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 <front>
  <journal-meta>
   <journal-id journal-id-type="publisher-id">Actual problems in dentistry</journal-id>
   <journal-title-group>
    <journal-title xml:lang="en">Actual problems in dentistry</journal-title>
    <trans-title-group xml:lang="ru">
     <trans-title>Проблемы стоматологии</trans-title>
    </trans-title-group>
   </journal-title-group>
   <issn publication-format="print">2077-7566</issn>
   <issn publication-format="online">2412-9461</issn>
  </journal-meta>
  <article-meta>
   <article-id pub-id-type="publisher-id">125468</article-id>
   <article-id pub-id-type="doi">10.18481/2077-7566-2026-22-2-171-177</article-id>
   <article-categories>
    <subj-group subj-group-type="toc-heading" xml:lang="ru">
     <subject>СТОМАТОЛОГИЯ ДЕТСКОГО ВОЗРАСТА, ПРОФИЛАКТИКА И ОРТОДОНТИЯ. ОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯ</subject>
    </subj-group>
    <subj-group subj-group-type="toc-heading" xml:lang="en">
     <subject>PEDIATRIC DENTISTRY, PROPHYLAXIS AND ORTHODONTICS. ORIGINAL RESEARCH PAPERS</subject>
    </subj-group>
    <subj-group>
     <subject>СТОМАТОЛОГИЯ ДЕТСКОГО ВОЗРАСТА, ПРОФИЛАКТИКА И ОРТОДОНТИЯ. ОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯ</subject>
    </subj-group>
   </article-categories>
   <title-group>
    <article-title xml:lang="en">EVALUATION OF THE EFFECTIVENESS OF DENTAL REHABILITATION OF CHILDREN WITH ORPHAN DISEASES AND DISORDERS OF PHOSPHORUS-CALCIUM METABOLISM BASED ON QUALITY OF LIFE CRITERIA</article-title>
    <trans-title-group xml:lang="ru">
     <trans-title>ОЦЕНКА ЭФФЕКТИВНОСТИ СТОМАТОЛОГИЧЕСКОЙ РЕАБИЛИТАЦИИ ДЕТЕЙ С ОРФАННЫМИ ЗАБОЛЕВАНИЯМИ С НАРУШЕНИЕМ ФОСФОРНО-КАЛЬЦИЕВОГО ОБМЕНА ПО КРИТЕРИЯМ КАЧЕСТВА ЖИЗНИ</trans-title>
    </trans-title-group>
   </title-group>
   <contrib-group content-type="authors">
    <contrib contrib-type="author">
     <contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-9409-3046</contrib-id>
     <name-alternatives>
      <name xml:lang="ru">
       <surname>Алексеева</surname>
       <given-names>Ирина Александровна</given-names>
      </name>
      <name xml:lang="en">
       <surname>Alekseeva</surname>
       <given-names>Irina Aleksandrovna</given-names>
      </name>
     </name-alternatives>
     <email>alexeeva.penza@yandex.ru</email>
     <bio xml:lang="ru">
      <p>доктор медицинских наук;</p>
     </bio>
     <bio xml:lang="en">
      <p>doctor of medical sciences;</p>
     </bio>
     <xref ref-type="aff" rid="aff-1"/>
    </contrib>
    <contrib contrib-type="author">
     <contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-2095-9473</contrib-id>
     <name-alternatives>
      <name xml:lang="ru">
       <surname>Кисельникова</surname>
       <given-names>Лариса Петровна</given-names>
      </name>
      <name xml:lang="en">
       <surname>Kisel'nikova</surname>
       <given-names>Larisa P.</given-names>
      </name>
     </name-alternatives>
     <email>lpkiselnikova@mail.ru</email>
     <bio xml:lang="ru">
      <p>доктор медицинских наук;</p>
     </bio>
     <bio xml:lang="en">
      <p>doctor of medical sciences;</p>
     </bio>
     <xref ref-type="aff" rid="aff-2"/>
    </contrib>
    <contrib contrib-type="author">
     <name-alternatives>
      <name xml:lang="ru">
       <surname>Ковылина</surname>
       <given-names>Ольга Сергеевна</given-names>
      </name>
      <name xml:lang="en">
       <surname>Kovylina</surname>
       <given-names>Olga S.</given-names>
      </name>
     </name-alternatives>
     <email>kovylina.os@gmail.com</email>
     <bio xml:lang="ru">
      <p>кандидат медицинских наук;</p>
