nidНефрология и диализNephrology and Dialysis1680-44222618-9801Российское диализное обществоnid-551Research ArticleОБЗОРЫ И ЛЕКЦИИREVIEWS AND LECTURESПоражение ЦНС у больных с Шига-токсин ассоциированным гемолитико-уремическим синдромом (STEC-ГУС): современные аспекты патогенеза, клиники и стратегии лечения (Обзор литературы)Neurological involvement in patients with STEC-Associated Hemolytic Uremic Syndrome (STEC-HUS): modern aspects of pathogenesis, clinical features and treatment modalitiesШпикаловаИ. Ю.ShpikalovaI. Yu.ikucherova@yandex.ruПанкратенкоТ. Е.PankratenkoT. E.noemail@neicon.ruЭмироваХ. М.EmirovaKh. M.noemail@neicon.ruЗверевД. В.ZverevD. V.noemail@neicon.ruТолстоваЕ. М.TolstovaE. M.noemail@neicon.ruДетская городская клиническая больница святого ВладимираРоссияSt. Vladimir’s clinical children hospitalRussian FederationГБУЗ МО «Московский областной научно-исследовательский клинический институт имени М.Ф.Владимирского»РоссияM.F.Vladimirskii Moscow’s regional scientific-research clinical instituteRussian FederationГОУ ВПО «Московский государственный медико-стоматологический университет имени А.И. Евдокимова» Минздравсоцразвития РоссииРоссияA.I.Evdokimov Moscow State University of Medicine and DentistryRussian Federation201413092024163328338Copyright © Шпикалова И.Ю., Панкратенко Т.Е., Эмирова Х.М., Зверев Д.В., Толстова Е.М., 20242024Шпикалова И.Ю., Панкратенко Т.Е., Эмирова Х.М., Зверев Д.В., Толстова Е.М.Shpikalova I.Y., Pankratenko T.E., Emirova K.M., Zverev D.V., Tolstova E.M.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nephro.ru/jour/article/view/551

Поражение центральной нервной системы (ЦНС), возникающее при STEC-ГУС у детей, является нередким (>20%) жизнеугрожающим осложнением и во многом определяет прогноз и исход заболевания. Самые частые неврологические симптомы - судороги (>70%), нарушения сознания (70-80%), кома (≈20%), парезы, глазодвигательные расстройства. Лечение и выхаживание таких пациентов и в настоящее время остается актуальной и очень сложной проблемой. В последние годы, благодаря улучшению качества медицинской помощи, улучшился прогноз заболевания. Более чем у 50% больных с поражением ЦНС наблюдается полное неврологическое восстановление. В обзоре представлены современные данные о патогенезе, клинической картине и методах лечения (плазмаферез, экулизумаб и IgG-иммуноадсорбция) типичного ГУС, осложненного тяжелой неврологической симптоматикой. Представленные данные во многом касаются последней эпидемии STEC-ГУС O104:Н4 2011 года в Европе, но клиническая картина и исход заболевания у детей сопоставимы с предыдущими cведениями о развитии ГУС, вызванными иными штаммами E.coli. Методы лечения детей в целом были менее агрессивными, чем у взрослых. Поддерживающая и заместительная почечная терапия оказалась эффективной у 74% детей больных STEC-ГУС, включая детей с поражением ЦНС. Плазмаферез был применен у 19% детей, в основном с неврологическими проявлениями. Ретроспективный анализ стратегий лечения показал отсутствие быстрого положительного эффекта от применения экулизумаба в сравнении с лечением плазмаферезом у пациентов с сопоставимой тяжестью ГУС. IgG-иммуноадсорбция оказалась эффективной в лечении тяжелых неврологических осложнений ГУС, хотя теория IgG-опосредованного повреждения ЦНС еще не доказана. Современные методы терапии, несомненно, позволят лечить детей, больных осложненными формами типичного ГУС, более успешно.

Acute neurologic involvement is a life threatening and not rare (>20%) complication of the STEC-HUS in children and often determines prognosis and outcome of the disease. The most frequent neurologic signs are seizures (>70%), impaired consciousness (70-80%), coma (≈20%), paresis, oculomotor disturbances. The prognosis of typical HUS has improved over the last decades mainly due to the better general medical care resulting in lower mortality rate in pediatric STEC-HUS. More than 50% of children completely recovered from their neurological complication. This review reports current state of pathogenesis, clinical features and treatment approaches for typical STEC-HUS with neurological complications. A lot of data are concerned of the recent outbreak of HUS caused by shiga toxin producing E.coli O104:Н4, but clinical feature and short-term outcome in pediatric patients were similar to previous outbreaks of STEC-HUS. The treatment modalities in children were generally less aggressive than those in adult patients. Most of children with O104:H4 HUS (74%) recovered with supportive treatment alone, including those with neurological complications. Plasmapheresis was performed in 19% pediatric patients, mainly with neurological complications (n=16). Retrospective analysis of treatment strategies showed no significant short term benefit of eculizumab compared with patients with the same severity of HUS treated with plasmapheresis. IgG-immunoadsorption turned out to be unexpectedly effective in treating the severe neurological complications of HUS, but antibody-mediated theory of CNS injury in HUS is not yet proved. Current treatment approaches will undoubtedly allow one to treat typical HUS with severe complications in children more successfully.

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