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Surgical treatment of hereditary pancreatitis in four children in one and the same family
- Authors: Кislenko A.A.1,2, Razumovskiy A.Y.1, Kholostova V.V.1,2
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Affiliations:
- The Russian National Research Medical University named after N.I. Pirogov
- Filatov N.F. Children's City Hospital
- Issue: Vol 28, No 1 (2024)
- Pages: 105-113
- Section: CASE REPORT
- Submitted: 23.08.2023
- Accepted: 26.10.2023
- Published: 03.04.2024
- URL: https://jps-nmp.ru/jour/article/view/726
- DOI: https://doi.org/10.17816/ps726
- ID: 726
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Abstract
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.
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About the authors
Alina A. Кislenko
The Russian National Research Medical University named after N.I. Pirogov; Filatov N.F. Children's City Hospital
Author for correspondence.
Email: kislenkolina@mail.ru
ORCID iD: 0000-0002-5530-4410
MD
Россия, Moscow; MoscowAlexandr Yu. Razumovskiy
The Russian National Research Medical University named after N.I. Pirogov
Email: 1595105@mail.ru
ORCID iD: 0000-0002-9497-4070
SPIN-code: 3600-4701
MD, Dr. Sci. (Medicine), Professor
Россия, MoscowVictoriya V. Kholostova
The Russian National Research Medical University named after N.I. Pirogov; Filatov N.F. Children's City Hospital
Email: vkholostova@yandex.ru
ORCID iD: 0000-0002-3463-9799
MD, Dr. Sci. (Medicine)
Россия, Moscow; MoscowReferences
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