Russian Journal of Pediatric SurgeryRussian Journal of Pediatric SurgeryДетская хирургия1560-95102412-0677Eco-Vector72610.17816/ps726CASE REPORTКЛИНИЧЕСКАЯ ПРАКТИКАResearch ArticleSurgical treatment of hereditary pancreatitis in four children in one and the same familyСерия клинических случаев хирургического лечения наследственного панкреатита у четверых детей в одной семьеhttps://orcid.org/0000-0002-5530-4410КislenkoAlina A.КисленкоАлина Александровна
Russian Federation

MD

kislenkolina@mail.ruhttps://orcid.org/0000-0002-9497-40703600-4701RazumovskiyAlexandr Yu.РазумовскийАлександр Юрьевич
Russian Federation

MD, Dr. Sci. (Medicine), Professor

д-р мед. наук, проф.

1595105@mail.ruhttps://orcid.org/0000-0002-3463-9799KholostovaVictoriya V.ХолостоваВиктория Валерьевна
Russian Federation

MD, Dr. Sci. (Medicine)

д-р мед. наук

vkholostova@yandex.ruThe Russian National Research Medical University named after N.I. PirogovРоссийский национальный исследовательский медицинский университет имени Н.И. ПироговаFilatov N.F. Children's City HospitalДетская городская клиническая больница имени Н.Ф. Филатова030420242811051132308202326102023Copyright ©; 2024, Eco-VectorCopyright ©; 2024, Эко-Вектор2024Eco-VectorЭко-Векторhttps://jps-nmp.ru/jour/article/view/726

BACKGROUND: Hereditary pancreatitis is an autosomal dominant disease with high gene penetration (80%), manifested with signs of chronic and recurrent pancreatitis. This pathology is rarely met in children. In the world literature, the idea of surgical approach in treating hereditary pancreatitis is evidently supported because it helps to eliminate symptoms of pancreatic obstruction, to prevent complications and to slow down the process of pancreatic atrophy.

CLINICAL CASE DESCRIPTION: The article describes a case of hereditary pancreatitis with an autosomal dominant type of inheritance, with a mutation in PRSS1 gene in children in one and the same family. Due to the longitudinal pancreatic jejunostomy, patients could have a good clinical remission.

CONCLUSION: The discussed clinical case describes a successful surgical technique which has given good outcomes in treating hereditary pancreatitis in children.

Обоснование. Наследственный панкреатит — это аутосомно-доминантное заболевание с высокой (80%) пенетрантностью генов, проявляющееся признаками хронического или рецидивирующего панкреатита. Болезнь редко встречается у детей. В мировой литературе прослеживается идея хирургического лечения наследственного панкреатита с целью купирования симптомов панкреатической обструкции, профилактики осложнений и замедления темпов атрофии поджелудочной железы.

Описание клинических случаев. В статье описана серия клинических случаев наследственного панкреатита с аутосомно-доминантным типом наследования — с мутацией в гене серин-протеазы 1 у 4 детей в одной семье. Продольная панкреатоеюностомия позволила достичь клинической ремиссии у всех пациентов.

Заключение. Эта серия случаев демонстрирует успешный вариант хирургического лечения наследственного панкреатита у детей.

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