Effect of empagliflozin on the oxidative burst response and intracellular MPO concentration in neutrophils. (A-F) The neutrophil oxidative burst response to PMA stimulation in the 7 patients with GSD1b and 11 healthy controls determined using a DHR assay. (A) Median ± quartiles of the percentage of responsive cells (DHR index) for the patients and controls, and (B) the individual profiles. (D) Median ± quartiles of fluorescence medium intensity (DHR index FMI) for the same neutrophils, and (E) the individual profiles. Spearman correlation analysis between plasma 1,5-AG concentration and (C) DHR index and (F) DHR index FMI. (G) Representative flow cytometry analysis for a control donor and patient 2. (H-I) Intracellular MPO concentrations determined by ELISA: median ± quartiles of (H) MPO concentration for the patients and controls, and (I) the individual profiles. (J) Spearman correlation analysis between plasma 1,5-AG concentration and intracellular MPO concentration. Statistically significant differences between patients and healthy controls (black) were determined by ordinary 1-way ANOVA for unpaired data, whereas mixed-effect analysis for paired data was used to compare values at 3 and 12 months after the start of empagliflozin treatment with values obtained before the start of treatment (blue). For clarity, only P values <.5 are shown.