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Congenital hyperinsulinism:Role of fluorine-18l-3, 4 hydroxyphenylalanine positron emission tomography scanning

World Journal of Radiology 2014年第6期6卷 252-260页

作者： Jaya Sujatha Gopal-Kothapani Khalid Hussain Department of Paediatric Endocrinology Royal Manchester Children’s Hospital Manches-ter M13 9WL United Kingdom Department of Paediatric Endocrinology Great Ormond Street Hospital for Children NHS Trust and the Institute of Child Health University College London London WC1N 1EH United Kingdom Developmental Endocrinology Research Group Molecular Genetics Unit Institute of Child Health University College London London WC1N 1EH United Kingdom

Congenital hyperinsulinism(CHI) is a rare but complex heterogeneous disorder caused by unregulated secre-tion 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 neurodevel-opmental 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 de-velopment of iatrogenic diabetes mellitus and pancre-atic exocrine insufficiency. However patients with focaldisease only require a limited pancreatectomy to re-move 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-hydroxy-phenylalanine positron emission tomography(18F-DOPA-PET) is now the gold standard for pre-operative differentiation of focal from diffuse disease and locali-sation 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 18F-DOPA-PET imaging in the management of CHI.

关键词： Congenital hyperinsulinism fluorine-18l-3,4-hydroxyphenylalanine positron emission tomography Focal congenital hyperinsulinism Diffuse congenital hyperinsulinism Ectopic congenital hyperinsulinism Standardized uptake value

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