perinatologyРоссийский вестник перинатологии и педиатрииRossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)1027-40652500-2228Ltd. “The National Academy of Pediatric Science and Innovation”perinatology-176Research ArticleМАТЕРИАЛЫ XII РОССИЙСКОЙ КОНФЕРЕНЦИИ «ПЕДИАТРИЯ И ДЕТСКАЯ ХИРУРГИЯ В ПРИВОЛЖСКОМ ФЕДЕРАЛЬНОМ ОКРУГЕ»МАТЕРИАЛЫ XII РОССИЙСКОЙ КОНФЕРЕНЦИИ «ПЕДИАТРИЯ И ДЕТСКАЯ ХИРУРГИЯ В ПРИВОЛЖСКОМ ФЕДЕРАЛЬНОМ ОКРУГЕ»Современные аспекты диагностики, лечения и наблюдения детей с галактоземией I типаCurrent aspects of the diagnosis, treatment, and follow-up of children with type I galactosemiaВолгинаС. Я.VolginaS. Ya.

д.м.н., проф. каф. госпитальной педиатрии Казанского государственного медицинского университета 420012 Казань, ул. Бутлерова, д. 49

АсановА. Ю.AsanovA. Yu.

д.м.н., проф., зав. каф. медицинской генетики Первого Московского государственного медицинского университета им. И.М. Сеченова119048 Москва, ул. Трубецкая, д. 8, стр. 2

СоколовА. А.SokolovA. A.

д.м.н., проф. каф. внутренних болезней и нефрологии Северо-Западного государственного медицинского университета им. И.И. Мечникова, руководитель группы по изучению редких болезней НИИ экспериментальной медицины СЗО РАМН 191015 Санкт-Петербург, ул. Кирочная, д. 41

Казанский государственный медицинский университетРоссияKazan State Medical UniversityRussian FederationПервый Московский государственный медицинский университет им. И.М. СеченоваРоссияI.M. Sechenov First Moscow State Medical UniversityRussian FederationСеверо-Западный государственный медицинский университет им. И.И. Мечникова, Санкт-ПетербургРоссияI.I. MechnikovNorth-Western State Medical University, Saint-PetersburgRussian Federation201514032016605179187Copyright © Ltd. “The National Academy of Pediatric Science and Innovation”, 20162016Ltd. “The National Academy of Pediatric Science and Innovation”Ltd. “The National Academy of Pediatric Science and Innovation”https://www.ped-perinatology.ru/jour/about/submissions#copyrightNoticehttps://www.ped-perinatology.ru/jour/article/view/176

Рассмотрены актуальные проблемы, возникающие у детей с наиболее тяжелой формой галактоземии I типа. Описаны особенности проведения неонатального скрининга новорожденных на галактоземию. Представлены диагностические критерии классического, клинического и биохимического вариантов галактоземии. Выделены основные характеристики клинической картины и отдаленных последствий заболевания. Особое внимание уделено тактике ведения больных детей с разработкой диетической коррекции, лечением осложнений, проведением профилактических мероприятий и диспансерного наблюдения.

The paper considers the topical problems arising in children with the most severe form of type I galactosemia. It describes the specific features of neonatal screening for galactosemia. Diagnostic criteria for the classic, clinical, and biochemical variants of galactosemia are presented. The basic characteristics of the clinical picture and late sequels of the disease are identified. Particular emphasis is placed on management tactics for ill children via dietary correction, complication treatments, preventive measures, and a follow-up.

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The authors declare that there are no conflicts of interest present.