Accuracy of [18F]Fluorodopa Positron Emission Tomography for Diagnosing and Localizing Focal Congenital Hyperinsulinism

Hardy, Olga T.; Hernandez-Pampaloni, Miguel; Saffer, Janet; Scheuermann, Joshua; Ernst, Linda M.; Freifelder, R.; Zhuang, Hongming; MacMullen, Courtney; Becker, Susan; Adzick, N. Scott; Divgi, Chaitanya; Alavi, Abass; Stanley, Charles A.

doi:10.1210/jc.2007-1637

Cited by 131 publications

(91 citation statements)

References 26 publications

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“…Fluorine-18 L-3, 4-dihydroxyphenylalanine positron emission tomography computerised tomography ( 18 F DOPA-PET CT) scan can help to differentiate the focal from diffuse forms of CHI and aids in the clinical management of these patients (24). Before the advent of 18 F DOPA-PET CT, pancreatic venous sampling (PVS) and selective pancreatic arterial calcium stimulation with hepatic venous sampling (ASVS) were used to differentiate between the two subtypes (25,26).…”

Section: Introductionmentioning

confidence: 99%

Clinical and histological heterogeneity of congenital hyperinsulinism due to paternally inherited heterozygous ABCC8/KCNJ11 mutations

Arya

Güemes

Nessa

et al. 2014

European Journal of Endocrinology

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Context: Congenital hyperinsulinism (CHI) has two main histological types: diffuse and focal. Heterozygous paternally inherited ABCC8/KCNJ11 mutations (depending upon whether recessive or dominant acting and occurrence of somatic maternal allele loss) can give rise to either phenotype. However, the relative proportion of these two phenotypes in a large cohort of CHI patients due to paternally inherited heterozygous ABCC8/KCNJ11 mutations has not been reported. Objective: The purpose of this study is to highlight the variable clinical phenotype and to characterise the distribution of diffuse and focal disease in a large cohort of CHI patients due to paternally inherited heterozygous ABCC8/KCNJ11 mutations. Design: A retrospective chart review of the CHI patients due to heterozygous paternally inherited ABCC8/KCNJ11 mutations from 2000 to 2013 was conducted. Results: Paternally inherited heterozygous ABCC8/KCNJ11 mutations were identified in 53 CHI patients. Of these, 18 (34%) either responded to diazoxide or resolved spontaneously. Fluorine-18 L-3, 4-dihydroxyphenylalanine positron emission tomography computerised tomography ( 18 F DOPA-PET CT) scanning in 3/18 children showed diffuse disease. The remaining 35 (66%) diazoxide-unresponsive children either had pancreatic venous sampling (nZ8) or 18

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Section: Introductionmentioning

confidence: 99%

Clinical and histological heterogeneity of congenital hyperinsulinism due to paternally inherited heterozygous ABCC8/KCNJ11 mutations

Arya

Güemes

Nessa

et al. 2014

European Journal of Endocrinology

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“…Премедикация препаратом «Кар-бидопа» -ингибитором периферической L-аминокислотной декарбоксилазы, не требуется [31]. Однако некоторые авторы утверждают, что медикаментозную терапию перед процеду-рой отменять необязательно [19,32].…”

Section: методика проведения пэт/кт с 18 F-дофаunclassified

“…За 24 часа до про-ведения исследования рекомендуется устано-вить назогастральный зонд без металлического наконечника, с помощью которого до проведе-ния КТ пациенту в желудок вводится 20 мл во-ды с целью негативного контрастирования две-надцатиперстной кишки [29]. Инъекцию 18 F-ДОФА осуществляют внутривенно из расчета 4 МБк на 1 кг массы пациента [29,32,33]. Деся-тиминутные ПЭТ сканы выполняют через 10, 20, 30, 40, и 50 минут после инъекции РФП [29,32].…”

Section: методика проведения пэт/кт с 18 F-дофаunclassified

“…Инъекцию 18 F-ДОФА осуществляют внутривенно из расчета 4 МБк на 1 кг массы пациента [29,32,33]. Деся-тиминутные ПЭТ сканы выполняют через 10, 20, 30, 40, и 50 минут после инъекции РФП [29,32].…”

