Congenital hyperinsulinism: localization of a focal lesion with 18F-FDOPA positron emission tomography
| Autor: | Diva D. De León, Susan A. Becker, Lisa J. States |
|---|---|
| Rok vydání: | 2021 |
| Předmět: |
medicine.medical_specialty
medicine.diagnostic_test business.industry medicine.medical_treatment Brain damage Hypoglycemia medicine.disease medicine.disease_cause Positron emission tomography Pediatrics Perinatology and Child Health Pancreatectomy Congenital hyperinsulinism medicine Radiology Nuclear Medicine and imaging Radiology medicine.symptom Hyperinsulinemic hypoglycemia business Hyperinsulinism Neuroradiology |
| Zdroj: | Pediatric Radiology. 52:693-701 |
| ISSN: | 1432-1998 0301-0449 |
| DOI: | 10.1007/s00247-021-05206-5 |
| Popis: | Hyperinsulinemic hypoglycemia of infancy, also known as congenital hyperinsulinism, is a group of disorders characterized by dysregulated insulin release. Neonates with severe, persistent hyperinsulinemic hypoglycemia who are unresponsive to medical therapy require pancreatectomy to prevent brain damage from hypoglycemia. To date, multiple genetic mutations and syndromes and several unique histopathological entities have been identified in children with hyperinsulinism. Histopathology is characterized as diffuse, focal or atypical. Surgical resection of a focal lesion results in a cure in up to 97% of these children. Imaging with 6-fluoro-(18F)-L-3,4-dihydroxyphenylalanine (18F-FDOPA) positron emission tomography (PET) is the test of choice for identifying and localizing a focal lesion and has proved to be an invaluable guide for surgical resection. Genetic evaluation is essential for determining who will benefit from PET imaging. This article provides an approach to determine who should be imaged, how to set up a protocol and how to interpret the imaging findings. The diagnosis and management of this disorder require a multidisciplinary approach to prevent brain damage from hypoglycemia. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|
Full text from SpringerLink