exudative enteropathy

August 12, 2017 17:51 | Genetic Diseases

term « exudative enteropathy » represent a pathological condition characterized by the loss of plasma proteins through the gastrointestinal tract.Typically, exudative enteropathy accompanied by a violation of intestinal absorption (resulting in a marked reduction of blood proteins), the appearance of edema, undigested fat in the stool.Unlike other malabsorption syndromes with exudative enteropathy may not be pronounced symptoms of small bowel lesions.In rare cases, the child may lag behind in physical development.

There are primary and secondary forms of exudative enteropathy.

Primary forms are due to the phenomenon of loss of lymph through the small intestine, which can be caused by abnormal expansion of lymphatic vessels or generalized lymph system.Loss of lymph can also occur as a result of violation of lymphatic drainage to the blockade did not initially modified lymphatic vessels or the difficulty of venous outflow (eg heart diseases).

Secondary causes of exudative enteropathy, leading to disru

ption of the integrity of the intestinal mucosa, include a number of diseases of the gastro-intestinal tract and in the kidneys, liver and lungs.Furthermore, in the development of exudative enteropathy may act as disorders of the immune system, allergic reactions and presence of many other diseases.

Clinical manifestations of the disease are determined by the loss of plasma proteins, the severity of subsequent violations, 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 of blood plasma occurs when protein loss exceeds the rate of its synthesis in the body.Due to differences in the rate of synthesis of the different fractions of proteins violation of their relationship has the following character: reduced amount of albumin and globulins ¡ serum.fibrinogen level is almost always remains within the normal range.Permanent loss of lymphocytes leads to permanent absolute or relative decrease in their number, which is an important criterion for diagnosis.Together with protein lost fats, vitamins and some minerals.Deficiency of these substances could alter the clinical picture in the direction of greater or lesser severity, and in some cases can be the master (e.g., seizures on the background of pronounced reduction amount of calcium in the blood).

Primary intestinal lymphangiectasia (enlargement of the lymph vessels of the small intestine) - a special form of the syndrome, flowing with the loss of blood plasma protein.This pathology was first described in 1966. It is assumed that it is inherited in an autosomal recessive manner.However, there is a possibility and the dominant inheritance with high frequency proyavlyaemosti and varying degrees of severity of the abnormal gene.

The clinical picture is on the foreground massive asymmetrical swelling, lingering for a long time, which are located mainly in the lower extremities, as well as in body cavities (peritoneal, pericardial, pleural cavity), reduction in the number of plasma proteins, violation of the ratio of their factions, symptomsdysfunction of the gastrointestinal tract, secondary immunodeficiency.In some children, the disease begins to manifest at birth.The expansion of lymphatic vessels with Noonan syndrome is accompanied by a severe form of constantly appearing swelling of hands and feet, toenails turn yellow, become convex, there is their transverse striations.There are cases of a combination of the expansion of lymphatic vessels bowel syndrome Di George, dental enamel hypoplasia.

diagnosis in this disease is based on the discovery of a reduced number of lymphocytes in the blood, changes in blood biochemical parameters.Diagnosis is possible with determination of blood serum proteins in the stool.Quantitatively, the loss of protein in the gut can be determined with special technically complex methods of research conducted in major hospitals.Investigation of the state of the lymphatic system by introducing a contrast agent often reveals hypoplasia of its peripheral regions and the apparent slowing lymph movement (up to its complete absence in some vessels).In some cases the absence of lymph nodes near the aorta, as well as the blockage of the thoracic duct with the arrival of the contrast agent into the intestinal lumen.Great importance is attached to the diagnostic clarification of the status of the intestinal mucosa.When bowel endoscopy study revealed the following picture: mucosal folds of the jejunum saved, pale pink or pink with pronounced vascular pattern are sometimes prescribed petechial hemorrhages, enlarged lymph nodes, there is also a kind of proliferation of the mucous membrane in the form of numerous bulging.Characteristic of the disease in question is the detection of histologically pieces of the intestinal mucosa, taken during endoscopy, dilated lymphatic vessels.

Therapeutic measures during exudative enteropathy reduced to intravenous protein drugs, a sharp restriction of animal fats in the diet and replacing them with vegetable oil.Use formulations containing fats which are easily cleaved by enzymes without pancreatic and bile acids are absorbed into the venous system, helping to reduce the formation of lymph and facilitating its movement.Signs of inflammation in the form of acceleration of ESR, increase the level of circulating immune complexes dictate the need for hormonal therapy, the treatment of which can lead to the elimination of the clinical manifestations of exudative enteropathy.In marked hydropic syndrome need diuretics (diuretics).Also required is the use of drugs potassium, calcium, iron and vitamins.

Prognosis depends on the extent of the defeat of the small intestine, the severity of the pathology of the lymphatic system, the possibility of correction of the underlying disease in secondary forms of the disease.