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<article article-type="research-article" dtd-version="1.3" xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" xml:lang="ru"><front><journal-meta><journal-id journal-id-type="publisher-id">nogr</journal-id><journal-title-group><journal-title xml:lang="ru">Экспериментальная и клиническая гастроэнтерология</journal-title><trans-title-group xml:lang="en"><trans-title>Experimental and Clinical Gastroenterology</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">1682-8658</issn><publisher><publisher-name>«Global Media Technologies»</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.31146/1682-8658-ecg-196-12-83-93</article-id><article-id custom-type="elpub" pub-id-type="custom">nogr-1803</article-id><article-categories><subj-group subj-group-type="heading"><subject>Research Article</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="ru"><subject>ОБЗОР</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>REVIEW</subject></subj-group></article-categories><title-group><article-title>Современные представления о первичном системном AL-амилоидозе: взгляд гастроэнтеролога</article-title><trans-title-group xml:lang="en"><trans-title>Modern concepts of primary systemic AL-amyloidosis: the viewpoint of a gastroenterologist</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-3655-9554</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Губергриц</surname><given-names>Н. Б.</given-names></name><name name-style="western" xml:lang="en"><surname>Gubergrits</surname><given-names>N. B.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Губергриц Наталья Борисовна, доктор мед. наук, профессор, президент Украинского клуба панкреатологов</p><p>030039, Киев, ул. 40-летия Октября, д. 2665114, Одесса, ул. Академика Королева, 17</p></bio><bio xml:lang="en"><p>Natalia B. Gubergrits, the President of Ukrainian Pancreatic Club, Dr. of Sci. (Med), Professor</p><p>030039, Kiev, 40th anniversary of October str, 26   65114, Odessa, Academician Koroleva Street, 17   </p></bio><email xlink:type="simple">profnbg@ukr.net</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Беляева</surname><given-names>Н. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Byelyayeva</surname><given-names>N. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Беляева Надежда Владимировна, кандидат мед. наук, врач-консультант медицинского центра</p><p>030039, Киев, ул. 40-летия Октября, д. 2665114, Одесса, ул. Академика Королева, 17</p></bio><bio xml:lang="en"><p>Nadezhda V. Byelyayeva, Cand. of Sci. (Med.), Consultant Doctor </p><p>030039, Kiev, 40th anniversary of October str, 26   65114, Odessa, Academician Koroleva Street, 17   </p></bio><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-8239-6093</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Можина</surname><given-names>Т. Л.</given-names></name><name name-style="western" xml:lang="en"><surname>Mozhynа</surname><given-names>T. L.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Можина Татьяна Леонидовна, кандидат мед. наук, врач-консультант</p><p>63000 Харьков, ул. Целиноградская, 58-Б</p></bio><bio xml:lang="en"><p>Tetiana L. Mozhyna, Cand. of Sci. (Med.), Consultant Doctor</p><p>63000, Kharkov, Tselinogradskaya str, 58-B  </p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-5721-6409</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Рахметова</surname><given-names>В. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Rakhmetova</surname><given-names>V. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Рахметова Венера Саметовна, доктор мед. наук, профессор кафедры внутренних болезней</p><p>010000, г. Нур-Султан, ул. Бейбитшилик, 49 а</p></bio><bio xml:lang="en"><p>Venera S. Rakhmetova, Dr. of Sci. (Med), Professor of Department of Internal Diseases</p><p>010000, Nur-Sultan city, Beybitshilik str., 49 а</p></bio><xref ref-type="aff" rid="aff-3"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Украинский Клуб панкреатологов; Медицинский центр «Медикап»</institution><country>Украина</country></aff><aff xml:lang="en"><institution>Ukrainian pancreatic Club; Medical Centre “Medikap”</institution><country>Ukraine</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>Центр здорового сердца доктора Крахмаловой</institution><country>Украина</country></aff><aff xml:lang="en"><institution>The Dr. Krakhmalova Center of the Healthy Heart</institution><country>Ukraine</country></aff></aff-alternatives><aff-alternatives id="aff-3"><aff xml:lang="ru"><institution>НАО «Медицинский университет Астана»</institution><country>Казахстан</country></aff><aff xml:lang="en"><institution>NJSC “Astana medical university”</institution><country>Kazakhstan</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2021</year></pub-date><pub-date pub-type="epub"><day>16</day><month>01</month><year>2022</year></pub-date><volume>0</volume><issue>12</issue><fpage>83</fpage><lpage>93</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Губергриц Н.Б., Беляева Н.В., Можина Т.Л., Рахметова В.С., 2022</copyright-statement><copyright-year>2022</copyright-year><copyright-holder xml:lang="ru">Губергриц Н.Б., Беляева Н.В., Можина Т.Л., Рахметова В.С.</copyright-holder><copyright-holder xml:lang="en">Gubergrits N.B., Byelyayeva N.V., Mozhynа T.L., Rakhmetova V.S.