From Big Medical Encyclopedia


of NAYa (enteropathia exsudativa) — heterogeneous group of diseases and morbid conditions, the general sign to-rykh is the hyperpermeability of an intestinal wall which is followed by a syndrome of disturbance of intestinal absorption, a hypoproteinemia, hypostases, dystrophy, a delay of physical development.

Allocation of insignificant amounts of blood protein in a gleam of intestines through its wall is revealed by I. P. Razenkov in 1949, to-ry considered it fiziol. process. The possibility of excretion of proteins of plasma intestines is for the first time established by G. Citrin in 1957 and confirmed by R. Gordon in 1959. In the next years the hyperpermeability of an intestinal wall for plasmatic proteins is found in premature children, and also at a number of diseases and patol. states.

In a crust, time allocate primary and secondary forms E. aa. Refer congenital (hereditary) anomalies to primary forms limf, vessels of intestines — a family hypoproteinemia with E. aa., intersticial lymphangiectasia, or lymphangioma. Secondary forms are caused by damage of intestines at various diseases and syndromes: Gee's diseases (see), a gastroenteritis (see), a mucoviscidosis (see), diseases of Girshsprunga (see Megacolon), diseases Krone (see Krone a disease), Whipple's diseases (see. An intestinal lipodystrophy), Menetriye's diseases (see Gastritis), intolerance of protein of cow's milk (see. Food allergy), nonspecific ulcer colitis (see. Ulcer nonspecific colitis), agammaglobulinemias and a hypogammaglobulinemia, a lymphosarcoma (see Sarcoma), diseases of a liver.

At E. aa. permeability of an intestinal wall increases so that not only low-molecular albumine, but also large molecules of globulins, including immunoglobulins in a significant amount get to intestinal contents and is removed with a stake. In the absence of a diarrhea the ground mass of protein coming from a blood plasma to a gleam of intestines is split to amino acids, to-rye are adsorbed and get to system of a portal vein. Loss of plasma proteins at E. aa. it is not filled with their resynthesis in a liver that leads to development of a considerable hypoproteinemia, and deficit of albumine is especially expressed. Along with deficit of plasma proteins the tsiyemiya, a hyper aminoaciduria, hypochromia anemia are found Hypaque of l. The hypoproteinemia is the main reason for an edematous syndrome, and also a giioim-munoglobulinemiya.

The main wedge, symptoms E. aa. are: a delay of physical

development of the child, hypostases, the syndrome of the broken intestinal absorption which is shown diarrhea, a weight loss (see Malabsorption a syndrome). In some cases hypostases are the only display of a disease. They can be limited (in extremities, lower parts of an abdominal wall) or widespread on type of a nephrotic syndrome (see) without signs of damage of kidneys and cardiovascular system. Decrease in content in blood of all classes of immunoglobulins causes a long current at such children inf. diseases. Disturbances of absorption of fats in intestines cause development of a steatorrhea (see).

Rentgenbl. changes are caused by hypostasis of an intestinal wall. Passage of a contrast agent timely. At a lymphangiectasia the intestinal wall can have a cone-shaped thickening of circular (ker-kringovy) folds in the form of a garland. In more expressed cases it is found pseudo-polyposes.

The exudative enteropathy can proceed sharply, tranzitorno and chronically. Development hron. forms perhaps from the first days of life of the child or later, at the same time it is frequent against the background of E. aa. the hypocalcemia and attacks of tetanic spasms are observed.

For the purpose of diagnosis use qualitative and quantitative methods of definition of proteins in blood, urine and Calais. In a filtrate a calla apply reaction to qualitative test of protein with trichloroacetic to - that. Quantitative definition of proteins to blood and Calais and their identification is carried out by means of an immunoelectrophoresis (see), researches of content of proteins, marked by isotopes. Have great diagnostic value also rentgenol. methods and morfol. a research of the material received at endoscopy of intestines.

At the expressed hypoproteinemia parenteral administration of proteinaceous drugs is shown (albumine, plasma, gamma-globulin, polyglobulin, etc.). At a steatorrhea appoint a diet poor in fat, and it is preferable to enter fat in the form of unsaturated fat to - t (vegetable oils). At depression of function of bark of adrenal glands administration of glucocorticoid hormones is recommended. Along with it use anabolic steroid hormones, drugs of potassium, calcium, a complex of vitamins, enzymes, Mexaformum, bifidumbacterium, a diet with restriction of lactose (fermented milk products, cottage cheese). At the expressed edematous syndrome diuretics are shown (lasixum, antagonists of Aldosteronum).

The forecast is defined by time of diagnosis, degree and prevalence of damage of intestines. At the earliest establishment of the diagnosis and adequate treatment in case of limited damage of intestines the forecast favorable.

To the purposes of prevention of primary forms E. aa. medicogenetic consultation serves (see. Medicogenetic consultation), and at secondary E. aa. — the complex to lay down. the actions preventing damage of intestines.

Bibliography: Diseases of digestive organs at children * under the editorship of A. V. Mazurin, page 507, M ** 1984; Livshits E, G. and Me of d-N of e T. A. Sindr of malabsorption in pediatric clinic, page 43, Riga, 1979;

Mazurin A. In, etc. the Klinikogenealogichesky research of a peptic ulcer at children, Vopr. okhr. mat. also it is put., t. 21, No. 2, page 22, 1977; R and z e N -

to about in I. P. About release of protein with digestive juices in a digestive tract as about new aspect of activity of digestive tract, in book: Probl. owls. fiziol., biochemical and pharmakol., under the editorship of L. A. Orbeli, etc., t. 1, page 13, M., 1949; Nelson textbook of pediatrics, ed. by R. E. Behrman a. V. C. Vaughan, Philadelphia, 1983; Waldmann T. A. Protein-losing enteropathy, Gastroenterology, v. 50, p. 422, 1966;

Walker-Smith J. A. Disease of the small intestine in childhood, N., Y., 1975.

V. A. Tabolin.