hrp0094p2-235 | Fetal, neonatal endocrinology and metabolism (to include hypoglycaemia) | ESPE2021
Juliana Christine
, Chai Jinghua
, Arroyo Pablo
, Rico-Bautista Elizabeth
, Betz Stephen
, De Leon Diva
,
Inactivating mutations of ß-cell KATP channels cause the most common and severe form of congenital hyperinsulinism (HI), a ß-cell disorder that results in dysregulated insulin secretion and persistent hypoglycemia. Children with KATPHI are typically unresponsive to diazoxide, the only FDA-approved drug for HI. Octreotide, an SST2-selective agonist peptide that inhibits insulin secretion, is used as second line therapy, but poor efficacy and SST2...