Medications for the treatment of congenital hyperinsulinism

Name	Dose	Route	Side effects
Diazoxide	5 to 15 mg/kg/day divided twice daily (up to maximum adult dose, 500 mg twice daily)	Oral	Fluid retention, pulmonary hypertension, hypertrichosis, neutropenia, thrombocytopenia, decreased appetite, hyperuricemia
Chlorothiazide^*	10 to 30 mg/kg/day divided twice daily	Oral	Electrolyte abnormalities
Octreotide	2 to 20 mcg/kg/day divided every 6 to 8 hours (up to maximum adult dose 500 mcg/day)	Subcutaneous	Gallstones, aminotransferase elevations, malabsorption, hypothyroidism, growth suppression, thrombosis, necrotizing enterocolitis
Lanreotide	60 to 120 mg every 28 days	Deep subcutaneous	Gallstones, aminotransferase elevations, malabsorption, growth suppression, hypothyroidism
Glucagon	2.5 to 20 mcg/kg/hour continuously	Intravenous	Nausea, vomiting, necrolytic migratory erythema

* Chlorothiazide is given as adjunctive therapy with diazoxide to prevent fluid overload.

Adapted from: Lord K, De Leon DD. Hyperinsulinism in the Neonate. Clin Perinatol 2018; 45:61.

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