Variant of X-Linked Chronic Granulomatous Disease Revealed by a Severe Burkholderia cepacia Invasive Infection in an Infant
Figure 1
NADPH oxidase activity evaluation in PMNs. (a) Superoxide generation was measured by assaying superoxide dismutase-inhibitable cytochrome-c reduction in PNMs after adding three doses of PMA (4, 40, and 400 ng/mL), for healthy controls (C+), CGD patient (C−), and our patient (P). (b) Histograms for the flow cytometric analysis of intracellular H2O2 production, using the fluorescent 123DHR probe in PMNs from a healthy control (C+), an X-linked CGD patient (C−), the proband (P), and the mother (H), before (NS) and after stimulation with PMA (4, 40, and 400 ng/mL). (c) PMNs from C+, C−, and P were left untreated or treated with TNF-α, IL-1β, and cytochalasin b and then stimulated with fMLF. The results shown are representative of two independent experiments.