     </bio>
     <bio xml:lang="en">
      <p>candidate of medical sciences;</p>
     </bio>
     <xref ref-type="aff" rid="aff-3"/>
    </contrib>
    <contrib contrib-type="author">
     <name-alternatives>
      <name xml:lang="ru">
       <surname>Цымлянская</surname>
       <given-names>Виктория Викторовна</given-names>
      </name>
      <name xml:lang="en">
       <surname>Tsymlyanskaya</surname>
       <given-names>Viktoria V.</given-names>
      </name>
     </name-alternatives>
     <email>dr.tsymlyanskaya@mail.ru</email>
     <xref ref-type="aff" rid="aff-4"/>
    </contrib>
   </contrib-group>
   <aff-alternatives id="aff-1">
    <aff>
     <institution xml:lang="ru">Московский государственный медико-стоматологический университет имени А.И. Евдокимова</institution>
    </aff>
    <aff>
     <institution xml:lang="en">Moscow State University of Medicine and Dentistry</institution>
    </aff>
   </aff-alternatives>
   <aff-alternatives id="aff-2">
    <aff>
     <institution xml:lang="ru">Российский университет медицины</institution>
     <city>Москва</city>
     <country>Россия</country>
    </aff>
    <aff>
     <institution xml:lang="en">Russian University of Medicine</institution>
     <city>Moskow</city>
     <country>Russian Federation</country>
    </aff>
   </aff-alternatives>
   <aff-alternatives id="aff-3">
    <aff>
     <institution xml:lang="ru">Российский университет медицины</institution>
     <city>Москва</city>
     <country>Россия</country>
    </aff>
    <aff>
     <institution xml:lang="en">Russian University of Medicine</institution>
     <city>Moscow</city>
     <country>Russian Federation</country>
    </aff>
   </aff-alternatives>
   <aff-alternatives id="aff-4">
    <aff>
     <institution xml:lang="ru">Московский государственный медико-стоматологический университет им. А. И. Евдокимова</institution>
     <city>Москва</city>
     <country>Россия</country>
    </aff>
    <aff>
     <institution xml:lang="en">Moscow State University of Medicine and Dentistry A. I. Evdokimova</institution>
     <city>Moscow</city>
     <country>Russian Federation</country>
    </aff>
   </aff-alternatives>
   <pub-date publication-format="print" date-type="pub" iso-8601-date="2026-06-29T19:24:06+03:00">
    <day>29</day>
    <month>06</month>
    <year>2026</year>
   </pub-date>
   <pub-date publication-format="electronic" date-type="pub" iso-8601-date="2026-06-29T19:24:06+03:00">
    <day>29</day>
    <month>06</month>
    <year>2026</year>
   </pub-date>
   <volume>22</volume>
   <issue>2</issue>
   <fpage>171</fpage>
   <lpage>177</lpage>
   <history>
    <date date-type="received" iso-8601-date="2026-06-07T00:00:00+03:00">
     <day>07</day>
     <month>06</month>
     <year>2026</year>
    </date>
   </history>
   <self-uri xlink:href="https://dental-press.ru/en/nauka/article/125468/view">https://dental-press.ru/en/nauka/article/125468/view</self-uri>
   <abstract xml:lang="ru">
    <p>В последние десятилетия оценка качества жизни стала неотъемлемой частью комплексного подхода к ведению пациентов с  редкими заболеваниями.&#13;
Интегральная оценка влияния стоматологического здоровья на ключевые сферы жизнедеятельности становится составляющей комплексной реабилитации детей, страдающих редкими заболеваниями, направленной на восстановление качества жизни.&#13;
Цель: изучить влияние стоматологической реабилитации на динамику  критериев качества жизни у детей с редкими заболеваниями фосфорно-кальциевого обмена с использованием опросника OHRQoL.&#13;
Материалы и методы&#13;
В группах детей (6–17 лет) с генетическими заболеваниями: Х-сцепленным гипофосфатемическим рахитом (Х-ГФР), гипофосфатазией (ГФФ), несовершенным остеогенезом (НО) было проведено клиническое и социологическое исследования. &#13;
