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Keywords Chronic.Durability.Efficacy.Glibenclamide.KCNJ11 gene.Kir6.2.Mutations.Permanent neonataldiabetes.Sulfonylurea.TherapyAbbreviationsDEND Developmental delay, epilepsy and neonataldiabetesiDEND Intermediate DENDTo the Editor: Patients with permanent neonatal- orinfancy-onset diabetes mellitus associated with activatingmutations of KCNJ11 and ABCC8 genes, which encodethe ATP-sensitive potassium channel of the pancreatic betacell, can be successfully weaned from insulin andtransferred to sulfonylureas [1, 2]. Individuals carryingspecific mutations of KCNJ11 or ABCC8 may present withdevelopmental delay, epilepsy and neonatal diabetes(DEND) syndrome or intermediate DEND (iDEND), i.e.may have, in additionto diabetes, motor/mental developmen-tal delay with or without epilepsy. Notably, patients withDEND may require higher doses of sulfonylureas to attainproper metabolic control [3]. Glibenclamide, a long-actingsulfonylurea largely used in the therapy of type 2 diabetes, isoften used to treat patients with neonatal- or infancy-onsetdiabetes due to KCNJ11 or ABCC8 mutations [1–4].

No beta cell desensitisation after a median of 68 months on glibenclamide therapy in patients with KCNJ11-associated permanent neonatal diabetes