Sulfonylurea Use During Entire Pregnancy in Diabetes Because of KCNJ11 Mutation: A Report of Two Cases

Mutations of the KCNJ11 gene are a common cause of permanent neonatal diabetes (PNDM) (1,2) and sometimes result in other diabetic phenotypes (2). Sulfonylureas (SUs) are effective and safe in most diabetic KCNJ11 mutation carriers (3). However, their application risk is sometimes uncertain. We have previously described glibenclamide use in a pregnant woman with KCNJ11 -related PNDM (4); for the first time, we report two cases treated with SU throughout the entire pregnancy. The first case was a Hungarian woman with the E229 K KCNJ11 mutation resulting in relapsing neonatal diabetes. The patient experienced remission between the ages of 3 and 10 years, at which point insulin was restarted. At the age of 13, after genetic testing, the patient was switched to gliclazide 60 mg/day. The woman became pregnant at the age of 16 years. When she was referred …