68ga-dotanoc and Congenital-Hyperinsulinism

Focal congenital hyperinsulinism (CHI) is curable by surgery, which is why identification of the focal lesion is crucial. We aimed to determine the use of 18F-fluoro-dihydroxyphenylalanine (18F-DOPA) PET/CT vs. 68Ga-1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic-acid-1-Nal3-octreotide (68Ga-DOTANOC) PET/CT as diagnostic tools in focal CHI.. PET/CT scans of children with CHI admitted to Odense University Hospital between August 2005 and June 2016 were retrospectively evaluated visually and by their maximal standardized uptake values (SUV. 18F-DOPA PET/CT was excellent in predicting focal CHI and superior compared to 68Ga-DOTANOC PET/CT. Further use of 68GA-DOTANOC PET/CT in predicting focal CHI is discouraged.

Topics: Child; Child, Preschool; Congenital Hyperinsulinism; Dihydroxyphenylalanine; Female; Humans; Infant; Male; Organometallic Compounds; Positron Emission Tomography Computed Tomography; Retrospective Studies

Congenital hyperinsulinism (CHI) is the most frequent cause of severe, persistent hypoglycemia in infancy and childhood. We report a case of CHI with diffuse pancreatic abnormality diagnosed preoperatively using the 68Ga octreotide (DOTA NOC) positron emission tomography scan. Genetic analysis revealed homozygous ABCC8 splicing mutation. Subtotal (95%) pancreatectomy was done, and the infant remained euglycemic and was discharged on breast feeds. The patient is continuously followed up and is asymptomatic until 9 months.

Topics: Alternative Splicing; ATP-Binding Cassette Transporters; Congenital Hyperinsulinism; Female; Humans; Infant; Infant, Newborn; Organometallic Compounds; Positron-Emission Tomography; Potassium Channels, Inwardly Rectifying; Receptors, Drug; Sulfonylurea Receptors