Abstract

Objective: To assess if tooth discoloration is a novel side effect of sulfonylurea therapy in patients with permanent neonatal diabetes due to mutations in KCNJ11.

Methods: Sixty-seven patients with a known KCNJ11 mutation who had been successfully transferred from insulin injections onto oral sylfonylureas were contacted and asked about the development of tooth discoloration following transfer.

Results: Altered tooth appearance was identified in 5 of the 67 patients. This was variable in severity, ranging from mild discoloration/staining (n=4) to loss of enamel (n=1), and was only seen in patients taking glibenclamide (glyburide).

Conclusions: These previously unreported side effects may relate to the developing tooth and/or to the high local concentrations in the children who frequently chewed glibenclamide tablets or took it as a concentrated solution. Given the multiple benefits of sulfonylurea treatment for patients with activating KCNJ11 mutations, this association warrants further investigation but should not preclude such treatment.