Congenital hyperinsulinism in infancy and childhood: challenges, unmet needs and the perspective of patients and families

Table 1 Treatment options for CHI [15, 17, 18]

Type	Drug name	Mode of action
Diazoxide		Activates K_ATP channels of pancreatic β cells and maintains them in an open state, inhibiting insulin secretion
Somatostatin analogue	Octreotide LAR	Decreases secretion of insulin through hyperpolarization of β cells and inhibition of calcium channels
Somatostatin analogue	Lanreotide
Sirolimus (formerly rapamycin)		Inhibits the mTOR signaling pathway, potentially limiting the production of insulin from β cells
Glucagon		Promotes hepatic glucose production and increases blood glucose levels

CHI, congenital hyperinsulinism; K_ATP, adenosine triphosphate-sensitive potassium; LAR, long-acting release; mTOR, mammalian target of rapamycin

ISSN: 1750-1172