Proposal for a Standardized Protocol for &lt;sup&gt;18&lt;/sup&gt;F-DOPA-PET (PET/CT) in Congenital Hyperinsulinism

Mohnike, Klaus; Blankenstein, Oliver; Christesen, Henrik Thybo; Lonlay, J De; Hussain, K; Koopmans, Klaas Pieter; Minn, Heikki; Mohnike, Wolfgang; Mutair, Angham N. Al; Otonkoski, Timo; Rahier, Jacques; Ribeiro, Maria-João; Schoenle, Eugen J.; Fekete, C

doi:10.1159/000093471

Cited by 59 publications

(59 citation statements)

References 42 publications

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“…In diazoxide-unresponsive patients, it is essential to have rapid genetic analysis for mutations in ABCC8/KCNJ11 and/or 18F-DOPA-PET/CT scan to plan subsequent treatment (91). Patients with genetically confirmed diffuse disease do not require further imaging studies.…”

Section: Introductionmentioning

confidence: 99%

Hyperinsulinaemic hypoglycaemia: genetic mechanisms, diagnosis and management

Senniappan

Balasubramaniam

James

et al. 2012

J of Inher Metab Disea

122

121

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Hyperinsulinaemic hypoglycaemia (HH) is due to the unregulated secretion of insulin from pancreatic β‐cells. A rapid diagnosis and appropriate management of these patients is essential to prevent the potentially associated complications like epilepsy, cerebral palsy and neurological impairment. The molecular basis of HH involves defects in key genes (ABCC8, KCNJ11, GLUD1, GCK, HADH, SLC16A1, HNF4A and UCP2) which regulate insulin secretion. The most severe forms of HH are due to loss of function mutations in ABCC8/KCNJ11 which encode the SUR1 and KIR6.2 components respectively of the pancreatic β‐cell KATP channel. At a histological level there are two major forms (diffuse and focal) each with a different genetic aetiology. The diffuse form is inherited in an autosomal recessive (or dominant) manner whereas the focal form is sporadic in inheritance and is localised to a small region of the pancreas. The focal form can now be accurately localised pre‐operatively using a specialised positron emission tomography scan with the isotope Fluroine‐18L‐3, 4‐dihydroxyphenyalanine (18F‐DOPA‐PET). Focal lesionectomy can provide cure from the hypoglycaemia. However the diffuse form is managed medically or by near total pancreatectomy (with high risk of diabetes mellitus). Recent advances in molecular genetics, imaging with 18F‐DOPA‐PET/CT and novel surgical techniques have changed the clinical approach to patients with HH.

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Section: Introductionmentioning

confidence: 99%

Hyperinsulinaemic hypoglycaemia: genetic mechanisms, diagnosis and management

Senniappan

Balasubramaniam

James

et al. 2012

J of Inher Metab Disea

122

121

View full text Add to dashboard Cite

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“…По различным данным, для получения наиболее достоверных результатов в качестве подготовки к процедуре пациентам рекомендуется отмена терапии ок-треотидом и глюкагоном за 48 часов до иссле-дования [31]. 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

“…Для топической диагностики очага пора-жения производится автоматическое совмеще-ние трехмерных изображений ПЭТ и КТ, вы-полненной с контрастным усилением [31].…”

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

“…Совмещенная ПЭТ/КТ c 18 F-ДОФА должна выполняться всем пациентам с персистирую-щим гиперинсулинизмом [31], которым плани-руется хирургическое лечение [16]. В данную Таблица №1.…”

Section: показания к проведению пэт/кт C 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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“…Ribeiro et al (10)(11)(12) were the first to report on the use of 18 F-DOPA PET for differentiating between focal and diffuse hyperinsulinemia of infancy, and their findings were subsequently verified by other investigators (13)(14)(15)(16)(17)(18). When uptake of 18 F-DOPA was focal, immunocytochemical analysis of surgical specimens confirmed the diagnosis of focal disease.…”

mentioning

confidence: 92%