rospedРоссийский педиатрический журнал имени М.Я. СтуденикинаM.Ya. Studenikin Russian Pediatric JournalФГАУ «НМИЦ здоровья детей» Минздрава России10.46563/1560-9561-2021-24-6-395-404lbzxjvrosped-394Research ArticleОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯORIGINAL INVESTIGATIONSПервичный склерозирующий холангит у детей с воспалительными заболеваниями кишечникаPrimary sclerosing cholangitis in children with inflammatory bowel diseasehttps://orcid.org/0000-0003-0661-6976УсольцеваОльга ВладимировнаUsoltsevaOlga V.

Аспирант, врач-педиатр гастроэнтерологического отделения с гепатологической группой ФГАУ «НМИЦ здоровья детей» Минздрава России

e-mail: usoltseva.olga93@gmail.com

Postgraduate student, paediatrician of the gastroenterological department with the hepatology group of the National Medical Research Center for Children’s Health, Moscow, 119991, Russian Federation

e-mail: usoltseva.olga93@gmail.com

usoltseva.olga93@gmail.comhttps://orcid.org/0000-0002-3697-4283СурковАндрей НиколаевичSurkovAndrey N.

Доктор мед. наук, зав. гастроэнтерологическим отделением с гепатологической группой, гл. науч. сотр. лаб. научных основ детской гастроэнтерологии и гепатологии, проф. каф. педиатрии и общественного здоровья Института подготовки медицинских кадров ФГАУ «НМИЦ здоровья детей» Минздрава России

noemail@neicon.ruhttps://orcid.org/0000-0003-2881-4703МовсисянГоар БорисовнаMovsisyanGoar B.

Канд. мед. наук, врач-педиатр гастроэнтерологического отделения с гепатологической группой ФГАУ «НМИЦ здоровья детей» Минздрава России

e-mail: gongurik@mail.ru

gongurik@mail.ruhttps://orcid.org/0000-0001-9798-9617КулебинаЕлена АнатольевнаKulebinaElena A.

Канд. мед. наук, врач-гастроэнтеролог гастроэнтерологического отделения с гепатологической группой, мл. науч. сотр. лаб. научных основ детской гастроэнтерологии и гепатологии ФГАУ «НМИЦ здоровья детей» Минздрава России

e-mail: e.kulebina@gmail.com

e.kulebina@gmail.comhttps://orcid.org/0000-0003-0292-1412СимерзинаСветлана АлександровнаSimersinaSvetlana A.

Врач-рентгенолог отд-ния магнитно-резонансной томографии и денситометрии ФГАУ «НМИЦ здоровья детей» Минздрава России

e-mail: simerzinasvetlana@yandex.ru

simerzinasvetlana@yandex.ruhttps://orcid.org/0000-0003-4905-2373ПотаповАлександр СергеевичPotapovAlexander S.

Доктор мед. наук, проф., гл. науч. сотр. лаб. новых технологий диагностики и лечения болезней детского возраста ФГАУ «НМИЦ здоровья детей» Минздрава России

e-mail: apotap@mail.ru

apotap@mail.ruhttps://orcid.org/0000-0002-8305-7592ЛохматовМаксим МихайловичLokhmatovMaksim M.

Доктор мед. наук, зав. отделением эндоскопических исследований, гл. науч. сотр. лаб. эндоскопических, морфологических и патологоанатомических исследований, ФГАУ «НМИЦ здоровья детей» Минздрава России, проф. каф. детской хирургии и урологии-андрологии им. проф. Л.П. Александрова ФГАОУ ВО «Первый МГМУ им. И.М. Сеченова» Минздрава России (Сеченовский Университет)

e-mail: lokhmatov@nczd.ru

lokhmatov@nczd.ruФГАУ «Национальный медицинский исследовательский центр здоровья детей» Минздрава РоссииNational Medical Research Center for Children’s HealthФГАУ «Национальный медицинский исследовательский центр здоровья детей» Минздрава России; ФГАОУ ВО «Первый Московский государственный медицинский университет имени И.М. Сеченова» Минздрава России (Сеченовский Университет)National Medical Research Center for Children’s Health; I.M. Sechenov First Moscow State Medical University202120022024246395404Copyright © Усольцева О.В., Сурков А.Н., Мовсисян Г.Б., Кулебина Е.А., Симерзина С.А., Потапов А.С., Лохматов М.М., 20242024Усольцева О.В., Сурков А.Н., Мовсисян Г.Б., Кулебина Е.А., Симерзина С.А., Потапов А.С., Лохматов М.М.Usoltseva O.V., Surkov A.N., Movsisyan G.B., Kulebina E.A., Simersina S.A., Potapov A.S., Lokhmatov M.M.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.rosped.ru/jour/article/view/394Актуальность

Актуальность. Особенности дебюта первичного склерозирующего холангита (ПСХ) при воспалительных заболеваниях кишечника (ВЗК) у детей, частота выявления маркеров цитолиза и холестаза, характер поражений гепатобилиарной системы и изменений эндоскопической картины изучены недостаточно.

Цель работы — определить особенности течения ПСХ при ВЗК у детей.

Материалы и методы

Материалы и методы. Комплексно обследованы 54 ребёнка с ПСХ+ВЗК (основная группа) и 79 больных с изолированными формами ВЗК: язвенным колитом (ЯК) или болезнью Крона (БК) (группа сравнения).

