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”10.21508/1027-4065-2026-71-1-125-133perinatology-2369Research ArticleВ ПОМОЩЬ ПРАКТИЧЕСКОМУ ВРАЧУFOR THE PRACTITIONERПервичный склерозирующий холангит и воспалительные заболевания кишечника: современный взгляд на проблемуPrimary sclerosing cholangitis and inflammatory bowel disease in children: a modern view of the problemhttps://orcid.org/0000-0002-5413-9599ВолынецГ. В.VolynetsG. V.

Волынец Галина Васильевна – заведующий отделением гастроэнтерологии; д.м.н., профессор кафедры инновационной педиатрии и детской хирургии Института непрерывного образования и профессионального развития

125412, г. Москва, ул. Талдомская, дом 2

117997, Москва

125412, Moscow

volynec_g@mail.ruНаучно-исследовательский клинический институт педиатрии и детской хирургии им. акад. Ю.Е. Вельтищева ФГАОУ ВО РНИМУ им. Н. И. Пирогова Минздрава России; Институт непрерывного образования и профессионального развития ФГАОУ ВО РНИМУ им. Н.И.ПироговаРоссияVeltischev Research and Clinical Institute for Pediatrics and Pediatric Surgery of the Pirogov Russian National Research Medical University; Institute of Continuing Education and Professional Development Pirogov Russian National Research Medical UniversityRussian Federation202620042026711125133Copyright © Ltd. “The National Academy of Pediatric Science and Innovation”, 20262026Ltd. “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/2369

Первичный склерозирующий холангит (ПСХ) — прогрессирующее заболевание печени с хроническим течением, характеризующееся деструкцией внутри- и внепечёночных желчных протоков. Многое о первичном склерозирующем холангите остаётся неизученным, включая его основную причину. Однако, имеющиеся данные свидетельствуют о многофакторной этиологии этого заболевания, когда взаимодействие комбинации генетической предрасположенности, воздействия факторов окружающей среды, дисбаланса кишечного микробиома и нарушения иммунной регуляции запускают активацию холангиоцитов и перибилиарных миофибробластов, функционирующих согласованно, вызывая прогрессирующее фибровоспалительное разрушение желчных протоков. В статье рассматриваются вопросы сочетания первичного склерозирующего холангита и воспалительных заболеваний кишечника и способы диагностики этих состояний.

Primary sclerosing cholangitis (PSC) is a progressive, chronic liver disease characterized by destruction of the intra- and extrahepatic bile ducts. Much remains unknown about primary sclerosing cholangitis, including its underlying cause. However, available data suggest a multifactorial etiology, where the interaction of a combination of genetic predisposition, environmental factors, gut microbiome imbalance, and immune dysregulation triggers activation of cholangiocytes and peribiliary myofibroblasts that function in concert to cause progressive fibroinflammatory destruction of the bile ducts. This article discusses the association of primary sclerosing cholangitis and inflammatory bowel disease and diagnostic methods for these conditions.

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