Exudative enteropathy
The term " exudative enteropathy " refers to a pathological condition characterized by loss of plasma proteins through the gastrointestinal tract. Usually, exudative enteropathy is accompanied by a violation of intestinal absorption( resulting in a marked decrease in the protein content of the blood), the appearance of edema, undigested fat in the feces. Unlike other malabsorption syndromes in exudative enteropathy, there may be no pronounced symptoms of small bowel disease. In rare cases, a child may lag behind in physical development.
Primary and secondary forms of exudative enteropathy are distinguished.
Primary forms are caused by the phenomenon of loss of lymph through the small intestine, which can be caused by abnormal lymphatic dilatation or generalized lesion of the lymphatic system. The loss of lymph can also be observed as a result of a violation of the outflow of lymph with a blockage of initially unchanged lymphatic vessels or obstructed venous outflow( eg, in heart diseases).
Secondary causes of development of exudative enteropathy, leading to disruption of the integrity of the intestinal mucosa, include a number of diseases from the gastrointestinal tract, from the kidneys, liver, lungs. In addition, in the development of exudative enteropathy may play the role of a violation of the immune system, the presence of allergic reactions and many other diseases.
Clinical manifestations of the disease are determined by the loss of plasma proteins, the severity of subsequent disorders, as well as age-related features. Isolation of a certain amount of protein through the intestine is the physiological norm. Reduction of the protein content in blood plasma occurs when the protein loss exceeds the rate of its synthesis in the body. Due to differences in the rate of synthesis of different protein fractions, the disruption of their ratio is as follows: the amount of albumins and ยก globulin in serum is reduced. Almost always, the level of fibrinogen remains within normal limits. The constant loss of lymphocytes leads to a stable absolute or relative decrease in their number, which is an important criterion for the diagnosis. Together with the protein, fats, trace elements and some vitamins are lost. The deficiency of these substances can change the clinical picture to a greater or lesser extent, and in some cases may be leading( for example, convulsions against the background of a marked decrease in the amount of calcium in the blood).
Primary intestinal lymphangiectasia( dilatation of the small intestine lymphatic vessels) is a special form of the syndrome that occurs with the loss of the plasma protein. This pathology was first described in 1966. It is assumed that it is inherited by an autosomal recessive type. However, the possibility of a dominant inheritance with a high frequency of occurrence and a different degree of severity of the pathological gene is not excluded.
In the clinical picture, massive asymmetric swelling that lasts for a long time, located mainly on the lower extremities, as well as in the body cavities( abdominal, pericardial cavity, pleural cavity), the decrease in the number of plasma proteins, the violation of the ratio of their fractions, the symptoms of violationfunction of the gastrointestinal tract, secondary immunodeficiency state. In some children, the disease begins to manifest itself at birth. Expansion of lymphatic vessels in the syndrome of Noonan is accompanied by a severe form of constantly emerging edema of the hands and feet, the nails on the legs turn yellow, become convex, their transverse striation appears. There are cases of a combination of expansion of the lymph vessels of the intestine with the syndrome of Di George, underdevelopment of tooth enamel.
Diagnosis for this disease is based on the detection of a reduced number of lymphocytes in the blood, changes in biochemical parameters of the blood. Diagnosis is possible when determining serum proteins in feces. Quantitatively, the loss of protein in the intestine can be determined by special technically complex research methods conducted in large hospitals. The study of the state of the lymphatic system by administering a contrast agent often reveals the underdevelopment of its peripheral parts and the apparent slowing of the movement of lymph( up to its complete absence in some vessels).In some cases, there may be a lack of lymph nodes near the aorta, as well as a blockage of the breast lymphatic duct with the entry of a contrast agent into the lumen of the intestine. A great diagnostic value is given to clarifying the state of the intestinal mucosa. When endoscopic examination of the intestine reveals the following picture: folds of the jejunum mucosa retained, pale pink or pink with a pronounced vascular pattern, sometimes spot pinpoint hemorrhages, an increase in the lymph nodes, and also a kind of proliferation of the mucosa in the form of numerous bulging. A characteristic feature of the disease under consideration is the detection of pieces of the intestinal mucosa taken during endoscopy, of enlarged lymphatic vessels during histological examination.
Therapeutic measures for exudative enteropathy are reduced to intravenous administration of protein preparations, a sharp restriction of animal fats in the diet, replacing them with vegetable oil. Use drugs containing fats that are easily broken down by pancreatic enzymes without the involvement of bile acids and absorbed into the venous system, helping to reduce lymphatic formation and facilitating its movement. Signs of the inflammatory process in the form of accelerating ESR, increasing the level of circulating immune complexes dictate the need for hormonal drugs, the treatment of which can lead to the elimination of clinical manifestations of exudative enteropathy. When expressed edematic syndrome, diuretics( diuretics) are necessary. In addition, it is necessary to use drugs of potassium, calcium, iron and vitamins.
The prognosis depends on the extent of the small intestine lesion, the severity of the pathology of the lymphatic system, the possibility of correcting the underlying disease in the secondary forms of the disease.