Результаты

Результаты. Cочетание ПСХ+ЯК было выявлено у 48 больных с преобладанием мальчиков. Самыми частыми проявлениями ПСХ в дебюте заболевания у детей с сочетанием ПСХ+ВЗК были диарея, абдоминальный болевой синдром, синдромы цитолиза и холестаза, гемоколит. У детей с ПСХ+ВЗК по сравнению с больными ВЗК отмечалось значимое увеличение сывороточных концентраций аланин- и аспартатаминотрансферазы, общего белка, свидетельствовавшее о нарастании активности гепатита; уровень γ-глутамилтрансферазы, щелочной фосфатазы и прямого билирубина указывал на нарастание выраженности синдрома холестаза; IgG — на выраженность мезенхимально-воспалительного синдрома при поражениях печени. При эндоскопии у этих больных был выявлен панколит с выраженным вовлечением правых отделов толстой кишки или изолированный проктит, а у пациентов с ПСХ+БК чаще отмечался илеоколит. Индексы PUCAI (р < 0,001) и PCDAI (р = 0,027) были существенно уменьшены у больных с ПСХ+ВЗК, чем при изолированном течении ВЗК. При МРТ органов брюшной полости и МР-холангиопанкреатографии у больных с сочетанием ПСХ+ВЗК характерными проявлениями были неоднородность паренхимы печени, неровность внутри- и внепеченочных желчных протоков, наличие участков расширения и сужения их диаметра.

Выводы

Выводы. Дебют ПСХ у больных с фенотипом ПСХ+ВЗК у детей проявляется преимущественно кишечной симптоматикой, чаще представлен ЯК с вовлечением правых отделов кишечника преимущественно у мальчиков и меньшим индексом активности по сравнению с изолированным течением ВЗК в сочетании с лабораторными синдромами и холестазом.

Участие авторов

Участие авторов:Усольцева О.В., Сурков А.Н., Мовсисян Г.Б. — концепция и дизайн исследования, написание текста;Усольцева О.В., Мовсисян Г.Б., Кулебина Е.А., Симерзина С.А. — сбор и обработка материала;Усольцева О.В., Мовсисян Г.Б. — статистическая обработка;Сурков А.Н., Мовсисян Г.Б., Потапов А.С., Лохматов М.М. — редактирование.Усольцева О.В., Сурков А.Н., Мовсисян Г.Б. — утверждение окончательного варианта статьи, ответственность за целостность всех частей статьи.

Информированное согласие

Информированное согласие. От родителей пациентов получено информированное согласие на участие в исследовании.

Финансирование

Финансирование. Исследование не имело финансовой поддержки.

Конфликт интересов

Конфликт интересов. Авторы заявляют об отсутствии конфликта интересов.

Поступила 29

Поступила 29.11.2021 Принята к печати 17.12.2021Опубликована 29.12.2021

 

 

Introduction

Introduction. Currently, in pediatric practice, there are no clear data on the features of the onset of primary sclerosing cholangitis (PSC) in inflammatory bowel diseases (IBD), the frequency of detection of markers of cytolysis and cholestasis, the nature of the lesion of the hepatobiliary system and changes in the endoscopic picture.

Purpose

Purpose: assessment of clinical, laboratory, and instrumental features of PSC course in IBD children (PSC+IBD).

Materials and methods

Materials and methods. For the period 2015–2021, a comprehensive examination of 54 children with PSC+IBD (main group) and 79 patients with isolated IBD (comparison group): ulcerative colitis (UC) or Crohn’s disease (CD) was carried out.

Results

Results. In 54 children of the main group, the combination of PSC+UC was detected in 48 (88.9%) patients with a predominance of boys. At the onset of the disease, the most frequent manifestations in children with PSC+IBD were diarrhea, abdominal pain syndrome, cytolysis and cholestasis syndromes, and hemocolitis. In children with PSC+IBD, compared with patients with IBD, there was a statistically significant increase in serum concentrations of ALT, AST and total protein, indicating an increase in hepatitis activity; GGT, alkaline phosphatase and direct bilirubin — about an increase in the severity of cholestasis syndrome; IgG — as a marker of the severity of mesenchymal-inflammatory syndrome in the framework of liver damage. According to the endoscopic data, with PSC+UC, pancolitis with more pronounced involvement of the right colon or isolated proctitis prevails, and with PSC+CD, ileocolitis is more common. Indices PUCAI (p < 0.001) and PCDAI (p = 0.027) were statistically significantly lower in the PSC+IBD group than in the isolated IBD. According to the data of transient liver elastometry, no statistically significant difference was obtained between the medians of the parameters of the biochemical blood test at different stages of fibrosis. According to the results of magnetic resonance imaging of the abdominal organs and MR-cholangiopancreatography, the most characteristic changes in PSC+IBD are heterogeneity of the liver parenchyma, unevenness of the intra- and extrahepatic bile ducts, the presence of areas of expansion and narrowing of their diameter.

Conclusions

Conclusions. The phenotype of PSC+IBD in children is manifested mainly by intestinal symptoms, more often UC with involvement of the right parts of the intestine in boys and a lower activity index than the isolated course of IBD, in combination with laboratory syndromes and cholestasis.

Contribution

Contribution:Usoltseva O.V., Surkov A.N., Movsisyan G.B. — research concept and design, text writing;Usoltseva O.V., Movsisyan G.B., Kulebina E.A., Simerzina S.A. — collection and processing of material;Usoltseva O.V., Movsisyan G.B. — statistical processing;Surkov A.N., Movsisyan G.B., Potapov A.S., Lokhmatov M.M. — editing.Usoltseva O.V., Surkov A.N., Movsisyan G.B. — approval of the final version of Informed consent.

Informed consent was received from the patients’ parents for the participation of a study.

Acknowledgement

Acknowledgement. The study had no sponsorship.

Conflict of interest

Conflict of interest. The authors declare no conflict of interest.

Received

Received: November 29, 2021Accepted: December 17, 2021Published: December 29, 2021

 

 

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