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"Child`s Health" 2 (45) 2013

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Autoimmune enteropathy in children: review of literature and own supervision

Authors: Lymarenko M.P., *Logvynenko N.G., Mayorenko O.P., Donetsk National Medical University named after M. Gorky, Ukraine, *Municipal specialized house of child, Donetsk

Categories: Pediatrics/Neonatology

Sections: Specialist manual

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Summary

Autoimmune enteropathy behaves to the rare diseases. Basis of illness is persistent diarrhea, attended with the products of autoantibodies - sign of active autoimmune Т-cell inflammation.


Keywords

autoimmune enteropathy, nephrotic syndrome, children.

Presently pathogeny of autoimmune enteropathy continues to be studied. Moes N. et al. report about opening of molecular background for this disease - mutations in the gene of Foxp3 on X-chromosome, supervisory the functions of regulator Т-cell. This opening gives new possibilities of diagnostics and treatment of illness to the clinicians.

Autoimmune enteropathy has the early beginning, characterized by a making progress flow. Diarrhea has persistent character. A chair is pappy or liquid, watery, quite often contains blood and mucus. A disease abenteric autoimmune displays can accompany: arthritis, dermatitis, thrombocytopenia, anaemia, pericarditis, hepatitis, glomerulonephritis, diabetes mellitus.

A disease is characterized by atrophy of villi and massive mononuclear infiltration of own plate of mucous membrane of thin intestine under histomorphological research. These changes are most shown in the mucous membrane of thin intestine, but can be revealed and in other departments of digestive tract - stomach or colon. The vast defeat of gastrointestinal tract has the most heavy displays and bad prognosis.

A main diagnostic sign of autoimmune enteropathy is circulatory autoantibodies mainly class of IgG to the intestinal epithelium.

Autoimmune enteropathy can have a different degree of weight from easy to very heavy, to threatening life. The criteria of bad prognosis are the early beginning of disease (in the first months of life), diarrhea above 150 ml/kg in twenty-four hours, expressed destruction of crypts, associated defeats of colon and overhead departments of gastrointestinal tract, involving of kidneys and high title of circulatory antiintestinal autoantibodies.

Basis of treatment of autoimmune enteropathy is made by immunosuppressors. Dietary limitations, parenterally feed and antisecretory agents, are additional funds. Patients which do not answer on immunodepressive therapy can be candidates on transplantation of bone marrow.

In the article an own supervision over of autoimmune enteropathy is brought for a baby.

A child is born from a V pregnancy, V labor, by mass 2950 g. From birth a girl added not enough in-bulk, diarrhea was marked. Supposition is outspoken about the syndrome malabsorption. On this occasion a child passed a deep inspection, which infectious (antenatal and purchased) pathology, mucoviscidosis, is eliminated as a result of. Ultrasonic research of all vital was executed, their function is studied.

In connection with the assumed lactase deficiency fixing for child of delactosed mixtures did not have a clinical and laboratory effect, diarrhea was still saved, an increase was absent in-bulk body. Permanent treatment, a few courses of antibacterial and antifungal therapy, was conducted a child enzymes.

Worsening of the state of girl was marked in age 5 months, when a languor grew, vomiting joined, persistent diarrhea, metabolic and microcirculatory violations, dermatitis of inguinal area was saved.

For a child a nephrotic syndrome was formed, complicated by sharp kidney insufficiency. A diuresis began to go down, edemata appeared up to an anasarca, uremia, high proteinuria, hypoproteinemia, hypercholesteremia. Intensive therapy was conducted with including of saluretics, osmodiuretics, transfusion of albuminous preparations, infusion of dopaminum, introduction of corticosteroids. The state of child progressively got worse due to the expressed oedematous syndrome, intoxication, microcirculatory disorders, making progress hypotrophy, resulting in death of child in age 6,5 months.

Results of histomorphological research. Wall of thin intestine: the expressed dystrofic changes of enterocytes up to atrophy of hair with shortening of crypts, hearths of colic metaplasia, mildly expressed polymorphocellular infiltration, fibrosis of submucous layer. Wall of colon: the expressed atrophy of mucous membrane, lymphohistiocytic infiltration of wall up to a muscular layer, sclerosis of armpit layer.

At pathoanatomical research the considerable atrophy changes of mucous membrane are educed by thin and thick intestines.

These hospital charts and results of pathoanatomical conclusion allowed to diagnose for a child autoimmune enteropathy. In behalf on this disease testified: beginning of diarrhea for a baby to 3th monthly age, long diarrhea during all life of child, 3 negative sowing of excrement on the causative agents of enteroideas, combination with a nephrotic syndrome, dermatitis, considerable atrophy changes of mucous membrane thin and thick intestines, absence of effect from the special diets, parenterally feed, treatment antibiotics.

Thus, autoimmune enteropathy is a rare disease. But about  him it is necessary to remember to the paediatricians, conducting differential diagnostics of syndrome malabsorption in children.


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