vspВопросы современной педиатрииCurrent Pediatrics1682-55271682-5535Издательство «ПедиатрЪ»10.15690/vsp.v24i1.2861vsp-3681Research ArticleОБЗОРЫ ЛИТЕРАТУРЫLITERATURE REVIEWНаследственный панкреатит: обзорHereditary Pancreatitis: Overviewhttps://orcid.org/0000-0002-3351-6084КарпееваЮ. С.KarpeevaYuliya S.

Карпеева Юлия Сергеевна, кандидат медицинских наук, старший научный сотрудник лаборатории медико-социальных проблем в педиатрии; врач-терапевт отделения терапии и врачей общей практики 

194100, Санкт-Петербург, ул. Литовская, д. 2Е

Saint Petersburg

povetyevaj@yandex.ruhttps://orcid.org/0000-0002-7115-1768БалуковаЕ. В.BalukovaEkaterina V.

Санкт-Петербург

Saint Petersburg

https://orcid.org/0000-0002-0992-1709НовиковаВ. П.NovikovaValeriya P.

Санкт-Петербург

Saint Petersburg

https://orcid.org/0000-0001-7308-7280ХавкинА. И.KhavkinAnatoly I.

Москва

Белгород

Moscow

Belgorod

СПбГПМУ; КДЦ с поликлиникойРоссияSaint Petersburg State Pediatrics Medical University; The Consultative and Diagnostic Centre with Polyclinic of the Administration of the President of the Russian FederationRussian FederationСПбГУРоссияSaint Petersburg State UniversityRussian FederationСПбГПМУРоссияSaint Petersburg State Pediatrics Medical UniversityRussian FederationНИКИ детства; НИУ БелГУРоссияResearch and Clinical Institute for Children; Belgorod State National Research UniversityRussian Federation2025250220252411318Copyright © Карпеева Ю.С., Балукова Е.В., Новикова В.П., Хавкин А.И., 20252025Карпеева Ю.С., Балукова Е.В., Новикова В.П., Хавкин А.И.Karpeeva Y.S., Balukova E.V., Novikova V.P., Khavkin A.I.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://vsp.spr-journal.ru/jour/article/view/3681

Панкреатиты относятся к сложным в диагностическом и лечебном отношении заболеваниям органов пищеварения, при которых происходит необратимое разрушение секреторной части органа, что в итоге может привести к летальному исходу. Этиология панкреатита многообразна, обусловлена различными экзогенными и эндогенными факторами, определяющими характер и тяжесть течения патологического процесса. В качестве основных причин у детей на первый план выходят обструкция холедоха или ампулы дуоденального сосочка, травма живота, вирусные инфекции, прием ряда медикаментов. В результате многочисленных исследований эксперты обнаружили связь заболевания и генетических факторов, которые являются причиной 75% случаев хронического панкреатита. К определенным на сегодняшний день генам, ассоциированным с наследственно-обусловленным панкреатитом, относят PRSS1 (ген катионного трипсиногена), SPINK1 (ингибитор сериновой протеазы Kazal, тип 1), CFTR (трансмембранный генератор муковисцидоза), CTRC (кодирующий функции химотрипсина С). Для наследственного панкреатита установлены диаг ностические критерии — как для любого другого панкреатита. Основным методом визуализации изменений в структуре поджелудочной железы остается ультразвуковое исследование (УЗИ) органов брюшной полости, эндоскопическое УЗИ или магнитно-резонансная томография. Детям с проявлениями панкреатита рекомендованные основные обследования целесообразно дополнять генетическим тестированием.

Pancreatitis refers to digestive system diseases that are challenging to diagnose and manage. They are also associated with irreversible changes in the organ’s parenchyma, that can lead to lethal outcome. The etiology of pancreatitis is diverse due to various exogenous and endogenous factors that determine the nature and severity of the pathological process itself. The major causes in children are the following: obstruction of ductus choledochus or duodenal papilla, abdominal trauma, viral infections, and some medications. Experts have revealed, after several studies, the association between pancreatitis and genetic factors causing up ton 75% of cases of chronic pancreatitis. Nowadays, genes associated with hereditary pancreatitis include PRSS1 (cationic trypsinogen gene), SPINK1 (serine peptidase inhibitor Kazal, type 1), CFTR (cystic fibrosis transmembrane conduction regulator), CTRC (encoding the functions of chymotrypsin C). The diagnostic criteria for hereditary pancreatitis have been established as for any other pancreatitis. The leading method for visualization of changes in pancreas remains ultrasound (US) examination of abdominal organs, endoscopic US, or magnetic resonance imaging. Molecular genetic testing is advisable for children with manifestations of pancreatitis in addition to recommended basic examinations.

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