Zollinger–Ellison syndrome within the framework of multiple neoplasia syndrome type 1: Description of the clinical case
https://doi.org/10.62751/2713-0177-2024-5-3-11
Abstract
Multiple endocrine neoplasia syndrome type 1 (MEN-1, Vermeer syndrome) is an autosomal dominant hereditary syndrome characterized by mutations in the MEN1 gene. The manifestation of the syndrome is associated with hyperplasia and tumor transformation of several endocrine glands: tumors of the parathyroid glands, pituitary gland, pancreas, adrenal glands. Within the framework of MEN-1, Zollinger–Ellison syndrome may develop – a clinical syndrome caused by hypergastrinemia, the source of which is gastrin-producing tumors of the pancreas and other localizations, resulting in hypersecretion of hydrochloric acid. This leads to the appearance of peptic ulcers of the duodenum and stomach, refractory to treatment and complicated by bleeding and stenosis. The article describes a clinical case of a young patient with the classic triad of components of MEN-1 syndrome, including multiple gastrinomas with frequent recurrences of gastric ulcer and duodenal ulcer.
About the Authors
A. A. Akhmedova
Pirogov Russian National Research Medical University
Russian Federation
Russian Federation
Aminat A. Akmedova – Clinical Resident
Moscow
M. A. Rasulov
Pirogov Russian National Research Medical University
Russian Federation
Russian Federation
Magomed A. Rasulov – Clinical Resident
Moscow
F. O. Ushanova
Pirogov Russian National Research Medical University
Russian Federation
Russian Federation
Fatima O. Ushanova – Can.Sci. (Med.), Associate Professor
Moscow
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Review
For citations:
Akhmedova A.A., Rasulov M.A., Ushanova F.O. Zollinger–Ellison syndrome within the framework of multiple neoplasia syndrome type 1: Description of the clinical case. FOCUS. Endocrinology. 2024;5(3):84-91. (In Russ.) https://doi.org/10.62751/2713-0177-2024-5-3-11
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