Clinical and genetic features of multiple endocrine neoplasia type 1 syndrome and principles of its treatment


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Abstract

The article is dedicated to modern concepts of epidemiology, pathogenesis and clinical presentation of multiple endocrine neoplasia syndrome type 1 (MEN 1). MEN-1 is a rare congenital autosomal dominant syndrome caused by mutation of the MEN1 gene, localized on chromosome 11q13. Combination of tumors of parathyroid glands, pituitary and pancreas is typical for MEN-1. The algorithm for diagnosis of MEN-1syndrome is considered, the possibility of its treatment, including the use of somatostatin analogs is discussed. It is emphasized that in patients with various manifestations of MEN-1 syndrome due to accurate diagnosis and modern methods of surgical and medical treatment prognosis is significantly improved and life expectancy is increased in recent years.

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