Surgical treatment of hereditary pancreatitis in four children in one and the same family

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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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作者简介

Alina Кislenko

The Russian National Research Medical University named after N.I. Pirogov; Filatov N.F. Children's City Hospital

编辑信件的主要联系方式.
Email: kislenkolina@mail.ru
ORCID iD: 0000-0002-5530-4410

MD

俄罗斯联邦, Moscow; Moscow

Alexandr Razumovskiy

The Russian National Research Medical University named after N.I. Pirogov

Email: 1595105@mail.ru
ORCID iD: 0000-0002-9497-4070
SPIN 代码: 3600-4701

MD, Dr. Sci. (Medicine), Professor

俄罗斯联邦, Moscow

Victoriya 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)

俄罗斯联邦, Moscow; Moscow

参考

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2. Fig. 1. Magnetic resonance imaging of a patient with hereditary pancreatitis: a — enlarged pancreatic head (arrow); b — dilated Virsung duct (arrow).

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3. Fig. 2. Outer view of the pancreas: it is reduced in size, with increased density.

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4. Fig. 3. Virsung duct opening.

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5. Fig. 4. Virsung duct cutting along the gland.

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6. Fig. 5. Virsung duct is cut along the entire length.

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7. Fig. 6. A posterior part of anastomosis is formed.

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8. Fig. 7. An anterior part of anastomosis is formed.

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9. Fig. 8. Magnetic resonance imaging of the abdominal cavity organs: dilated Virsung duct because of calculi.

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10. Fig. 9. Magnetic resonance imaging of the abdominal cavity organs: dilated and tortuous Virsung duct.

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11. Fig. 10. Magnetic resonance imaging of the abdominal cavity organs. Virsung duct is unevenly dilated, pancreatic gland is edematous.

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