</copyright-holder><license xml:lang="ru" license-type="creative-commons-attribution" xlink:href="https://creativecommons.org/licenses/by/4.0/" xlink:type="simple"><license-p>Данная работа распространяется под лицензией Creative Commons Attribution 4.0.</license-p></license><license xml:lang="en" license-type="creative-commons-attribution" xlink:href="https://creativecommons.org/licenses/by/4.0/" xlink:type="simple"><license-p>This work is licensed under a Creative Commons Attribution 4.0 License.</license-p></license></permissions><self-uri xlink:href="https://www.nogr.org/jour/article/view/1803">https://www.nogr.org/jour/article/view/1803</self-uri><abstract><p>Цель обзора—представить анализ современных литературных данных о патогенезе, диагностике и терапии первичного системного амилоидоза легких цепей иммуноглобулинов (AL-амилоидоз), раскрыть особенности гастроэнтерологических проявлений заболевания. AL-амилоидоз обусловлен избыточным синтезом легких цепей иммуноглобулинов клоном плазматических клеток, расположенных в костном мозге, с последующим образованием амилоида.Отложение амилоида в органах мишенях (сердце, почки, печень, желудочно-кишечный тракт, периферическая и автономная нервная системы, мягкие ткани) сопровождается прямым и косвенным цитотоксическим действием на органы и ткани. К гастроинтестинальным проявлениям AL-амилоидоза относят поражение печени, желудочно-кишечные кровотечения, псевдообструкцию тонкого и/или толстого кишечника, появление полиповидных, дивертикуловидных, опухолевидных образований, мальабсорбцию, нарушение моторики желудочно-кишечного тракта, протеин-теряющую гастропатию. Повреждение печени у больных AL-амилоидозом, как правило, сопровождается минимальными клиническими проявлениями, повышением уровня щелочной фосфатазы при условии отсутствия других причин изменения концентрации этого маркера холестаза.Приведен детальный анализ положений практического руководства клиники Мэйо по диагностике, лечению AL-амилоидоза. Диагностика гастроинтестинального AL-амилоидоза основывается на гистомикроскопическом анализе биоптатов пораженных органов с окраской конго красным и последующим осмотром в поляризованном свете; золотым стандартом диагностики заболевания является масс-спектрометрия. Современная фармакотерапия AL-амилоидоза предполагает проведение комбинированной высокодозовой химиотерапии с применением моноклональных антител, ингибиторов протеасом, цитостатиков, гормонов, а также выполнение аутологической трансплантации стволовых клеток. Коррекция гастроэнтерологических проявлений заболевания основана на назначении симптоматических средств. Продолжительность жизни больных AL-амилоидозом определяют посредством нескольких прогностических моделей; шкала Бостонского университета, основанная на определении двух показателей, максимально удобна для применения в практической деятельности.</p></abstract><trans-abstract xml:lang="en"><p>The purpose of the review is to present an analysis of modern literature data on the pathogenesis, diagnosis and therapy of primary systemic amyloidosis of immunoglobulin light chains (AL-amyloidosis), to reveal the features of gastroenterological manifestations of the disease. AL-amyloidosis is caused by overproduction of immunoglobulin light chains by a clone of plasma cells located in the bone marrow, followed by the formation of amyloid.Deposition of amyloid in target organs (heart, kidneys, liver, gastrointestinal tract, peripheral and autonomic nervous systems, soft tissues) is accompanied by direct and indirect cytotoxic effects on organs and tissues. Gastrointestinal manifestations of AL-amyloidosis include liver damage, gastrointestinal bleeding, pseudo-obstruction of the small intestine and colon, the appearance of polyp-, diverticul-, tumor-like formations, malabsorption, impaired motility of the gastrointestinal tract, protein-losing gastropathy. Liver damage in patients with AL-amyloidosis, as a rule, is accompanied by minimal clinical manifestations, an increase of the level of alkaline phosphatase without any other reasons for this change. A detailed analysis of the Mayo Clinic’s practical guidelines for the diagnosis and treatment of AL-amyloidosis is presented. Diagnosis of gastrointestinal AL-amyloidosis is based on histomicroscopic analysis of biopsies of target organs with Congo red and subsequent examination in polarized light; mass spectrometry is the gold standard of diagnostic. Modern pharmacotherapy of AL-amyloidosis includes a combination of high-dose chemotherapy with monoclonal antibodies, proteasome inhibitors, cytostatics, hormones, as well as performing autologous stem cell transplantation. Correction of gastroenterological manifestations of the disease is based on the symptomatic therapy. Life expectancy of patients with AL-amyloidosis is determined by several prognostic models; the Boston University model, based on the definition of two markers, is most convenient for clinical use.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>амилоид</kwd><kwd>AL-амилоидоз</kwd><kwd>гастроинтестинальные проявления</kwd></kwd-group><kwd-group xml:lang="en"><kwd>amyloid</kwd><kwd>AL-amyloidosis</kwd><kwd>gastrointestinal manifestations</kwd></kwd-group></article-meta></front><back><ref-list><title>References</title><ref id="cit1"><label>1</label><citation-alternatives><mixed-citation xml:lang="ru">Benson M.D., Buxbaum J. N., Eisenberg D. S., et al. 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