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OBJECTIVE: Sulfonylureas (SUs) are effective at controlling glycemia in permanent neonatal diabetes mellitus (PNDM) caused by KCNJ11 (Kir6.2) mutations. RESEARCH DESIGN AND METHODS: We report the case of a woman with PNDM who continued high doses of glibenclamide (85 mg/day) during her pregnancy. The baby was born preterm, and presented with macrosomia and severe hyperinsulinemic hypoglycemia requiring high-rate intravenous glucose infusion. RESULTS: Postnatal genetic testing excluded a KCNJ11 mutation in the baby. Glibenclamide was detected in both the baby's blood and the maternal milk. CONCLUSIONS: We hypothesize that high doses of glibenclamide in the mother led to transplacental passage of the drug and overstimulation of fetal β-cells, which resulted in severe hyperinsulinemic hypoglycemia in the neonate (who did not carry the mutation) and contributed to fetal macrosomia. We suggest that glibenclamide (and other SUs) should be avoided in mothers with PNDM if the baby does not carry the mutation or if prenatal screening has not been performed, while glibenclamide may be beneficial when the fetus is a PNDM carrier.

Original publication

DOI

10.2337/dc14-1247

Type

Journal article

Journal

Diabetes Care

Publication Date

12/2014

Volume

37

Pages

3333 - 3335

Keywords

Adult, Congenital Hyperinsulinism, Diabetes Mellitus, Female, Fetal Macrosomia, Glyburide, Humans, Hypoglycemic Agents, Infant, Newborn, Maternal-Fetal Exchange, Mutation, Potassium Channels, Inwardly Rectifying, Pregnancy