18F-DOPA positron emission tomography/computed tomography application in congenital hyperinsulinism

Yang, Jigang; Yuan, Leilei; Meeks, Jacqui K.; Zhang, Nan; Li, Chunlin; Hao, Ruirui

doi:10.1515/jpem-2012-0114

Cited by 7 publications

(6 citation statements)

References 28 publications

(46 reference statements)

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Таким образом, вызывает сомнения необходимость проведения сканирования через 10 мин после исследования. Затруднения в применении ПИ могут возникнуть за счет физиологического повышенного захвата 18 F-ДОФА в области головки поджелудочной железы, при оценке гигантских фокусов или мультифокусов [21,22].…”

Section: методы регистрации исходовunclassified

“…Данное наблюдение не удивительно, учитывая эмбриогенетическую связь поджелудочной железы с двенадцатиперстной кишкой. В целом мультифокальное и эктопическое расположение фокусов у пациентов с ВГИ встречается достаточно редко [21,22].…”

Section: методы регистрации исходовunclassified

See 1 more Smart Citation

Clinical, genetic, and radionuclide characteristics of the focal form of congenital hyperinsulinism

Gubaeva¹,

Melikyan²,

Ryzhkova³

et al. 2019

Probl Endokrinol (Mosk)

View full text Add to dashboard Cite

BACKGROUND: Congenital hyperinsulinism (CHI) is a severe disease with a high risk of complications including neurological deficit. Persistent hypoglycemia in patients with focal form of CHI can not be managed with medical treatment in 96.4% of cases, what subsequently leads to surgical treatment. Currently, there is a lack of information regarding patients with focal form of CHI. This study is aimed at finding better approaches for diagnosis and treatment of patients with focal form of CHI. AIMS: To study clinical, genetic and PET/CT findings of the focal form of CHI in Russian group of patients. MATERIALS AND METHODS: The observational research included all patients with a histologically confirmed focal form of CHI, who were admitted to Endocrinology Research Centre during the period from January 2008 to January 2019. A statistical analysis of clinical data, genotype, and positron emission tomography (PET) with 18F-dihydroxyphenylalanine (18F-DOPA) was performed. The median follow-up was 18 months. RESULTS: The study included 31 patients with focal CHI (14 boys, 45.2%). All patients had a neonatal presentation of the disease and demanded high levels of continuous glucose infusion to maintain euglycemia. The difference between the age of hypoglycemia presentation and the age of diagnosis ranged from 1 day to 3.9 months. In all cases, diazoxide was found to be ineffective. However, in 9 patients, it was possible to withdraw continuous glucose infusion and maintain euglycemia using octreotide in the preoperative period. A molecular genetic study allowed us to detect diverse pathogenic variants in ABCC8 and KCNJ11 genes in 30 patients. According to PET data with 18F-DOPA, the pancreatic index (PI) varied widely from 1.16 to 3.59. After partial resection of the pancreatic region with insulin hypersecretion, all patients showed complete recovery. CONCLUSIONS: The focal form of CHI is a severe condition with high prevalence of neurological complications. For preoperative diagnosis of the morphological form of the disease, it is necessary to conduct genetic analysis and radionuclide studies. Solely evaluation of mathematical parameters in 18F-DOPA PET without taking into account the visual data and the results of genetic analysis does not allow establishing the robust diagnosis. Timely diagnosis, identification of risk factors, and prevention of complications of persistent hypoglycemia are important tasks for clinicians.

show abstract

Section: методы регистрации исходовunclassified

Clinical, genetic, and radionuclide characteristics of the focal form of congenital hyperinsulinism

Gubaeva¹,

Melikyan²,

Ryzhkova³

et al. 2019

Probl Endokrinol (Mosk)

View full text Add to dashboard Cite

show abstract

“…Ряд обстоятельств, которые могут привести к ложноотрицательным результатам: малые размеры очага гиперинсулинемии (менее 1 см) или мультифокальность, особенности формы аденоматоза (плоский очаг), суперпози-ция левой почки, желчного пузыря, двенадца-типерстной кишки на патологический фокус [36]. По данным литературы, наименьший раз-мер фокуса, который был идентифицирован-ный при ПЭТ, составил 5х4 мм [33].…”

Section: диагностическая точность методаunclassified

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

Gubaeva¹,

Melikyan²,

Ryzhkova³

et al. 2017

Rejr

View full text Add to dashboard Cite

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.

show abstract

“…Fluorine-18 labeled Ldihydroxyphenylalanine ( 18 F-DOPA) is used [2,7,26,33,34]. The uptake of 18 F-DOPA is increased in b-cells which produce higher rates of insulin [1,7,35]. Thus, PET scan imaging can detect primary or metastatic pancreatic tumors, hyperfunctional pancreatic islets and allows differentiate between diffuse and focal forms of CH and localize the lesion in focal forms [1,2,7,26,29,33,35].…”

Section: Diagnosis and Treatmentmentioning

confidence: 99%

“…The uptake of 18 F-DOPA is increased in b-cells which produce higher rates of insulin [1,7,35]. Thus, PET scan imaging can detect primary or metastatic pancreatic tumors, hyperfunctional pancreatic islets and allows differentiate between diffuse and focal forms of CH and localize the lesion in focal forms [1,2,7,26,29,33,35]. This is very important for surgery planning, as enucleation of local lesions may result in complete cure [2,7].…”

Section: Diagnosis and Treatmentmentioning

confidence: 99%

Congenital hyperinsulinism

et al. 2014

View full text Add to dashboard Cite

Hyperinsulinism is the most common cause of hypoglycemia in infants. In many cases conservative treatment is not effective and surgical intervention is required. Differentiation between diffuse and focal forms and localization of focal lesions are the most important issues in preoperative management. We present a case of persistent infancy hyperinsulinism. Clinical presentation, conservative treatment modalities, diagnostic possibilities of focal and diffuse forms, and surgical treatment, which led to total recovery, are discussed.

show abstract

18F-DOPA positron emission tomography/computed tomography application in congenital hyperinsulinism

Cited by 7 publications

References 28 publications

Clinical, genetic, and radionuclide characteristics of the focal form of congenital hyperinsulinism

Clinical, genetic, and radionuclide characteristics of the focal form of congenital hyperinsulinism

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

Congenital hyperinsulinism

Contact Info

Product

Resources

About