Neonatal diabetes (monogenic forms)

Group of rare monogenic forms of diabetes presenting in the first 6 months of life. About half are caused by mutations in KCNJ11 and ABCC8 encoding ATP-sensitive potassium channel subunits of the β-cell.

ICD-10 P70.21 drug

Also known as: Monogenic neonatal diabetes, KCNJ11 diabetes, ABCC8 diabetes

Drugs used for this condition

AGlibenclamideFirst line · Oral hypoglycemic agents. SulfonylureasIn patients with neonatal diabetes due to activating mutations in KCNJ11 or ABCC8, glibenclamide replaces insulin and restores physiologic glycemic regulation. The mechanism: sulfonylurea closes the mutant ATP-sensitive potassium channel of the β-cell and restores glucose-responsive insulin secretion. Switching from insulin to glibenclamide in these patients is supported by international expert groups (ISPAD 2022, Diabetologia 2006 Pearson) at doses higher than standard adult dosing, up to 1–2 mg/kg daily.

How to read tiers A–F

Evidence tiers belong to the drug-indication pair, not to the drug as a whole: the same substance can be A for one condition and F for another.

AStrong evidence – first or second line in international guidelines
BRecommended in selected cases
CStudy data contradict each other
DBenefit not proven, no documented harm
FDocumented harm, or risk without benefit