KCNQ1 and Hypoglycemia: Channel Defects (ChD; genes ABCC8, KCNJ11, KCNQ1, CACNA1D): among these mutations, ABCC8 and KCNJ11 (KATP channel subunits Kir6.2 and SUR1, respectively) causes the most common and severe forms of CH especially in case of biallelic mutation, although there have been reported patients carrying ABCC8 biallelic mutations with optimal response to Diazoxide (DZX) (33, 34) even showing progressive resolution of hypoglycemia (35).