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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">perinatology</journal-id><journal-title-group><journal-title xml:lang="ru">Российский вестник перинатологии и педиатрии</journal-title><trans-title-group xml:lang="en"><trans-title>Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">1027-4065</issn><issn pub-type="epub">2500-2228</issn><publisher><publisher-name>Ltd. “The National Academy of Pediatric Science and Innovation”</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.21508/1027-4065-2024-69-3-51-54</article-id><article-id custom-type="elpub" pub-id-type="custom">perinatology-2003</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>ОРИГИНАЛЬНЫЕ СТАТЬИ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>ORIGINAL ARTICLES</subject></subj-group></article-categories><title-group><article-title>Течение и исходы беременности у женщин с синдромом Альпорта</article-title><trans-title-group xml:lang="en"><trans-title>Course and outcome of pregnancy in women with Alport syndrome</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-3699-1884</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Аксенова</surname><given-names>М. Е.</given-names></name><name name-style="western" xml:lang="en"><surname>Aksenova</surname><given-names>M. Е.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Аксенова Марина Евгеньевна — к.м.н., вед.науч. сотр. отдела наследственных и приобретенных болезней почек им. проф. М.С. Игнатовой</p><p>125412 Москва, ул. Талдомская, 2</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/0000-0002-3050-7748</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Длин</surname><given-names>В. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Dlin</surname><given-names>V. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Длин Владимир Викторович — д.м.н., рук. отдела наследственных и приобретенных болезней почек им. проф. М.С. Игнатовой</p><p>125412 Москва, ул. Талдомская, 2</p></bio><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ОСП «Научно-исследовательский клинический институт педиатрии и детской хирургии им. академика Ю.Е. Вельтищева» (Институт Вельтищева) ФГАОУ ВО РНИМУ им. Н.И. Пирогова Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Veltischev Research and Clinical Institute for Pediatrics and Pediatric Surgery of the Pirogov Russian National Research Medical University</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2024</year></pub-date><pub-date pub-type="epub"><day>12</day><month>07</month><year>2024</year></pub-date><volume>69</volume><issue>3</issue><fpage>51</fpage><lpage>54</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Ltd. “The National Academy of Pediatric Science and Innovation”, 2024</copyright-statement><copyright-year>2024</copyright-year><copyright-holder xml:lang="ru">Ltd. “The National Academy of Pediatric Science and Innovation”</copyright-holder><copyright-holder xml:lang="en">Ltd. “The National Academy of Pediatric Science and Innovation”</copyright-holder><license xlink:href="https://www.ped-perinatology.ru/jour/about/submissions#copyrightNotice" xlink:type="simple"><license-p>https://www.ped-perinatology.ru/jour/about/submissions#copyrightNotice</license-p></license></permissions><self-uri xlink:href="https://www.ped-perinatology.ru/jour/article/view/2003">https://www.ped-perinatology.ru/jour/article/view/2003</self-uri><abstract><p>Беременность у пациенток с хроническими болезнями почек ассоциируется с повышенным риском неблагоприятных исходов.</p><sec><title>Цель исследования</title><p>Цель исследования. Определение особенностей течения, исходов беременности у женщин с синдромом Альпорта.</p></sec><sec><title>Материалы и методы</title><p>Материалы и методы. В одноцентровое ретроспективное исследование включены 88 пациенток с генетически подтвержденным синдромом Альпорта. Для анализа использованы данные медицинской документации и опроса, включая информацию о клинических проявлениях и осложнениях синдрома Альпорта, терапии на момент начала, в течение и после беременности, течении беременности и родов, состоянии новорожденного. Преждевременными и ранними преждевременными считались роды на сроках гестации &lt;37 и &lt;34 нед соответственно. Длину тела новорожденного &lt;2 z-критериев (z) от нормы по отношению к гестационному возрасту расценивали как задержку внутриутробного развития плода, массу тела &lt;2 z от нормы по отношению к гестационному возрасту определяли как низкую.