Clinical Research Directory

Inclusion Criteria: * Any age, but primarily infants 0-6 months given typical age of initial presentation. * Children with diagnosis of FoHI or DiHI based on clinical criteria (fasting hypoglycemia accompanied by inadequate suppression of plasma insulin, inappropriately low plasma free fatty acid and plasma-hydroxybutyrate concentrations, and an inappropriate glycemic response to glucagon injection) o confirmed by genetic testing for mutations in ABCC8 and KCNJ1 was1. * Hypoglycemia uncontrolled with medical management (diazoxide, octreotide). * Able to withdraw medications in time to wash out prior to the scheduled PET scan. * Patients fulfilling criteria above but with uncontrolled hypoglycemia after initial surgical management (partial or near-total pancreatectomy) * Normal hepatic and renal function. Exclusion Criteria: * Treatment with other, third-line, medications for hyperinsulinism (nifedipine, glucagon). * Patients with hepatic or renal insufficiency.