epilepsiaEpilepsy and paroxysmal conditionsЭпилепсия и пароксизмальные состояния2077-83332311-4088IRBIS LLCepilepsia-158Research ArticleCLINICAL CASESКЛИНИЧЕСКИЕ СЛУЧАИNEUROLOGICAL PAROXYSMAL DISORDERS IN CHILDREN WITH HYPOGLYCEMIA IN CONGENITAL HYPERINSULINISM: POLYMORPHISM OF CLINICAL IMPLICATIONSНЕВРОЛОГИЧЕСКИЕ ПАРОКСИЗМАЛЬНЫЕ НАРУШЕНИЯ У ДЕТЕЙ С ГИПОГЛИКЕМИЕЙ НА ФОНЕ ВРОЖДЕННОГО ГИПЕРИНСУЛИНИЗМА: ПОЛИМОРФИЗМ КЛИНИЧЕСКИХ ПРОЯВЛЕНИЙЩедеркинаИ. О.ShchederkinaI. O.

Щедеркина Инна Олеговна – кандидат медицинских наук, невролог, заведующая неврологическим кабинетом для детей с органическим поражением ЦНС и нарушением психики консультативного центра Морозовской детской городской клинической больницы, доцент кафедры неврологии, нейрохирургии и медицинской генетики педиатрического факультета Российского Научного исследовательского Университета им. Пирогова.

Адрес: 4-й Добрынинский пер., 1/9, Москва, Россия, 119037.

Shсhederkina Inna Olegovna – PhD, neurologist, head of neurological Cabinet for children with organic CNS and Mental Disorders Advisory Center Morozov Children’s Clinical Hospital, docent, Neurology, Neurosurgery and Medical Genetics Depertment, Pirogov Russian National Research Medical University.

Address: 4 Dobrininsky per., 1|9, Moscow, Russia, 119037.

schederkina@mail.ruМеликянМ. А.MelikyanM. A.

Меликян Мария Арменаковна – кандидат медицинских наук, эндокринолог, старший научный сотрудник. 

Адрес: ул. Дмитрия Ульянова, д. 11, Москва, Россия, 117036.

Melikyan Maria Armenakovna – PhD, endocrinologist, senior researcher. 

Address: Dmitry Ulyanov, 11, Moscow, Russia, 117036.

melikian.maria@gmail.comЗаваденкоА. Н.ZavadenkoA. N.

Заваденко Александра Николаевна – кандидат медицинских наук, невролог, ассистент кафедры неонатологии. 

Адрес: ул. Островитянова, д. 1, Москва, Россия, 117997. 

Zavadenko Aleksandra Nikolaevna – PhD, neurologist, assistant of the Neonatology Department. 

Address: ul. Ostrovityanova, 1, Moscow, Russia, 117997.

aleks.zavadenko@gmail.comКозловаЕ. В.KozlovaE. V.

Козлова Елизавета Викторовна – кандидат медицинских наук, невролог. 

Адрес: ул. С. Машела, д. 1, ГСП-7, Москва, Россия, 117997.

Kozlova Elizaveta Viktorovna – PhD, neurologist.

Address: ul. S. Machel, 1, GSP-7, Moscow, Russia, 117997. 

neurologev@rambler.ruЗаваденкоН. Н.ZavadenkoN. N.

Заваденко Николай Николаевич – доктор медицинских наук, невролог, профессор, заведующий кафедрой неврологии, нейрохирургии и медицинской генетики педиатрического факультета РНИМУ им. Пирогова.

Адрес: ул. Островитянова, д. 1, Москва, Россия, 117997.

Zavadenko Nikolaj Nikolaevich – MD, neurologist, Professor, Head of Department of Neurology, Neurosurgery and Medical Genetics, Pirogov Russian National Research Medical University.

Address: ul. Ostrovityanova, 1, Moscow, Russia, 117997. 

zavadenko@mail.ruМорозовская детская городская клиническая больница, Москва; Российский национальный исследовательский медицинский университет имени Н.И. Пирогова, МоскваРоссияMorozov Children’s Clinical Hospital, Moscow; Pirogov Russian National Research Medical University, MoscowRussian FederationРоссийский эндокринологический научный центр, МоскваРоссияRussian Endocrinology Reaseach Center, MoscowRussian FederationРоссийский национальный исследовательский медицинский университет имени Н.И. Пирогова, МоскваРоссияMorozov Children’s Clinical Hospital, Moscow; Pirogov Russian National Research Medical University, MoscowRussian FederationФедеральный научно-клинический центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачёва; Морозовская детская городская клиническая больница, МоскваРоссияFederal scientific clinical center of children hematology, oncology and immunology of D. Rogachov, Moscow ; Morozov Children’s Clinical Hospital, MoscowRussian Federation201511062016724958Copyright © Shchederkina I.O., Melikyan M.A., Zavadenko A.N., Kozlova E.V., Zavadenko N.N., 20162016Щедеркина И.О., Меликян М.А., Заваденко А.Н., Козлова Е.В., Заваденко Н.Н.Shchederkina I.O., Melikyan M.A., Zavadenko A.N., Kozlova E.V., Zavadenko N.N.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.epilepsia.su/jour/article/view/158

Hypoglycemia in congenital hyperinsulinism (WGI) in children increases the risk of brain damage, seizures and development delay. The aim of our study was to evaluate the clinical manifestations in children with CHI. Hypoglycemia is often manifests with seizures, which are sometimes in the future may lead to the development of epilepsy. Seizures in CHI do not always require a diagnosis of epilepsy and prescription of anticonvulsants. EEG changes in these children may be observed in the absence of epileptic seizures. Developmental delay was more common in children with neurological abnormalities and MRI changes. Children with ABCC8 gene mutations have more complicated for CHI and more prominent developmental delay. 

Гипогликемия при врожденном гиперинсулинизме (ВГИ) у детей увеличивает риск повреждения мозга, развития судорог и появления задержки психоречевого развития. Целью нашего исследования была оценка клинических проявлений у детей с ВГИ. Гипогликемия часто манифестирует с судорог, которые иногда в будущем могут приводить к развитию эпилепсии. Судорожные приступы при ВГИ не всегда требуют постановки диагноза эпилепсии и назначения антиконвульсантов. Изменения на ЭЭГ у этих детей могут наблюдаться и при отсутствии эпилептических приступов. Задержка развития чаще отмечалась у детей с отклонениями в неврологическом статусе и МРТ-изменениями. Дети с мутациями в гене ABCC8 имели более тяжелое течение ВГИ и более выраженную задержку развития. 

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