problendoПроблемы ЭндокринологииProblems of Endocrinology0375-96602308-1430Endocrinology Research Centre10.14341/probl12572problendo-12572Research ArticleДетская эндокринологияPediatric EndocrinologyТранзиторные варианты врожденного гиперинсулинизма у новорожденных детейTransient hyperinsulinism in neonateshttps://orcid.org/0000-0003-3127-5974БолмасоваАнна ВикторовнаBolmasovaAnna V.

к.м.н.; ORCID: 0000-0003-3127-5974, eLibrary SPIN: 7843-1604

117997, Москва, ул. Академика Опарина, д. 4

MD, PhD; ORCID: orcid.org/0000-0003-3127-5974, eLibrary SPIN: 7843-1604

4, Oparin street, Moscow

anna_bolmasova@mail.ruhttps://orcid.org/0000-0002-1491-2460МеликянМария АрменаковнаMelikyanMaria A.

к.м.н.; ORCID: 0000-0002-1491-2460; eLibrary SPIN: 4184-4383

Москва

MD, PhD; ORCID: 0000-0002-1491-2460; eLibrary SPIN: 4184-4383

Moscow

melikian.maria@gmail.comhttps://orcid.org/0000-0002-3788-0887КрыловаНаталья АнатольевнаKrylovaNatalya A.

детский эндокринолог; ORCID: 0000-0002-3788-0887; eLibrary SPIN: 6289-2376

Москва

MD; ORCID: 0000-0002-3788-0887; eLibrary SPIN: 6289-2376

Moscow

nataliya_2007_78@mail.ruИоновОлег ВадимовичIonovOleg V.

к.м.н.; ORCID: 0000-0002-4153-133X; eLibrary SPIN: 3492-6281

Москва

MD, PhD; ORCID: 0000-0002-4153-133X; eLibrary SPIN: 3492-6281

Moscow

o_ionov@oparina4.ruРюминаИрина ИвановнаRyuminaIrina I.

д.м.н.; ORCID: 0000-0003-1831-887X; eLibrary SPIN: 1054-8996

Москва

MD, PhD prof; ORCID: 0000-0003-1831-887X; eLibrary SPIN: 1054-8996

Moscow

i_ryumina@oparina4.ruБокерияЕкатерина ЛеонидовнаBockeriaEkaterina L.

д.м.н.; ORCID: 0000-0002-8898-961; eLibrary SPIN: 7367-6290

Москва

MD, PhD prof; ORCID: 0000-0002-8898-961; eLibrary SPIN: 7367-6290

Moscow

e-bockeria@mail.ruПекареваНаталья АркадьевнаPekarevaNatalya A.

д.м.н.; ORCID: 0000-0002-6931-9672, eLibrary SPIN: 5304-2544

Москва

MD, PhD; ORCID: 0000-0002-6931-9672, eLibrary SPIN: 5304-2544

Moscow

n_pekareva@oparina4.ruhttps://orcid.org/0000-0003-0822-751XДегтяреваАнна ВладимировнаDegtyarevaAnna V.

д.м.н., профессор; ORCID: 0000-0003-0822-751X, eLibrary SPIN: 2361-9978

Москва

MD, PhD prof; ORCID: 0000-0003-0822-751X, eLibrary SPIN: 2361-9978

Moscow

annadim@yahoo.comНациональный медицинский исследовательский центр акушерства, гинекологии и перинатологии им. акад. В.И. Кулакова; Национальный медицинский исследовательский центр эндокринологииРоссияKulakov Federal Rеseаrch Center for Obstetrics, Gynecology and Perinatology; Endocrinology research CentreRussian FederationНациональный медицинский исследовательский центр эндокринологииРоссияKulakov Federal Rеseаrch Center for Obstetrics, Gynecology and PerinatologyRussian FederationНациональный медицинский исследовательский центр акушерства, гинекологии и перинатологии им. акад. В.И. КулаковаРоссияKulakov Federal Rеseаrch Center for Obstetrics, Gynecology and PerinatologyRussian FederationНациональный медицинский исследовательский центр акушерства, гинекологии и перинатологии им. акад. В.И. Кулакова; Первый Московский государственный медицинский университет имени И.М. Сеченова Министерства здравоохранения Российской Федерации (Сеченовский Университет)РоссияKulakov Federal Rеseаrch Center for Obstetrics, Gynecology and PerinatologyRussian Federation2020260920206646167Copyright © Болмасова А.В., Меликян М.А., Крылова Н.А., Ионов О.В., Рюмина И.И., Бокерия Е.Л., Пекарева Н.А., Дегтярева А.В., 20202020Болмасова А.В., Меликян М.А., Крылова Н.А., Ионов О.В., Рюмина И.И., Бокерия Е.Л., Пекарева Н.А., Дегтярева А.В.Bolmasova A.V., Melikyan M.A., Krylova N.A., Ionov O.V., Ryumina I.I., Bockeria E.L., Pekareva N.A., Degtyareva A.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.probl-endojournals.ru/jour/article/view/12572

Врожденный гиперинсулинизм — редкое заболевание, характеризующееся неадекватно высокой секрецией инсулина бета-клетками поджелудочной железы, приводящее к развитию гипогликемии. Выделяют персистирующие и транзиторные формы гиперинсулинизма.

Транзиторные варианты врожденного гиперинсулинизма являются наиболее частой причиной развития тяжелых гипогликемий в раннем неонатальном периоде. Этиология данного заболевания до конца не изучена, при этом выделяют факторы риска развития транзиторного гиперинсулинизма: асфиксия при рождении, недоношенность, сахарный диабет у матери, низкий или крупный вес к сроку гестации. Учитывая тяжелое течение гиперинсулинических гипогликемий, необходимы ранняя диагностика и адекватная терапия, направленные на поддержание стойкой эугликемии особенно в период новорожденности.

В статье описаны 3 клинических случая развития транзиторного гиперинсулинизма у детей разного гестационного возраста с сопутствующей патологией. Всем детям проводилась инсулиностатическая терапия диазоксидом с положительным эффектом в виде развития эугликемии и отмены дополнительной дотации глюкозы. У всех детей инсулиностатическая терапия была впоследствии отменена. На момент публикации статьи физическое и психомоторное развитие детей соответствует возрасту.

Congenital hyperinsulinusm is rare disease characterized high secretion of insulin by pancreatic beta cells leading to the development of hypoglycemia. Persistent and transient forms of hyperinsulinism are distinguished. Transient hyperinsulinism are the most common cause of severe hypoglycemia in newborns. The etiology of this disease is not known. There are risk factors for the development of transient hyperinsulinism: asphyxia at birth, prematurity, maternal diabetes, low or large weight by gestation. Hypoglycemia with hyperinsulinism is severe. Therefore, early diagnosis and therapy especially during the neonatal period, are necessary.

The article describes 3 clinical cases of transient hyperinsulinism in children with different gestational age and concomitant pathology. All children recevied insulinostatic therapy with diazoxide with a positive effect: euglycemia without glucose requirement . In all children, therapy was completed subsequently. At the time of publication of the article, the physical and psychomotor development of children is normal.

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