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Congenital hyperinsulinism: localization of a focal lesion with 18F-FDOPA positron emission tomography.
States, Lisa J; Becker, Susan A; De León, Diva D.
Afiliação
  • States LJ; Department of Radiology, Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, 3401 Civic Center Blvd., Philadelphia, PA, 19104, USA. states@chop.edu.
  • Becker SA; Research Institute, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
  • De León DD; Department of Pediatrics, Division of Endocrinology and Diabetes, Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
Pediatr Radiol ; 52(4): 693-701, 2022 Apr.
Article em En | MEDLINE | ID: mdl-34668049
ABSTRACT
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.
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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Hiperinsulinismo Congênito Tipo de estudo: Guideline / Prognostic_studies Limite: Child / Humans / Infant / Newborn Idioma: En Ano de publicação: 2022 Tipo de documento: Article

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Hiperinsulinismo Congênito Tipo de estudo: Guideline / Prognostic_studies Limite: Child / Humans / Infant / Newborn Idioma: En Ano de publicação: 2022 Tipo de documento: Article