Изучено влияние стоматологической реабилитации на состояние тканей зубов у детей по клиническим индексам (OHI-S, CAST) и динамику критериев качества жизни с использованием родительского опросника Oral Health-Related Quality of Life (OHRQoL).&#13;
Анкета (опросник) (OHRQоL) – инструмент оценки влияния стоматологических проблем на различные сферы повседневной жизни, отражающий физический дискомфорт в полости рта; коммуникативный барьер ребенка; поддержку и решение стоматологических проблем законными представителями.&#13;
Результаты &#13;
Реализация стоматологической реабилитации в группах детей с Х-ГФР,  ГФФ, НО показала значительную клиническую эффективность и снижение физического дискомфорта в полости рта, выраженное редукцией показателей функционального домена опросника OHRQоL. Наряду с тем, выявлено, что у родителей детей, страдающих орфанными заболеваниями, повысилась приверженность к стоматологическому лечению и профилактическим мероприятиям.</p>
   </abstract>
   <trans-abstract xml:lang="en">
    <p>In recent decades, quality of life assessment has become an integral part of a comprehensive approach to managing patients with rare diseases.&#13;
An integrated assessment of the impact of dental health on key areas of life is becoming a component of comprehensive rehabilitation for children with rare diseases, aimed at restoring quality of life.&#13;
Objective: To study the impact of dental rehabilitation on the dynamics of quality of life criteria in children with rare phosphorus-calcium metabolism disorders using the OHRQoL questionnaire.&#13;
Materials and Methods&#13;
A clinical and sociological study was conducted in groups of children (6–17 years) with the genetic diseases X-linked hypophosphatemic rickets (X-HPR), hypophosphatasia (HPP), and osteogenesis imperfecta (OI). &#13;
The effects of dental rehabilitation on the condition of dental tissues in children were studied according to clinical indices (OHI-S, CAST) and the dynamics of quality of life criteria using the Oral Health-Related Quality of Life (OHRQoL) parental questionnaire. &#13;
The questionnaire (OHRQoL) is a tool for assessing the impact of dental problems on various areas of daily life, reflecting physical discomfort in the oral cavity; the child's communication difficulties; support and resolution of dental problems by legal representatives.&#13;
Results. Dental rehabilitation in groups of children with X-linked hypophosphatemic rickets (X-HPR), hypophosphatasia (HPP), and osteogenesis imperfecta (OI) demonstrated significant clinical efficacy and a reduction in physical discomfort in the oral cavity, as evidenced by a reduction in the functional domain scores of the OHRQoL questionnaire. Furthermore, it was found that parents of children with orphan diseases showed increased adherence to dental treatment and preventive measures.</p>
   </trans-abstract>
   <kwd-group xml:lang="ru">
    <kwd>дети с орфанными заболеваниями</kwd>
    <kwd>стоматологический статус</kwd>
    <kwd>оценка качества жизни</kwd>
    <kwd>анкета опросник Oral Health-Related Quality of Life (OHRQоL)</kwd>
    <kwd>стоматологическая реабилитация</kwd>
   </kwd-group>
   <kwd-group xml:lang="en">
    <kwd>children with orphan diseases</kwd>
    <kwd>dental status</kwd>
    <kwd>quality of life assessment</kwd>
    <kwd>Oral Health-Related Quality of Life (OHRQoL) questionnaire</kwd>
    <kwd>dental rehabilitation</kwd>
   </kwd-group>
  </article-meta>
 </front>
 <body>
  <p></p>
 </body>
 <back>
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