Hypoglycemia Infrequent in Sulfonylurea-Treated KCNJ11-Related Neonatal Diabetes

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A wide range of both neuroglycopenic and adrenergic symptoms of hypoglycemia were reported among subjects.
A wide range of both neuroglycopenic and adrenergic symptoms of hypoglycemia were reported among subjects.

Sulfonylurea appears to be associated with low rates of hypoglycemia in patients with well-controlled KCNJ11-related permanent neonatal diabetes, according to study findings published in Pediatric Diabetes.

Researchers obtained data from patients with KCNJ11-related permanent neonatal diabetes (n=30) from the University of Chicago Monogenic Diabetes Registry as well as data from parent/caregiver questionnaires reporting hypoglycemia frequency and severity from the initiation of sulfonylurea treatment. Additionally, investigators collected data on continuous glucose monitoring in 7 participants.

The median dose of sulfonylurea in the participants with KCNJ11-related permanent neonatal diabetes was 0.39 mg/kg/day (0.24-0.88 interquartile range). Of these subjects, the median hemoglobin A1c level was 5.7% (39 mmol/mol) (5.5-6.1 interquartile range, 37-43 mmol/mol). The rates of hypoglycemia, defined as a blood glucose level <70 mg/dL, was infrequent in the majority of cases (89.3%).

A total of 3 patients (10.7%), however, reported incidences of hypoglycemia once a week or more. No hypoglycemic episodes involved unconsciousness or seizures. In 7 patients using continuous glucose monitoring, blood sugar levels <70 mg/dL occurred 5.8% of the time. The investigators reported that these ranges are similar to patients without a diabetes diagnosis.

Because this study was conducted before 2014 and prior to the change of the definition of severe hypoglycemia, the findings are limited in their generalizability to current clinical practice. In addition, because patient information was based on reports of parents and caregivers, the data were possibly vulnerable to recall bias compared with data collected from medical records or glucose monitoring devices.

According to the investigators, the infrequency of true severe hypoglycemia in KCNJ11-related permanent neonatal diabetes, “may help to reassure healthcare providers prescribing high doses of sulfonylurea to this unique group of patients.”

Reference

Lanning MS, Carmody D, Szczerbiński Ł, Letourneau LR, Naylor RN, Greeley SAW. Hypoglycemia in sulfonylurea-treated KCNJ11-neonatal diabetes: mild-moderate symptomatic episodes occur infrequently but none involving unconsciousness or seizures [published online December 5, 2017]. Pediatr Diabetes. doi: 10.1111/pedi.12599

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