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МЕДЛАЙН.РУ
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Фундаментальные исследования • Патологическая анатомия
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. (статья в формате PDF. Для просмотра необходим Adobe Acrobat Reader) открыть статью в новом окне Список литературы 1. De León D.D., Stanley C.A. Mechanisms of disease: advances in diagnosis and treatment of hyperinsulinism in neonates. Nat Clin Pract Endocrinol Metab. 2007; 3: 57-68. 2. Laidlaw G.F. Neisidioblastoma, the islet tumour of pancreas. Am J Pathol. 1938; 14:125-139. 3. Stanley C.A. Advances in diagnosis and treatment of hyperinsulinism in infants and children. J Clin Endocrinol Metab. 2002; 87: 4857-9. 4. James C, Kapoor RR, Ismail D, et al. The genetic basis of congenital hyperinsulinism. J Med Genet 2009;46(5):289-99. 5. 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