Section: методика проведения пэт/кт с 18 F-дофаunclassified

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The Use of 18f-Dopa Pet/Ct Imaging in Congenital Hyperinsulinism

Gubaeva¹,

Melikyan²,

Ryzhkova³

et al. 2017

Rejr

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рожденный гиперинсулинизм (ВГИ) является частой причиной гипогликемии у детей. По данным гистологического исследования, заболевание подразделяют на диффузную, фокальную и атипичную формы. Хирургическая тактика ле-чения зависит от формы ВГИ: при фокальной форме выполняют резекцию патологической области поджелудочной железы с последующим полным выздоровлени-ем пациента, при диффузной форме с фармакорезистентным течением проводят ре-зекцию 95-98% поджелудочной железы, что приводит к развитию сахарного диабета и экзокринной панкреатической недостаточности. Дифференциальная диагностика фо-кальной и диффузной форм по клиническим и рентгенологическим признакам невоз-можна. ПЭТ/КТ с 18 F-ДОФА является «золотым стандартом» дифференциальной диагно-стики диффузной и фокальной форм заболевания. Настоящая статья является обзором литературных данных за последние 10 лет и посвящена методологическим основам и оценке информативности ПЭТ/КТ с 18 F-ДОФА у детей с ВГИ. ongenital hyperinsulinism is the most common cause of hypoglycemia in children. Congenital hyperinsulinism is divided into diffuse, focal and atypical forms. The surgical management for each form of congenital hyperinsulinism is crucially different. Focal form of congenital hyperinsulinism is cured by selective resection of the pathologic area leading to patient`s recovery. Medically unresponsive diffuse form of congenital hyperinsulinism requires the removal of 95-98% of pancreatic tissue, which may result in diabetes mellitus and exocrine pancreas insufficiency. Differential diagnosis of focal and diffuse forms according to clinical and radiological signs is impossible. Conducting with PET/CT with 18 F-DOPA is currently the "gold standard" of differential diagnosis between diffuse and focal forms of the disease. This article is a review of scientific papers for last 10 years and dedicated of methodology and diagnostic accuracy of PET/CT with 18 F-DOPA in children with congenital hyperinsulinism.

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“…For this purpose, it appears possible that technologies such as MRI and PET could be applied in clinical settings in the near future. Of note, PET facilitates the pre-surgical discrimination of focal vs diffuse lesions in children with congenital hyperinsulinism and therefore represents an important clinical instrument in this setting [10]. One of the main challenges for MRI and PET in terms of the non-invasive determination of beta cell mass is to identify tissue-/cell-specific tracers.…”

Section: Towards Non-invasive Monitoring Of the Pancreas And Its Disementioning

confidence: 99%

Imaging the pancreas: from ex vivo to non-invasive technology

Holmberg

Ahlgren

2008

Diabetologia

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While many recently published reviews have covered non-invasive nuclear imaging techniques, the aim of this review is to focus on current developments in optical imaging technologies for investigating the pancreas. Several of these modalities are being developed into non-invasive, real-time monitoring routines for pancreatic diseases. However, they also provide pre-clinical ex vivo and/or intravital tools for three-dimensional quantitative assessments of cellular and molecular events, with levels of specificity and resolution difficult to achieve with other currently available modalities.

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Accuracy of [18F]Fluorodopa Positron Emission Tomography for Diagnosing and Localizing Focal Congenital Hyperinsulinism

Cited by 131 publications

References 26 publications

Clinical and histological heterogeneity of congenital hyperinsulinism due to paternally inherited heterozygous ABCC8/KCNJ11 mutations

Clinical and histological heterogeneity of congenital hyperinsulinism due to paternally inherited heterozygous ABCC8/KCNJ11 mutations

The Use of 18f-Dopa Pet/Ct Imaging in Congenital Hyperinsulinism

Imaging the pancreas: from ex vivo to non-invasive technology

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