Intestinal Lymphangiectasia

(Idiopathic hypoproteinemia)

Intestinal lymphangiectasia is a rare disease, which is characterized by the obstruction or the malformation of lymphatic vessels in the intestinal mucosa. It occurs most often in children and young adults. The symptoms consists of a malabsorption with edema and growth retardation. The diagnosis is made with a small bowel biopsy. Treatment is generally supportive.

Intestinal Lymphangiectasia is a Malabsorptionsstörung.

Intestinal lymphangiectasia is a rare disease, which is characterized by the obstruction or the malformation of lymphatic vessels in the intestinal mucosa. It occurs most often in children and young adults. The symptoms consists of a malabsorption with edema and growth retardation. The diagnosis is made with a small bowel biopsy. Treatment is generally supportive. Intestinal Lymphangiectasia is a Malabsorptionsstörung. A malformation of the lymphatic system can be congenital or acquired. Congenital cases manifest themselves usually in children (usually before 3 years of age diagnosed) and less frequently in adolescents or young adults. Men and women are equally affected. In acquired cases, the damage may be secondary to a retroperitoneal fibrosis, constrictive pericarditis, pancreatitis, in neoplasia and infiltrative diseases that block the lymphatic vessels occur. A reduced lymphatic drainage leads to elevated pressure and at a Lymphleck into the intestinal lumen. The reduction in Chylomikronen- and Lipoproteinabsorption leads to malabsorption of fat and protein. Because carbohydrates are not absorbed through the lymphatic system, their intake is not affected. Symptoms and complaints to the early manifestations of intestinal Lymphangiekatsien include strong and often asymmetric, peripheral edema, intermittent diarrhea, nausea, vomiting and abdominal pain. Some patients have a low or moderate steatorrhea. Chylous pleural effusions (chylothorax) and chylous ascites may occur. The growth is delayed when the disease occurs in the first decade of life. Endoscopic diagnosis small bowel biopsy Occasionally contrast enhanced lymphography The diagnosis of intestinal Lymphangiekatsien usually requires an endoscopic intestinal biopsy, which shows a marked dilation and ectasia of the lymphatic vessels in the mucosa and submucosa. Alternatively, a lymphangiography (administration of contrast agent through the lymphatic vessels of the foot) can show the pathological lymphatic vessels of the gut. Pathological laboratory values ??exist in lymphocytopenias and low levels of serum albumin, cholesterol, IgA, IgM, IgG, transferrin and ceruloplasmin. Bariumkontraststudien show thickened, bloody mucosal folds, similar to the rolls of coins. The d-xylose absorption is normal. Intestinal protein loss is shown using 51 Cr-labeled albumin. Therapy Supportive care Sometimes surgical resection or repair Pathological lymph vessels can not be corrected. A supportive therapy in intestinal Lymphangiekatsien consists in a diet low in fat (<30 g / day) and is rich in protein and contains the medium chain triglycerides. A replacement of calcium and fat-soluble vitamins is performed. Bowel resection or anastomosis of pathological lymph vessels to the venous flow path may be beneficial. Pleural effusions are drained.

Health Life Media Team

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