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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">patmedfar</journal-id><journal-title-group><journal-title xml:lang="ru">Пациентоориентированная медицина и фармация</journal-title><trans-title-group xml:lang="en"><trans-title>Patient-Oriented Medicine and Pharmacy</trans-title></trans-title-group></journal-title-group><issn pub-type="epub">2949-1924</issn><publisher><publisher-name>LLC Izdatelstvo OKI</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.37489/2949-1924-0079</article-id><article-id custom-type="edn" pub-id-type="custom">FRIMHH</article-id><article-id custom-type="elpub" pub-id-type="custom">patmedfar-137</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>УРОЛОГИЯ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>UROLOGY</subject></subj-group></article-categories><title-group><article-title>Наследственный амилоидоз с поражением почек как клиническая проблема: случай из практики</article-title><trans-title-group xml:lang="en"><trans-title>Hereditary amyloidosis with renal damage as a clinical problem: a case report</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-2062-0421</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Шорманов</surname><given-names>И. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Shormanov</surname><given-names>I. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Шорманов Игорь Сергеевич — д. м. н., профессор, зав. кафедрой урологии с нефрологией.</p><p>Ярославль</p></bio><bio xml:lang="en"><p>Igor S. Shormanov — Dr. Sci (Med.), Professor, Head of the Department of Urology with Nephrology.</p><p>Yaroslavl</p></bio><email xlink:type="simple">i-s-shormanov@yandex.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-2464-572X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Жигалов</surname><given-names>С. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Zhigalov</surname><given-names>S. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Жигалов Сергей Алексеевич — к. м. н., доцент кафедры урологии с нефрологией.</p><p>Ярославль</p></bio><bio xml:lang="en"><p>Sergey A. Zhigalov — Cand. Sci. (Med.), Associate Professor, Department of Urology with Nephrology.</p><p>Yaroslavl</p></bio><email xlink:type="simple">sergey.zhigalow@gmail.com</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-5612-3227</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Соловьёв</surname><given-names>А. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Solovyev</surname><given-names>A. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Соловьёв Андрей Сергеевич — к. м. н., доцент кафедры урологии с нефрологией.</p><p>Ярославль</p></bio><bio xml:lang="en"><p>Andrey S. Solovyov — Cand. Sci. (Med.), Associate Professor, Department of Urology with Nephrology.</p><p>Yaroslavl</p></bio><email xlink:type="simple">a-s-soloviev89@yandex.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0009-0004-5741-261X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Бажина</surname><given-names>О. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Bazhina</surname><given-names>O. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Бажина Ольга Викторовна — к. м. н., доцент кафедры урологии с нефрологией, РИНЦ AuthorID: 266698.</p><p>Ярославль</p></bio><bio xml:lang="en"><p>Olga V. Bazhina — Cand. Sci. (Med.), Associate Professor, Department of Urology with Nephrology, RSCI AuthorID: 266698.</p><p>Yaroslavl</p></bio><email xlink:type="simple">mascot212@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0009-0002-4547-224X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Ганерт</surname><given-names>О. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Ganert</surname><given-names>O. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Ганерт Ольга Александровна — врач-нефролог.</p><p>Ярославль</p></bio><bio xml:lang="en"><p>Olga A. Ganert — MD, nephrologist.</p><p>Yaroslavl</p></bio><email xlink:type="simple">olgaganert@mail.ru</email><xref ref-type="aff" rid="aff-2"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ФГБОУ ВО «Ярославский государственный медицинский университет»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Yaroslavl State Medical University</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>ГБУЗ Ярославской области «Областная клиническая больница»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Yaroslavl region "Regional Clinical Hospital"</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2025</year></pub-date><pub-date pub-type="epub"><day>24</day><month>03</month><year>2025</year></pub-date><volume>3</volume><issue>1</issue><fpage>37</fpage><lpage>41</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Шорманов И.С., Жигалов С.А., Соловьёв А.С., Бажина О.В., Ганерт О.А., 2025</copyright-statement><copyright-year>2025</copyright-year><copyright-holder xml:lang="ru">Шорманов И.С., Жигалов С.А., Соловьёв А.С., Бажина О.В., Ганерт О.А.</copyright-holder><copyright-holder xml:lang="en">Shormanov I.S., Zhigalov S.A., Solovyev A.S., Bazhina O.V., Ganert O.A.</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.pomph.ru/jour/article/view/137">https://www.pomph.ru/jour/article/view/137</self-uri><abstract><p>Данная статья описывает клинический случай 28-летней женщины с длительной историей хронической анемии (наблюдение с 2017 г.), гепатоспленомегалии, мезентериальной лимфаденопатии и протеинурии, первоначально диагностированной как хронический тубулоинтерстициальный нефрит, 2 стадия хронической болезни почек. В апреле 2024 года пациентка была госпитализирована для уточнения диагноза: проведён ряд лабораторно-инструментальных исследований, в том числе нефробиопсия, выполненная с использованием стандартных методик, которая не выявила амилоидных отложений. В ноябре 2024 года, на основании повторной оценки ранее полученных нефробиоптатов с применением дополнительных методов окрашивания (иммунофлуоресценция и иммуногистохимия), обнаружены массивные амилоидные отложения, преимущественно состоящие из лизоцима, что позволило установить окончательный диагноз — генетически детерминированный лизоцимовый амилоидоз почек. Этот случай подчёркивает важность динамического наблюдения, повторного морфологического анализа при расхождении клинической и морфологической картин и применения специализированных методов исследования для уточнения редких вариантов амилоидоза.</p></abstract><trans-abstract xml:lang="en"><p>This article describes a clinical case of a 28-year-old woman with a long history of chronic anemia (observed since 2017), hepatosplenomegaly, mesenteric lymphadenopathy, and proteinuria, initially diagnosed as chronic tubulointerstitial nephritis with CKD Stage 2. In April 2024, the patient was hospitalized to clarify the diagnosis: a series of laboratory and instrumental studies were conducted, including a nephrobiopsy using standard methods, which did not reveal amyloid deposits. In November 2024, based on a re-evaluation of previously obtained nephrobiopsies using additional staining methods (Congo red staining, immunofluorescence, and immunohistochemistry), massive amyloid deposits were detected, predominantly consisting of lysozyme, which allowed establishing the final diagnosis — genetically determined lysozyme amyloidosis of the kidneys. This case underscores the importance of dynamic observation and repeated morphological analysis in cases of clinical discordance and the use of specialized research methods to clarify rare variants of amyloidosis.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>лизоцимовый амилоидоз</kwd><kwd>почки</kwd><kwd>редкое заболевание</kwd><kwd>комплексная диагностика</kwd><kwd>генетическая предрасположенность</kwd><kwd>протеинурия</kwd><kwd>иммуногистохимия</kwd></kwd-group><kwd-group xml:lang="en"><kwd>lysozyme amyloidosis</kwd><kwd>kidneys</kwd><kwd>rare disease</kwd><kwd>comprehensive diagnostics</kwd><kwd>genetic predisposition</kwd><kwd>proteinuria</kwd><kwd>immunohistochemistry</kwd></kwd-group><funding-group><funding-statement xml:lang="ru">Работа выполнялась без спонсорской поддержки</funding-statement><funding-statement xml:lang="en">The work was carried out without sponsorship</funding-statement></funding-group></article-meta></front><back><ref-list><title>References</title><ref id="cit1"><label>1</label><citation-alternatives><mixed-citation xml:lang="ru">Merlini G, Bellotti V. 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