transmedТрансляционная медицинаTranslational Medicine2311-44952410-5155Almazov National Medical Research Centre, Saint Petersburg, Russia10.18705/2311-4495-2020-7-2-12-20transmed-529Research ArticleЭНДОКРИНОЛОГИЧЕСКИЕ ЗАБОЛЕВАНИЯMETABOLIC DISEASESПатофизиологические и морфологические аспекты врожденного гиперинсулинизма (обзор литературы)Pathophysiological and morphological aspects of congenital hyperinsulinism. Reviewhttps://orcid.org/0000-0002-1946-0029ПерминоваА. А.PerminovaA. A.

Перминова Анастасия Аркадьевна, аспирант

ул. Аккуратова, д. 2, Санкт-Петербург, 197341

Perminova Anastasiia A., PhD Student

Akkuratova str. 2, Saint Petersburg, 197341

perminova_aa@almazovcentre.ruФедеральное государственное бюджетное учреждение «Национальный медицинский исследовательский центр имени В. А. Алмазова» Министерства здравоохранения Российской ФедерацииРоссияAlmazov National Medical Research CentreRussian Federation202019052020721220Copyright © Перминова А.А., 20202020Перминова А.А.Perminova A.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://transmed.almazovcentre.ru/jour/article/view/529

Врожденный гиперинсулинизм является одной из наиболее частых причин гипогликемии у детей в возрасте до 1 года. При отсутствии адекватной фармакологической коррекции гипогликемических приступов возможно развитие необратимых изменений в центральной нервной системе с последующей инвалидизацией таких больных. Однако из-за неэффективности консервативной терапии во многих случаях требуется хирургическое вмешательство, объем которого зависит от формы заболевания. Но вследствие несовершенства методов предоперационной диагностики, дифференциальная диагностика между очаговой и диффузной формами врожденного гиперинсулинизма производится интраоперационно патологоанатомами. В связи с тем, что патоморфология поджелудочной железы при врожденном гиперинсулинизме остается малоизученной, гистологическая и дальнейшая иммуногистохимическая диагностика представляет значительные трудности.В данном обзоре представлены имеющиеся в литературе данные о гистологической, гистохимической и иммуногистохимической характеристике эндокринной части поджелудочной железы, которые могут оказаться полезными в ходе дальнейшего изучения врожденного гиперинсулинизма. В частности, подробно изложены существующие на сегодняшний день подходы к морфологической классификации и интраоперационной гистологической диагностике различных форм врожденного гиперинсулинизма. Кроме того, дано подробное описание экспрессии факторов транскрипции NeuroD1, Nkx2.2 и Isl1 в ткани поджелудочной железы при иммуногистохимическом исследовании. Описан профиль иммуногистохимического окрашивания в поджелудочной железе дофаминовых и соматостатиновых рецепторов, а также соматостатина и хромогранина А.Объединенные в этом обзоре данные многочисленных исследований способны помочь в дальнейшем поиске решений диагностических и терапевтических проблем, связанных с врожденным гиперинсулинизмом.

Congenital hyperinsulinism is one of the most common causes of hypoglycemia in infants. In the absence of adequate pharmacological correction of hypoglycemic attacks, the development of irreversible changes in the central nervous system with the subsequent disability of such patients is possible. Due to the ineffectiveness of conservative therapy, in many cases surgical intervention is required. However, due to the imperfection of preoperative diagnosis, pathologists intraoperatively perform the differentiation between focal and diffuse forms of congenital hyperinsulinism. But the pancreatic pathology with congenital hyperinsulinism remains poorly understood, therefore histological and further immunohistochemical diagnosis presents significant difficulties.This review presents data on the histological, histochemical, and immunohistochemical characteristics of the endocrine pancreas, which may be useful in the further studies of congenital hyperinsulinism. In particular, we describe in detail the current approaches to morphological classification and intraoperative histological diagnosis of various forms of congenital hyperinsulinism. In addition, we give a detailed description of the expression of transcription factors NeuroD1, Nkx2.2 and Isl1 in pancreatic tissue during immunohistochemical study. Also, we describe the profile of immunohistochemical staining of dopamine and somatostatin receptors, as well as somatostatin and chromogranin A.The data of numerous studies combined in this review can help researchers in the further search for solutions to the diagnostic and therapeutic problems associated with congenital hyperinsulinism.

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