БИОМЕДИЦИНСКИЙ ЖУРНАЛ МЕДЛАЙН.РУ
Содержание журнала

Архив

Редакция
Учредители

Федеральное государственное бюджетное учреждение «Научно-клинический центр токсикологии имени академика С.Н. Голикова Федерального медико-биологического агентства»


Федеральное государственное бюджетное учреждение науки
Институт теоретической и экспериментальной биофизики
Российской академии наук


ООО "ИЦ КОМКОН"

Адрес редакции и реквизиты

199406, Санкт-Петербург, ул.Гаванская, д. 49, корп.2

ISSN 1999-6314


Фундаментальные исследования • Патологическая физиология

Том: 20
Статья: « 32 »
Страницы:. 385-403
Опубликована в журнале: 7 декабря 2019 г.

English version

Врожденный гиперинсулинизм: этиопатогенез и подходы к лечению (обзор литературы)

Перминова А.А.

ФГБУ НМИЦ им. В.А. Алмазова МЗ РФ


Резюме
Врожденный гиперинсулинизм - одна 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. Обзор составлен в целях объединения имеющихся в англоязычной литературе данных о врожденном гиперинсулинизме, которые несмотря на свою немногочисленность отличаются крайней разрозненностью. В данном обзоре представлены данные об этиологии, патогенезе и методах диагностики врожденного гиперинсулинизма, а также о возможностях его медикаментозной коррекции. В частности, описаны различные мутации и патогенетические механизмы, возможности и трудности предоперационной и интраоперационной диагностики, тактика и возможности лекарственной терапии врожденного гиперинсулинизма.


Ключевые слова
врожденный гиперинсулинизм, гиперинсулинизм, незидиобластоз, гиперинсулинемическая гипогликемия, островки Лангерганса.



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