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ORIGIN AND ETIOPATHOGENESIS OF BEAN SYNDROME

Abstract

Bean Syndrome - this is diffuse mucocutaneous angiomatosis. The disease occurs when there is a mutation in the TEK gene, which is located at the 9p21 locus. The pathology is manifested by multiple malformations of the veins – blue "rubber" formations located in the skin, mucous membranes of the gastrointestinal tract, and some internal organs. To diagnose Bean syndrome, a hemogram, coagulogram, endoscopic examination of the gastrointestinal tract, and other imaging methods are performed, taking into account clinical manifestations. Combined treatment: conservative therapy to suppress cell proliferation and correct signs of anemia, surgical or minimally invasive intervention to excise abnormal venous vessels.

Keywords

mucocutaneous angiomatosis, abnormal venous vessel.

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