Патофизиологические и морфологические аспекты врожденного гиперинсулинизма (обзор литературы)

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.

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