A prevalent pathogenic variant identified in Caucasian GSD-Ia patients is G6PC1-c.247C > T/p.R83C (hereafter referred to as G6PC1-R83C), which accounts for 32% of sequenced alleles in patients, and results in severe hypoglycemia due to lack of G6Pase-α enzyme activity4. This evidence concerns the gene G6PC1 and Hypoglycemia.