Medications for the treatment of congenital hyperinsulinism

Name	Dose	Route	Side effects
Diazoxide	5 to 15 mg/kg/day, 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, twice daily	Oral	Electrolyte abnormalities
Octreotide	2 to 20 mcg/kg/day, 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	1 mg/day, 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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