Terapevticheskii arkhivTerapevticheskii arkhiv0040-36602309-5342LLC Obyedinennaya Redaktsiya3276710.26442/terarkh201890396-98Research ArticlePrimary lymphangiectasia of the gastrointestinal tractEsedovE M-AbasovaA Salbinadgma@mail.ruAkhmedovaF D-MusaevaL N-FSBEI HE "Dagestan State Medical University" Ministry of Health15032018903969811042020Copyright © 2018, Consilium Medicum2018Intestinal lymphangiectasia is a very rare pathology, characterized by the presence of enlarged lymphatic vessels in all layers of the intestinal wall and in the mesentery. As a result, "lymphatic lakes" are formed, through which lymph exudates into the lumen of the intestine. The main manifestation is hypoproteinemic edema. Diagnosis of the disease is based on laboratory-instrumental methods of investigation, including by determining in the stool α-1 antitrypsin as a screening test. Treatment includes: a diet rich in calcium, trace elements, be sure to use medicinal foods enriched with medium chain triglycerides, substitution and symptomatic therapy.intestinal lymphangiectasiahypoproteinemic edemamedium chain triglyceridesclinical observationинтестинальная лимфангиэктазиягипопротеинемические отекисреднецепочечные триглицеридыклиническое наблюдение[Wen J, Tang Q, Wu J, Wang Y, Cai W. Primary intestinal lymphangiectasia: four case reports and a review of the literature. Dig Dis Sci. 2010; 55(12):3466-3472.][Waldmann T.A, Steinfeld J.L, Dutcher T.F, Davidson J.D, Gordon R.S. The role of the gastrointestinal system in “idiopathic hypoproteinemia”. Gastroenterology. 1961;41:197-207.][Полякова С.И. Первичная лимфангиэктазия тонкой кишки. Клиническая медицина. 2005; 83(9):62-65.][Парфенов А.И., Крумс Л.М. Энтеропатия с потерей белка. Терапевтический архив. 2017; 89 (2): 4-9. doi: 10.17116/terarkh20178924-9.][Shrikiran Aroor, Suneel Mundkur, Shravan Kanaparthi, Sandeep Kumar Waldmann’s Disease (Primary Intestinal Lymphangiectasia) with Atrial Septal Defect. J Clin Diagn Res. 2017; 11 (4). SD03-SD04. doi: 10.7860/JCDR/2017/24989.9742][Vignes S, Bellanger J. Primary intestinal lymphangiectasia (Waldmann’s disease). Orphanet Journal of Rare Diseases. 2008; 3: 5.][Proujansky R. Paediatric gastrointestinal disease: Pathophysiology, diagnosis and management. 4th ed. Ontario: BC Decker. Protein losing enteropathy. In: Walker, Goulet, Kleinman, Sherman, Shneider, Sanderson, editors; 2004: 194-199.][Marjet J.B, Koert M.D, Merit M.T. Protein - losing enteropathy in children. Eur J Pediatr. 2010; 169: 1179-85.][Soon J.L, Hyun J.S, Boo S-J, Na S-Y, Heung U.K, Chang L.H. Primary intestinal lymphangiectasia with generalized warts. World Journal of Gastroenterology. 2015; 21 (27): 8467-8472.]