gnckКолопроктологияKoloproktologia2073-75562686-7303Russian Association of Coloproctology10.33878/2073-7556-2019-18-2-82-89gnck-1496Research ArticleКЛИНИЧЕСКИЕ НАБЛЮДЕНИЯCASE REPORTИШЕМИЧЕСКИЙ КОЛИТ НА ФОНЕ АТИПИЧНОГО ГЕМОЛИТИКОУРЕМИЧЕСКОГО СИНДРОМА (клиническое наблюдение)ISCHEMIC COLITIS IN AN ADULT PATIENT WITH ATYPICAL HEMOLYTIC UREMIC SYNDROME (case report)ОзероваО. С.OzerovaO. S.

ул. Саляма Адиля, д. 2, Москва, 123423 

info@gnck.ruПолторыхинаЕ. А.PoltorykhinaE. A.ВарданянА. В.VardanyanA. V.МайновскаяО. А.MaynovskayaO. A.ВеселовВ. В.VeselovV. V.ЧернышовС. В.ChernyshovS. V.ФГБУ «ГНЦК им. А.Н. Рыжих» Минздрава РоссииРоссияState Scientific Centre of ColoproctologyRussian Federation201919062019182(68)8289Copyright © Озерова О.С., Полторыхина Е.А., Варданян А.В., Майновская О.А., Веселов В.В., Чернышов С.В., 20192019Озерова О.С., Полторыхина Е.А., Варданян А.В., Майновская О.А., Веселов В.В., Чернышов С.В.Ozerova O.S., Poltorykhina E.A., Vardanyan A.V., Maynovskaya O.A., Veselov V.V., Chernyshov S.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.ruproctology.com/jour/article/view/1496

В литературе описано две разновидности гемолитико-уремического синдрома – типичный (постдиарейный), ассоциированный с E.Coli (штамм 0157:Н7), а также Shigella dysenteriae I типа и т.д., а также атипичный гемолитико-уремический синдром (аГУС), обусловленный гиперактивацией альтернативного пути системы комплемента, относящийся к наследственным орфанным заболеваниям. аГУС характеризуется тромбоцитопенией, микроангиопатической гемолитической анемией, поражением почек, головного мозга, желудочно-кишечного тракта, легких, сердца. В литературе обнаружено описание лишь одной пациентки с поражением желудочно-кишечного тракта в форме ишемического колита, в связи с чем представляет интерес еще одно клиническое наблюдение.

Atypical hemolytic uremic syndrome (aHUS) is a rare life-threatening condition caused by uncontrolled complement activation due to mutations in the alternative pathway of complement components. aHUS is characterized by microangiopathic hemolytic anemia, thrombocytopenia, acute renal failure and affecting multiple organ systems. Extra-renal manifestations of aHUS take place in 20% of patients including involvement of the central nervous system, cardiovascular system, lungs, skin and gastrointestinal tract. This case report describes a severe course of atypical hemolytic uremic syndrome in a 21-year-old female, developed ischemic colitis.

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