ArticlePediatric diabetes2018
Hypoglycemia in sulfonylurea-treated KCNJ11-neonatal diabetes: Mild-moderate symptomatic episodes occur infrequently but none involving unconsciousness or seizures.
Article in Pediatric diabetes, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed, 26 citations in OpenAlex.
- Knowledge, Attitudes, and Practices in Neonatal Diabetes Mellitus Management: the JEnious-NeOnatal-DIabetes (JENODI) Survey.Diabetes therapy : research, treatment and education of diabetes and related disorders · 2025Article
- Long-Term Effects of Incretin-Based Drugs on Glycemic Control in Permanent Neonatal Diabetes.JCEM case reports · 2024Article
- ISPAD Clinical Practice Consensus Guidelines 2022: The diagnosis and management of monogenic diabetes in children and adolescents.Pediatric diabetes · 2022Article
- Achievements, prospects and challenges in precision care for monogenic insulin-deficient and insulin-resistant diabetes.Diabetologia · 2022Review
- Growth and development in monogenic forms of neonatal diabetes.Current opinion in endocrinology, diabetes, and obesity · 2022Review
- Monogenic Diabetes: From Genetic Insights to Population-Based Precision in Care. Reflections From aDiabetes care · 2020Review
- Article
- Update of variants identified in the pancreatic β-cell KHuman mutation · 2020Review
- Uncommon Presentations of Diabetes: Zebras in the Herd.Clinical diabetes : a publication of the American Diabetes Association · 2020Article
- Precision Medicine: Long-Term Treatment with Sulfonylureas in Patients with Neonatal Diabetes Due to KCNJ11 Mutations.Current diabetes reports · 2019Review
- Patterns of postmeal insulin secretion in individuals with sulfonylurea-treatedBMJ open diabetes research & care · 2019Article
- Precision medicine in KCNJ11 permanent neonatal diabetes.The lancet. Diabetes & endocrinology · 2018Article
- Monogenic diabetes: the impact of making the right diagnosis.Current opinion in pediatrics · 2018Review
- Congenital Diabetes: Comprehensive Genetic Testing Allows for Improved Diagnosis and Treatment of Diabetes and Other Associated Features.Current diabetes reports · 2018Review
- Congenital forms of diabetes: the beta-cell and beyond.Current opinion in genetics & development · 2018Review
- Future Roadmaps for Precision Medicine Applied to Diabetes: Rising to the Challenge of Heterogeneity.Journal of diabetes research · 2018Review
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Authors and funding
6 authors at 2 institutions in 2 countries.
Funding
Abstract
backgroundNeonatal diabetes mellitus (NDM) caused by mutations in KCNJ11 can be successfully treated with high dose oral sulfonylureas; however, little data is available on the risk of hypoglycemia.
objectiveTo determine the frequency, severity, and clinical significance of hypoglycemia in KCNJ11-related NDM.
methodsUtilizing the University of Chicago Monogenic Diabetes Registry, parents completed an online questionnaire addressing hypoglycemia. Continuous glucose monitoring (CGM) data was available for 7 subjects.
resultsThirty subjects with KCNJ11-related permanent NDM (166 patient-years on sulfonylurea) had median sulfonylurea dose of 0.39 mg/kg/day (0.24-0.88 IQR, interquartile range) with median HbA1c 5.7% (39 mmol/mol) (5.5-6.1 IQR, 37-43 mmol/mol). Hypoglycemia (<70 mg/dL) was reported monthly once or less frequently in 89.3% of individuals, but 3 (10.7%) reported once weekly or more. Of all hypoglycemic episodes reported, none involved seizures or unconsciousness and thus did not meet the current ISPAD definition of severe hypoglycemia. Seven individuals wore a CGM for a total of 912 hours with blood sugars falling below 70 mg/dL for 5.8% of the time recorded, similar to ranges reported for people without diabetes.
conclusionsIn 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.
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