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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">pediatricjournal</journal-id><journal-title-group><journal-title xml:lang="ru">Архив педиатрии и детской хирургии</journal-title><trans-title-group xml:lang="en"><trans-title>Archives of Pediatrics and Pediatric Surgery</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">2949-4664</issn><issn pub-type="epub">3033-6783</issn><publisher><publisher-name>НИКИ детства Минздрава Московской области</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.31146/2949-4664-apps-2-1-163-169</article-id><article-id custom-type="elpub" pub-id-type="custom">pediatricjournal-70</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>КЛИНИЧЕСКИЙ СЛУЧАЙ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>CLINICAL CASE</subject></subj-group></article-categories><title-group><article-title>Белок теряющая энтеропатия после операции Фонтена: описание клинического случая</article-title><trans-title-group xml:lang="en"><trans-title>Protein-Losing Enteropathy after Fontan Operation: A Clinical Case Description</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-1695-0978</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Камилова</surname><given-names>А. Т.</given-names></name><name name-style="western" xml:lang="en"><surname>Kamilova</surname><given-names>A. T.</given-names></name></name-alternatives><email xlink:type="simple">noemail@neicon.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/0009-0009-6384-8176</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Усманова</surname><given-names>К. Ш.</given-names></name><name name-style="western" xml:lang="en"><surname>Usmanova</surname><given-names>K. Sh.</given-names></name></name-alternatives><email xlink:type="simple">kamola.u.sh@gmail.com</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>Убайходжаева</surname><given-names>Х. Т.</given-names></name><name name-style="western" xml:lang="en"><surname>Ubayxodjayeva</surname><given-names>X. T.</given-names></name></name-alternatives><email xlink:type="simple">noemail@neicon.ru</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Республиканский специализированный научно-практический медицинский центр педиатрии Министерства Здравоохранения РУз</institution></aff><aff xml:lang="en"><institution>Republican Specialized Scientific and Practical Medical Center of Pediatrics of the Ministry of Health of the Republic of Uzbekistan</institution></aff></aff-alternatives><pub-date pub-type="collection"><year>2024</year></pub-date><pub-date pub-type="epub"><day>26</day><month>03</month><year>2024</year></pub-date><volume>2</volume><issue>1</issue><fpage>163</fpage><lpage>169</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Камилова А.Т., Усманова К.Ш., Убайходжаева Х.Т., 2024</copyright-statement><copyright-year>2024</copyright-year><copyright-holder xml:lang="ru">Камилова А.Т., Усманова К.Ш., Убайходжаева Х.Т.</copyright-holder><copyright-holder xml:lang="en">Kamilova A.T., Usmanova K.S., Ubayxodjayeva X.T.</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://journal.nikid.ru/jour/article/view/70">https://journal.nikid.ru/jour/article/view/70</self-uri><abstract><p>Приведено собственное клиническое наблюдение мальчика с белок-теряющей энтеропатией после операции Фонтена. Мальчик С. М., 2 года, поступил с жалобами на боли в животе, вздутие и увеличение живота, частый жидкий стул, периферические отеки на руках и ногах. У ребенка в родильном доме был диагностирован врожденный порок сердца, для коррекции которого проведена операция Фонтена. Через 1 месяц появилась диарея и отёки на лице, руках и ногах. Отеки на время устранялись симптоматической терапией. В возрасте 2х лет ребенок был госпитализирован в отделение гастроэнтерологии нашего центра. При обследовании установлены низкие значения общего белка и альбумина, повышение значений альфа 1-антитрипсина. Был установлен диагноз белок-теряющая энтеропатия после операции Фонтена. Была назначена диета, обогащенная среднецепочечными триглицеридами, верошпирон и преднизолон. Через 6 месяцев в катамнезе отеков не наблюдалось, общее состояние ребенка было удовлетворительным. Однако в течении последнего года до настоящего времени у мальчика вновь появились незначительные отеки на руках, ногах и на лице, в связи с чем он периодически получает альбуминотрансфузию. Операция Фонтена и белок-теряющая энтеропатия представляют собой разные аспекты медицины, однако иногда один диагноз становится следствием другого. Мы коллектив авторов провели исследование, обработали, получили результат, и мы считаем, что мы правы, и заявляем, что ответственность за результат и активность в написании материала остается за авторами.</p></abstract><trans-abstract xml:lang="en"><p>The clinical observation of a boy with protein-losing enteropathy after the Fontan operation is presented. The boy, S.M., 2 years old, was admitted with complaints of abdominal pain, bloating, enlarged abdomen, frequent watery stools, and peripheral edema in the arms and legs. The child was diagnosed with a congenital heart defect at birth, for which Fontan surgery was performed. One month later, diarrhea and swelling appeared on the face, arms, and legs. Edema was temporarily relieved by symptomatic therapy. At the age of 2, the child was hospitalized in the gastroenterology department of our center. Examinations revealed low levels of total protein and albumin, and an increase in alpha-1 antitrypsin values. The diagnosis of protein-losing enteropathy after the Fontan operation was established. The child was prescribed a diet enriched with medium-chain triglycerides, spironolactone, and prednisolone. After 6 months of follow-up, no edema was observed, and the child’s overall condition was satisfactory. However, over the past year until the present, the boy has experienced slight swelling in the arms, legs, and face, requiring periodic albumin transfusions. Fontan operation and protein-losing enteropathy represent different aspects of medicine; however, sometimes one diagnosis becomes a consequence of another. As a collective of authors, we conducted the research, processed the data, obtained results, and we believe that we are correct. We affirm that the responsibility for the results and activity in writing the material remains with the authors.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>Белок-теряющая энтеропатия</kwd><kwd>операция Фонтена</kwd><kwd>врожденный порок сердца</kwd><kwd>клиническое наблюдение</kwd></kwd-group><kwd-group xml:lang="en"><kwd>Protein-Losing Enteropathy</kwd><kwd>Fontan Operation</kwd><kwd>Congenital Heart Defect</kwd><kwd>Clinical Observation</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">Ozen A., Lenardo M. J. Protein-Losing Enteropathy. N Engl J Med. 2023 Aug 24;389(8):733-748. doi: 10.1056/NEJMra2301594.</mixed-citation><mixed-citation xml:lang="en">Ozen A., Lenardo M. J. Protein-Losing Enteropathy. 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