Figure 2.
Empagliflozin lowers plasma 1,5AG and intracellular 1,5AG6P and corrects neutropenia in GSD-Ib patients. (A) Time course of ANCs before and during empagliflozin treatment in 4 GSD-Ib patients. The top part of the right scale refers to the concentration of GCSF, and the bottom to empagliflozin. (B) Decrease in the concentration of plasma 1,5AG determined by liquid chromatography–mass spectrometry (LC-MS) during treatment. (C) Neutrophil 1,5AG6P determined in blood cells after centrifugation to remove plasma (for determination of 1,5AG) and normalized to total metabolite content (total ion current [TIC])14 and ANC. (D) 1,5AG6P in isolated granulocytes (polymorphonuclear neutrophils [PMNs]) and peripheral blood mononuclear cells (PBMCs) obtained from patient 1 (PT1) 1 day before and 28 or 50 days into empagliflozin treatment and compared with levels found in a healthy control (CT).