vspВопросы современной педиатрииCurrent Pediatrics1682-55271682-5535Издательство «ПедиатрЪ»10.15690/vsp.v25i1.3003vsp-3926Research ArticleОБЗОР ЛИТЕРАТУРЫREVIEWГипокалиемический периодический паралич: обзор литературыHypokalemic Periodic Paralysis: Narrative Reviewhttps://orcid.org/0000-0002-6137-6138ИвановА. А.IvanovArtem A.

Иванов Артем Александрович - врач-педиатр, эксперт-аналитик управления по реализации функций Национального медицинского исследовательского центра по педиатрии РНИМУ им. Н.И. Пирогова (Пироговский Университет), врач-педиатр педиатрического диагностического отделения Российской детской клинической больницы.

117513, Москва, ул. Островитянова, д. 1

Moscow

trt.iy@yandex.ruhttps://orcid.org/0009-0001-1613-4498ЕршоваН. С.ErshovaNatalya S.

Москва

Moscow

https://orcid.org/0000-0002-7447-0625КуличенкоТ. В.KulichenkoTatiana V.

Москва

Moscow

Российский национальный исследовательский медицинский университет им. Н.И. Пирогова (Пироговский Университет)РоссияPirogov Russian National Research Medical UniversityRussian Federation2026050220262511116Copyright © Иванов А.А., Ершова Н.С., Куличенко Т.В., 20262026Иванов А.А., Ершова Н.С., Куличенко Т.В.Ivanov A.A., Ershova N.S., Kulichenko T.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://vsp.spr-journal.ru/jour/article/view/3926

Гипокалиемический периодический паралич — редкая генетическая патология, характеризующаяся спонтанной мышечной слабостью вплоть до развития параличей, которые сопровождаются гипокалиемией. Развитие заболевания связано с изменениями генов CACNA1S, SCN4A, KCNJ2 и KCNJ18, регулирующих функционирование ионных каналов в клетках скелетной мускулатуры. В статье рассмотрены особенности этиологии, клинической картины и методов лечения гипокалиемического периодического паралича, а также обсуждаются вопросы его дифференциальной диагностики с другими видами периодических параличей. Освещаются способы профилактики эпизодов мышечной слабости в целях повышения качества жизни пациентов.

Hypokalemic periodic paralysis is a rare genetic pathology characterized by spontaneous muscle weakness up to paralysis along with hypokalemia. The disease development is associated with changes in the CACNA1S, SCN4A, KCNJ2, and KCNJ18 genes regulating ion channels functioning in skeletal muscle cells. This article discusses features of etiology, clinical signs, and treatment methods for hypokalemic periodic paralysis, as well as issues of differential diagnosis with other types of periodic paralysis. Variants of prevention of muscle weakness episodes to improve patients’ quality of life are also covered.

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