</p></sec><sec><title>Результаты</title><p>Результаты. В исследование вошли 88 пациенток (117 беременностей и родов: 2 беременности у 26 и 3 — у 3 женщин). Осложненное течение беременности отмечалось в 1/3 случаев (q=0,37): протеинурия (q=0,23), угроза прерывания (q=0,21), артериальная гипертензия (q=0,1). Преждевременные роды были в 1/4 случаев (q=0,26), включая ранние преждевременные — в 3%. Внутриутробная задержка развития плода и низкая гестационная масса диагностированы у 9 и 11% новорожденных соответственно; 7% детей нуждались в интенсивной терапии. Протеинурия увеличивала риск развития преэклампсии (отношение шансов — 42,35 3±1,1; p&lt;0,001), преждевременных родов (ОШ 11,8±0,5; p&lt;0,001), внутриутробной задержки развития плода (ОШ 12,2±0,7; p&lt;0,001), низкой гестационной массы новорожденного (ОШ 7,2±0,6; p&lt;0,001).</p></sec><sec><title>Заключение</title><p>Заключение. Риск осложненного течения беременности и задержки внутриутробного развития плода при синдроме Альпорта сопоставим с общепопуляционным; наличие заболевания повышает риск преждевременных родов; протеинурия служит фактором риска неблагоприятного течения и исхода беременности.</p></sec></abstract><trans-abstract xml:lang="en"><p>Pregnancy in patients with CKD is associated with maternal and fetal risks.</p><sec><title>Purpose</title><p>Purpose. To evaluate course and outcome of pregnancy in Alport syndrome women.</p></sec><sec><title>Material and methods</title><p>Material and methods. Single-center retrospective study included 88 women with genetically confirmed disease. The information about clinical data at conception, course of pregnancy, delivery (preterm delivery &lt;37 gestation weeks; early preterm delivery &lt;34 gestation weeks), fetus characteristics (intrauterine fetal growth restriction: height &lt;2 z-score for gestation age; small for gestation age: weight &lt;2 z-score) were obtained from medical charts and a cross-sectional survey of women.</p></sec><sec><title>Results</title><p>Results. Information about 117 term pregnancies (2 — in 26, 3 — in 3 patients) was obtained. The 1/3 of women (q=0.37) had complications of pregnancy: proteinuria (q=0.23), blood hypertension (q=0.1), threat of miscarriage (q=0.21). Preterm and early preterm delivery were seen in 1/4 of women (q=0.26), including early preterm in 3% of cases. Intrauterine fetal growth restriction and small for gestation age were recorded in 9% and 11% neonates, respectively; 7% of babies required a neonatal intensive care unit stay. Proteinuria was the risk factor for preeclampsia (OR=42.35 3±1.1; p&lt;0.001), preterm delivery (OR=11.8±0.5; p&lt;0.001), intrauterine fetal growth restriction (OR=12.2±0.7; p&lt;0.001), small for gestation age (OR=7.2±0.6; p&lt;0.001).</p></sec><sec><title>Conclusion</title><p>Conclusion. The risk of preeclampsia and fetal growth restriction in women with Alport syndrome and normal kidney function appears comparable to that in the general population. But the disease should be considered as a potential risk factor for preterm delivery. Proteinuria is associated with unfavorable pregnancy and fetal outcome in Alport syndrome.</p></sec></trans-abstract><kwd-group xml:lang="ru"><kwd>новорожденные</kwd><kwd>cиндром Альпорта</kwd><kwd>COL4A5</kwd><kwd>COL4A4</kwd><kwd>беременность</kwd><kwd>исходы беременности</kwd><kwd>исходы плода</kwd></kwd-group><kwd-group xml:lang="en"><kwd>newborns</kwd><kwd>Alport syndrome</kwd><kwd>COL4A5</kwd><kwd>COL4A4</kwd><kwd>pregnancy</kwd><kwd>pregnancy outcome</kwd><kwd>fetal outcome</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">Piccoli G.B., Zakharova E., Attini R., Ibarra Hernandez M., Orozco Guillien A., Alrukhaimi M. et al. Pregnancy in Chronic Kidney Disease: Need for Higher Awareness. A Pragmatic Review Focused on What Could Be Improved in the Different CKD Stages and Phases. J Clin Med 2018; 7(11): 415. 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