vtioВестник трансплантологии и искусственных органовRussian Journal of Transplantology and Artificial Organs1995-1191Academician V.I.Shumakov National Medical Research Center of Transplantology and Artificial Organs", Ministry of Health of the Russian Federation10.15825/1995-1191-2013-4-47-54vtio-80Research ArticleКлиническая трансплантологияClinical TransplantologyПАТОМОРФОЛОГИЯ БИЛИАРНОЙ АТРЕЗИИ У ДЕТЕЙ – РЕЦИПИЕНТОВ ДОНОРСКОЙ ПЕЧЕНИPATHOMORPHОLOGY OF BILIARY ATRESIA IN CHILDREN – RECIPIENT OF LIVERИрышкинО. Е.IryshkinO. E.ИльинскийИ. М.IljinskyI. M.ЦирульниковаО. М.TsirulnikovaO. M.ГотьеС. В.GautierS. V.ФГБУ «ФНЦ трансплантологии и искусственных органов имени академика В.И. Шумакова» Минздрава России (директор – академик РАМН, проф. С.В. Готье), Москва, Российская ФедерацияAcademician V.I. Shumakov Federal Research Center of Transplantjlogy and Artificial Organs (Director – academic of RAMSci, prof. S.V. Gautier), Moscow, Russian FederationФГБУ «ФНЦ трансплантологии и искусственных органов имени академика В.И. Шумакова» Минздрава России (директор – академик РАМН, проф. С.В. Готье), Москва, Российская Федерация Кафедра трансплантологии и искусственных органов (зав. – академик РАМН, проф. С.В. Готье) ГБОУ ВПО «Первый МГМУ им. И.М. Сеченова» (ректор – член-корр. РАМН, проф. П.В. Глыбочко), Москва, Российская ФедерацияAcademician V.I. Shumakov Federal Research Center of Transplantjlogy and Artificial Organs (Director – academic of RAMSci, prof. S.V. Gautier), Moscow, Russian Federation Chair of transplantology and artificial organs (Head – academician of RAMSci, prof. S.V. Gautier) I.M. Sechenov First Moscow State Medical University (Rector – corresponding member of RAMSci, prof. P.V. Glybochko), Moscow, Russian Federation201314052014154ВЕСТНИК ТРАНСПЛАНТОЛОГИИ И ИСКУССТВЕННЫХ ОРГАНОВ том XV No 4–20134754Copyright © Ирышкин О.Е., Ильинский И.М., Цирульникова О.М., Готье С.В., 20142014Ирышкин О.Е., Ильинский И.М., Цирульникова О.М., Готье С.В.Iryshkin O.E., Iljinsky I.M., Tsirulnikova O.M., Gautier 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://journal.transpl.ru/vtio/article/view/80

Цель работы. Изучение клинико-морфологических особенностей билиарной атрезии у детей – реципиентов донорской печени, которым выполнили операцию Kasai, по сравнению с пациентами, которым такое оперативное вмешательство не проводилось. Материал и методы. Проанализированы истории болезни и проведено гистологическое исследование удаленной печени 49 детей, страдавших билиарной атрезией, и которым была проведена трансплантация фрагмента печени. Были сформированы две группы: 1-я – 21 ребенок с билиарной атрезией, которым портоэнтеростомия не выполнялась, и 2-я – 28 детей, которым проводилась операция Kasai до трансплантации печени. Результаты. При изучении патоморфологической картины печени установлены характерные особенности в структуре пролиферирующих желчных протоков: отек и дистрофические изменения холангиоцитов у пациентов первой группы (p = 0,035) и склеротические изменения стенки желчных протоков с исчезновением эпителиальной выстилки у пациентов второй группы (p = 0,01). Также обнаружена статистически достоверная более высокая активность воспалительного процесса в печени у пациентов первой группы (p = 0,02). Заключение. На основании проведенного нами исследования можно утверждать, что операция Kasai не предотвращает фиброзирование внутрипеченочных желчных протоков и развитие фиброза, а затем и цирроза печени. 

Aim of the work. Сlinical and morphological research of the features of biliary atresia at children-recipients of liver, to whom a portoenterostomy was made in comparison with patients to whom such surgery wasn't carried out. Materials and methods. Clinical records and histological research of liver of 49 children suffering a bili- ary atresia are analyzed. Two groups were created: the 1st group – 21 children without portoenterostomy before liver transplantation and 2nd group – 28 children with portoenterostomy before liver transplantation. Results. Pathomorphology of liver the characteristics in structure of proliferation bile ducts are established: hypostasis and dystrophic changes of cholangiocytes at patients of the first group (p = 0,035) and sclerosis of bile ducts with disappearance of an epithelium at patients of the second group (p = 0,01). In first group of children activity of inflammatory process in liver were significantly higher than in second group (p = 0,02). Conclusion. Our experi- ence has demonstrated that portoenterostomy doesn’t prevent fibrosis of intrahepatic bile ducts and development of fibrosis, and then a development of cirrhosis. 

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