Hypoglycemia in sulfonylurea‐treated KCNJ11 ‐neonatal diabetes: Mild‐moderate symptomatic episodes occur infrequently but none involving unconsciousness or seizures

Abstract

In our cohort of KCNJ11-related permanent NDM, hypoglycemia is infrequent and mild despite the high doses of sulfonylurea used and near-normal level of glycemic control. Long-term follow-up on larger numbers will be required to clarify the incidence and determinants of hypoglycemia in this unique population.

Update of variants identified in the pancreatic β‐cell K ATP channel genes KCNJ11 and ABCC8 in individuals with congenital hyperinsulinism and diabetes

“…Patients who are unable to transfer to sulphonylureas tend to have a longer duration of diabetes before attempting transfer or functionally severe mutations (Babiker et al, 2016; Thurber et al, 2015). Few side effects and no episodes of severe hypoglycemia involving seizures or loss of consciousness have been reported in individuals with sulphonylurea‐treated neonatal diabetes (Bowman, Sulen, et al, 2018; Codner, Flanagan, Ellard, Garcia, & Hattersley, 2005; Kumaraguru et al, 2009; Lanning et al, 2018).…”

Update of variants identified in the pancreatic β-cell KATPchannel genes KCNJ11 and ABCC8 in individuals with congenital hyperinsulinism and diabetes

“…Patients who are unable to transfer to sulphonylureas tend to have a longer duration of diabetes before attempting transfer or functionally severe mutations (Babiker et al, 2016;Thurber et al, 2015). Few side effects and no episodes of severe hypoglycemia involving seizures or loss of consciousness have been reported in individuals with sulphonylurea-treated neonatal diabetes (Bowman, Sulen, et al, 2018;Codner, Flanagan, Ellard, Garcia, & Hattersley, 2005;Kumaraguru et al, 2009;Lanning et al, 2018).…”

Congenital Diabetes: Comprehensive Genetic Testing Allows for Improved Diagnosis and Treatment of Diabetes and Other Associated Features

“…A recent study from our Monogenic Diabetes Registry sought to address how frequently hypoglycemia occurs in patients with KCNJ11 -related congenital diabetes [14••]. We collected subject- or caregiver-reported survey data (n =30), as well as continuous glucose monitoring data (available for seven participants).…”