title: Congenital hyperinsulinism: Role of fluorine-18L-3, 4 hydroxyphenylalanine positron emission tomography scanning.
abstract: Congenital hyperinsulinism (CHI) is a rare but complex heterogeneous disorder caused by unregulated secretion of insulin from the β-cells of the pancreas leading to severe hypoglycaemia and neuroglycopaenia. Swift diagnosis and institution of appropriate management is crucial to prevent or minimise adverse neurodevelopmental outcome in children with CHI. Histologically there are two major subtypes of CHI, diffuse and focal disease and the management approach will significantly differ depending on the type of the lesion. Patients with medically unresponsive diffuse disease require a near total pancreatectomy, which then leads on to the development of iatrogenic diabetes mellitus and pancreatic exocrine insufficiency. However patients with focal disease only require a limited pancreatectomy to remove only the focal lesion thus providing complete cure to the patient. Hence the preoperative differentiation of the histological subtypes of CHI becomes paramount in the management of CHI. Fluorine-18L-3, 4-hydroxyphenylalanine positron emission tomography ((18)F-DOPA-PET) is now the gold standard for pre-operative differentiation of focal from diffuse disease and localisation of the focal lesion. The aim of this review article is to give a clinical overview of CHI, then review the role of dopamine in β-cell physiology and finally discuss the role of (18)F-DOPA-PET imaging in the management of CHI.
authors: Jaya Sujatha Gopal-Kothandapani, Department of Paediatric Endocrinology, Royal Manchester Children's Hospital, Manchester M13 9WL, United Kingdom.
authors: Jaya Sujatha
authors: JS
authors: Gopal-Kothandapani
authors: Jaya Sujatha Gopal-Kothandapani, Department of Paediatric Endocrinology, Royal Manchester Children's Hospital, Manchester M13 9WL, United Kingdom.
authors: Khalid
authors: K
authors: Hussain
keywords: Congenital hyperinsulinism; Diffuse congenital hyperinsulinism; Ectopic congenital hyperinsulinism; Fluorine-18L-3, 4-hydroxyphenylalanine positron emission tomography; Focal congenital hyperinsulinism; Standardized uptake value