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Патофизиологические и морфологические аспекты врожденного гиперинсулинизма (обзор литературы)

https://doi.org/10.18705/2311-4495-2020-7-2-12-20

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Аннотация

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.

Об авторе

А. А. Перминова
Федеральное государственное бюджетное учреждение «Национальный медицинский исследовательский центр имени В. А. Алмазова» Министерства здравоохранения Российской Федерации
Россия

Перминова Анастасия Аркадьевна, аспирант

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



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Для цитирования:


Перминова А.А. Патофизиологические и морфологические аспекты врожденного гиперинсулинизма (обзор литературы). Трансляционная медицина. 2020;7(2):12-20. https://doi.org/10.18705/2311-4495-2020-7-2-12-20

For citation:


Perminova A.A. Pathophysiological and morphological aspects of congenital hyperinsulinism. Review. Translational Medicine. 2020;7(2):12-20. (In Russ.) https://doi.org/10.18705/2311-4495-2020-7-2-12-20

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ISSN 2311-4495 (Print)
ISSN 2410-5155 (Online)