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 Federation10.15825/1995-1191-2025-4-67-73vtio-1917Research ArticleКлиническая трансплантологияClinical TransplantologyПедиатрическая трансплантация печени в Узбекистане: первый клинический случай и анализ результатовPediatric liver transplantation in Uzbekistan: first clinical case and outcome analysishttps://orcid.org/0000-0001-5830-5235СёмашК. О.SemashK. O.

Семаш Константин Олесьевич

100171, Ташкент, Яшнабадский р-н, ул. Паркентская, д. 294

Konstantin Semash

Address: 294, Parkentskaya str., Yashnobod District, Tashkent, 100171, Uzbekistan

doctorsemash@gmail.comhttps://orcid.org/0000-0002-8857-947XДжанбековТ. А.DzhanbekovT. A.

Тимур Айдарбекович Джанбеков 

Ташкент

Tashkent

house-md@mail.ruhttps://orcid.org/0000-0002-1365-9125НасыровМ. М.NasyrovM. M.

Мансур Мухторович Насыров 

Ташкент

Tashkent

mansoornasyrov@gmail.comhttps://orcid.org/0000-0002-3537-6601МонаховА. Р.MonakhovA. R.

Артём Рашидович Монахов 

Москва

Moscow

a.r.monakhov@gmail.comhttps://orcid.org/0009-0000-8940-8835ГаджиеваП. М.GadzhievaP. M.

Патимат Магомедовна Гаджиева

Москва

Moscow

gadzievap80@gmail.comМасютинС. А.MasyutinS. A.

Сергей Александрович Масютин 

Москва

Moscow

sergmas@mail.ruНациональный детский медицинский центрУзбекистанNational Children’s Medical CenterUzbekistanФГБУ «Национальный медицинский исследовательский центр трансплантологии и искусственных органов имени академика В.И. Шумакова» Минздрава РоссииРоссияShumakov National Medical Research Center of Transplantology and Artificial OrgansRussian Federation2025100120262746773Copyright © Сёмаш К.О., Джанбеков Т.А., Насыров М.М., Монахов А.Р., Гаджиева П.М., Масютин С.А., 20262026Сёмаш К.О., Джанбеков Т.А., Насыров М.М., Монахов А.Р., Гаджиева П.М., Масютин С.А.Semash K.O., Dzhanbekov T.A., Nasyrov M.M., Monakhov A.R., Gadzhieva P.M., Masyutin S.A.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/1917

Введение: Трансплантация печени является единственным жизнеспасающим методом лечения детей с терминальной стадией заболеваний печени. В Узбекистане программа трансплантации печени была инициирована в 2018 году, однако педиатрическая трансплантация печени до недавнего времени не проводилась.

Цель: Представить первый задокументированный случай родственной педиатрической трансплантации печени в Республике Узбекистан, а также обсудить ключевые аспекты послеоперационного ведения, включая криз отторжения, реактивацию аутоиммунного гепатита и инновационное применение бортезомиба как терапевтического агента при лечении стероидрезистентных отторжений.

Материалы и методы: 15-летний пациент с циррозом печени в исходе аутоиммунного гепатита был подготовлен к трансплантации правой доли печени от живого донора. Проведена тщательная предоперационная подготовка, хирургическое вмешательство и многоэтапное послеоперационное лечение.

Результаты: Операция прошла успешно, но в раннем послеоперационном периоде развился криз отторжения, устойчивый к стандартной терапии глюкокортикостероидами и антитимоцитарным иммуноглобулином. Диагностирована реактивация аутоиммунного гепатита, для лечения которой был применён бортезомиб. В результате терапии наблюдалась нормализация лабораторных показателей и восстановление функции трансплантата.

Заключение: Первый случай педиатрической трансплантации печени в Узбекистане продемонстрировал возможность успешного выполнения сложного хирургического вмешательства и эффективного управления послеоперационными осложнениями. Применение бортезомиба при стероид-резистентном отторжении и реактивации аутоиммунного гепатита может стать перспективным направлением в лечении подобных состояний. Данный опыт открывает новые горизонты для развития трансплантологической помощи в стране.

Background

Background. Liver transplantation (LT) remains the only life-saving option for children with end-stage liver disease. In Uzbekistan, a national LT program was launched in 2018; however, pediatric LT had not been performed until recently.

Objective

Objective: to report the fi rst documented case of related pediatric LT in the Republic of Uzbekistan and to highlight key aspects of postoperative management, including rejection crises, recurrent autoimmune hepatitis (AIH), and the innovative use of bortezomib for treating steroid-resistant rejection.

Materials and methods

Materials and methods. A 15-year-old patient with liver cirrhosis secondary to AIH was selected for transplantation. The right hepatic lobe from a living donor was transplanted following comprehensive preoperative evaluation and preparation. The procedure involved surgical intervention followed by a multistage postoperative treatment protocol.

Results

Results. The transplant procedure was successful. However, in the early postoperative period, the patient developed a rejection crisis that proved resistant to standard therapy with glucocorticosteroids and antithymocyte globulin. Subsequent evaluation revealed a recurrent AIH. Bortezomib was administered as part of the therapeutic strategy, leading to normalization of laboratory parameters and restoration of graft function.

Conclusion

Conclusion. This fi rst case of pediatric LT in Uzbekistan demonstrates the feasibility of performing complex surgical interventions and managing challenging postoperative complications. The use of bortezomib for steroid-resistant rejection associated with AIH highlights a potentially promising therapeutic approach. These results mark an important step forward in the development of transplant care in the country.

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