problendoПроблемы ЭндокринологииProblems of Endocrinology0375-96602308-1430Endocrinology Research Centre10.14341/probl9726problendo-9726Research ArticleКлинические случаиCase ReportsДифференциальная диагностика морфологических форм врожденного гиперинсулинизма методом ПЭТ/КТ с [18F]-фторДОФАDifferential diagnosis of morphological forms of congenital hyperinsulinism using [18F]-DOPA PET/CThttps://orcid.org/0000-0003-3922-2869ГубаеваДиляра НаилевнаGubaevaDiliara N.

аспирант

MD

gubaevadn@gmail.comhttps://orcid.org/0000-0002-1491-2460МеликянМария АрменаковнаMelikyanMaria A.

к.м.н.

MD, PhD, leading research associate

melikian.maria@gmail.comhttps://orcid.org/0000-0002-7086-9153РыжковаДарья ВикторовнаRyzhkovaDaria V.

д.м.н., профессор

MD, PhD, Professor

d_ryjkova@mail.ruhttps://orcid.org/0000-0003-0735-7822МитрофановаЛюбовь БорисовнаMitrofanovaLubov B.

д.м.н.

MD, PhD

lubamitr@yandex.ruhttps://orcid.org/0000-0003-4013-0785НикитинаИрина ЛеоровнаNikitinaIrina L.

д.м.н., профессор

MD, PhD, Professor

nikitina0901@gmail.com<p>ФГБУ &laquo;Национальный медицинский исследовательский центр эндокринологии&raquo; Минздрава России</p>Россия<p>Endocrinology Research Certre</p>Russian Federation<p>ФГБУ &laquo;Национальный медицинский исследовательский центр им. В.А. Алмазова&raquo; Минздрава России</p>Россия<p>Almazov National Medical Research Center</p>Russian Federation201829122018645306311Copyright &#x00A9; Губаева Д.Н., Меликян М.А., Рыжкова Д.В., Митрофанова Л.Б., Никитина И.Л., 20182018Губаева Д.Н., Меликян М.А., Рыжкова Д.В., Митрофанова Л.Б., Никитина И.Л.Gubaeva D.N., Melikyan M.A., Ryzhkova D.V., Mitrofanova L.B., Nikitina I.L.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.probl-endojournals.ru/jour/article/view/9726

В основе врожденного гиперинсулинизма (ВГИ) лежит гиперпродукция инсулина β-клетками поджелудочной железы. Из-за высокого риска осложнений хронической гипогликемии у пациентов с ВГИ приоритетной задачей является своевременная диагностика заболевания и незамедлительное начало терапии. Выбор тактики лечения во многом зависит от морфологической формы заболевания. Клинико-лабораторная картина заболевания, данные УЗИ, МРТ, компьютерной и позитронной-эмиссионной томографии (ПЭТ) с [18F]-фтордезоксиглюкозой не позволяют установить морфологическую форму заболевания. Ранее для дифференциальной диагностики применялись стимуляционный тест с кальцием и чрескожно-чреспеченочный забор крови из ветвей воротной вены, однако эти инвазивные исследования дают недостаточно точные результаты. В настоящий момент дифференциальная диагностика диффузной и фокальной форм ВГИ на предоперационном этапе базируется на данных молекулярно-генетической и радионуклидной диагностики (ПЭТ с [18F]-фторДОФА). В статье представлены первые результаты применения ПЭТ с [18F]-фторДОФА у пациентов с ВГИ в России. Радионуклидное исследование было выполнено 17 пациентам с фармакорезистентным течением ВГИ. В дальнейшем результаты ПЭТ/КТ с [18F]-фторДОФА сопоставлялись с гистологической картиной препаратов поджелудочной железы, полученных интраоперационно («золотой стандарт» дифференциальной диагностики морфологических форм ВГИ).

Congenital hyperinsulinism (CHI) is caused by insulin hyperproduction by β-pancreatic cells. CHI is associated with high risk of complications of chronic hypoglycemia, and therefore timely diagnosis of the disease and immediate initiation of therapy is a top-priority task. The choice of treatment tactics largely depends on the morphological form of the disease. Morphological form cannot be established based on clinical and laboratory presentation of the disease, ultrasound, MRI, computed and positron emission tomography (PET) with [18F]-fluorodeoxyglucose. Calcium stimulation test and percutaneous transhepatic blood sampling from the portal vein were previously used for differential diagnosis, but the results provided by these invasive studies are imprecise. At present, preoperative differential diagnosis of diffuse and focal forms of CHI is based on the data of genetic testing and radionuclide diagnosis ([18F]-DOPA PET). The article presents the first results of the use of [18F]-DOPA PET in CHI patients in the Russian Federation. Radionuclide study was performed in 17 patients with pharmacoresistant CHI followed by comparative analysis of the results of 18F-FDG PET/CT and histological picture of intraoperative pancreatic tissue samples, which is known as the gold standard for the differential diagnosis of HI histological forms.

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The authors declare that there are no conflicts of interest present.