Aims Does the the early initiation of sulphonylurea treatment have an effect on the duration and control of transient neonatal diabetes caused by a Kir6.2 mutation.
Methods We identified 2 siblings born to a diabetic mother affected by a E227K mutation which is associated with neonatal diabetes. Both siblings developed neonatal diabetes, the elder child (born in 2009) was treated with a combination of subcutaneous and intravenous insulin, the younger sibling (born in 2015) was initially commenced on intravenous insulin but transferred to an oral sulphonylurea after the genetic diagnosis was confirmed.
Results Both children showed substantial fluctuations in their blood sugars when treated with subcutaneous/intravenous insulin in the neonatal period.
The elder sibling was converted to a twice daily insulin regime and then to a once daily long acting insulin due to issues with hypoglycaemia and blood glucose control. By the age of 6 months the insulin dose had been weaned and diabetes had resolved.
When the younger sibling was commenced on an oral sulphonylurea, her blood glucose stabilised quickly and the dose required rapid reduction to prevent hypoglycaemia. This patient was able to stop treatment after 41 days and their blood glucose has remained within normal limits
Conclusions The sibling commenced on oral sulphonylurea had rapid resolution of transient neonatal diabetes and their blood glucose was stabilised more efficiently when on that treatment. In comparison, her sibling had a much more prolonged period of diabetes with much more fluctuations in blood glucose. Diabetes caused by a E227K mutation is known to respond well to sulphonylureas treatment and this case suggests early introduction of sulphonylureas may reduce the duration of neonatal diabetes.
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