied, however, no stimulation of O2 consumption, superoxide production, or hexose monophosphate shunt activity could be observed. Zymosan particles were not iodinated. These findings are comparable to those found in chronic granulomatous disease. In sharp contrast to this latter disease, however, a completely normal stimulation of cell metabolism was found after phagocytosis of IgG[immunoglobulin G]-coated latex particles or IgG aggregates. Since latex and IgG-coated latex were equally well ingested, the absence of metabolic stimulation after uptake of latex must be due to a defect in the triggering of the oxidative burst. This is the 1st time that a defect in the triggering of the metabolic stimulation during phagocytosis could be demonstrated. Adherence and subsequent ingestion of particles are apparently not sufficient for the metabolic stimulation of granulocytes.