18F-Fluoro-L- DOPA PET Imaging for the Detection and Localization of Focal Congenital Hyperinsulinism
The goal of this project is to determine the role of FDOPA/PET as a pre-operative diagnostic imaging procedure for differentiating focal and diffuse forms of congenital hyperinsulinism and locating focal lesions in the pancreas to guide surgical resection.
• 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.