Figure 1.

(A) Flow cytometric evaluation of dihydrorhodamine-123 (DHR) assay on neutrophils in control, corresponding mother (to distinguish XL-CGD from autosomal CGD patient), and CGD patients. Unstimulated neutrophils (sky blue) showed no oxidation of dihydrorhodamine-123 (DHR) reagent to rhodamine in contrast to stimulated neutrophils (blue) by PMA. (A-1) Oxidation of DHR in patient P1 representative of autosomal recessive CGD(P1,P2,P3)/de novo X-linked CGD (P5) patient, control, and mother's sample. (A-2) Oxidation of DHR in patient P4-X-linked CGD patient, carrier mother, and control sample. (B) Flow cytometric evaluation of p47^phox and p22^phox expression on neutrophils. Median fluorescent intensities were recorded for stained (blue) and unstained (sky blue) neutrophils in control, patient, and mother's sample. (B-1) Defective p47phox component expression in patient P1, control, and mother. (B-2) Defective p22^phox component expression in patient P5, control, and mother*. (B-3) Defective p22^phox component expression in patient P4, control, and mosaic pattern in mother clearly indicating X-linked defect (gp91^phox defect) in patient P4. *Important to further confirm by molecular characterization in patient and parents.