Diseases of the gastrointestinal tract in violation of the immune system

August 12, 2017 17:51 | Genetic Diseases

Acute diarrhea and malabsorption develop the majority of children with impaired immune system function.Diseases which are based on the primary failure of the immune system, have different clinical manifestations, but their common feature is the tendency to recurrent severe (with periodic exacerbations) bacterial, viral, and (or) fungal infections with chronic.Acquired immunodeficiency states in the majority of cases are hereditary defects of the immune system.

there are two of the immune system in the human body: cell and humoral.The first system is the specific cells that are directly involved in the immune response.The second system - humoral (lat humor -. «Liquid») - represented by antibodies (immunoglobulins) are proteins that represent different types of circulating in the blood and is also involved in immune reactions.

Common variable hypogammaglobulinemia (decrease in the number of proteins ¡ globulin in the blood, involved in immunity) - a group of immunodeficient states, the development o

f which is associated with impaired ability of B cells to transform into cells synthesizing immunoglobulins (antibodies are involved in the immuneresponse).In addition to recurrent or chronic microbial-inflammatory processes of ENT organs, lungs, skin and other purulent processes, the main manifestations of this pathology is a chronic disorder of the digestive.malabsorption syndrome is often caused by infection lyamblioznoy.In 30% of families of patients with common variable hypogammaglobulinemia relatives are autoimmune diseases.The total content ¡ globulin in the blood is not reduced to the extreme values, but still significantly deviates from the average age norm.Timing of the first manifestations of the disease vary considerably, although most occur in early childhood.Late forms of hypogammaglobulinemia common variable in adolescents and adults do not belong to the monogenic and to multifactorial diseases.The disease occurs with equal frequency in boys and girls.Inherited by this pathology augosomno recessive manner.

In primary agammaglobulinemia (no ¡ globulin in the blood), transmitted by X-linked sex-type, with 5-6 months of age, children have a variety of intestinal problems: identify giardiasis, disaccharidase deficiency, goiter thinintestine.Deficiency of antibodies found in 50% of patients with primary forms of immunodeficient states, very often - with common variable hypogammaglobulinemia.Antibody deficiency in older children is shown, as a rule, chronic pneumonia and malabsorption syndrome.There are several types of antibodies in humans.Immunodeficiency states can develop with a deficiency of any of the types of antibodies, but in most cases the determination of the content of the total number of antibodies, it remains within the normal range, and sometimes even a little improved.

When deficiency of cellular immune responses also observed violations of the gastrointestinal tract.

When syndrome Di George revealed , that the mucous membrane of the small intestine has a swollen appearance with flattened villi, and the level of immunoglobulins (antibodies) normal.Patients may develop unexplained malabsorption and watery diarrhea, they are susceptible to infection by bacteria, fungi, herpes simplex virus and cytomegalovirus.

When severe combined immune deficiency forms develop chronic watery diarrhea and malabsorption, which first appear in children aged 3 to 6 months.The villi of the small intestine mucosa altered or missing, giving the impression of "bald" with bowel endoscopy.

When Aldrich syndrome Viskotta- original function of the immune system is normal, but deteriorates with age.Bloody diarrhea - typical symptoms in early childhood.Most children with the disease die by the end of the first decade of life from recurring infections, bleeding or malignancy.

Lymphocytic dysgenesis (Nezelofa syndrome) is characterized by the absence or depressed cell-mediated immunity in normal or slightly reduced content of immunoglobulins in the blood plasma.The disease is rare, inherited in an autosomal recessive manner, it takes place in conjunction with malabsorption, constantly recurring infections and fungal lesions of various organs and systems.

Syndrome "naked» lymphocytes.It was found that in most cases of this disease the parents of sick children are to consanguineous marriage.This fact gives grounds to assume an autosomal recessive mode of inheritance, coupled with the inheritance is not detected the type of flooring.The disease first manifests itself between the ages of 3-4 months, diarrhea, delayed growth and development, repeated respiratory infections, bronchitis.Gradually deteriorating condition of children, developing malabsorption syndrome, they are extremely poor is added to the mass.Frequently observed pneumonia, fungal diseases are attached.In the terminal stages of the disease comes back development of the thymus gland.

Defects in humoral immune system trying to treat the introduction ¡ globulins or fresh frozen plasma.The most effective and promising method for the treatment of primary immunodeficiency disorders currently recognized early bone marrow transplantation.

heavy chain disease are tumor diseases with very diverse clinical manifestations.The disease mainly affects children and young people of both sexes to 30 years of age.There are two variants of it: abdominal (peritoneal) and pulmonary (the latter form is very rare).The clinical picture of abdominal form of the disease characterized by malabsorption syndrome with chronic diarrhea, the appearance in the feces undigested fat, malnutrition, edema, decrease the amount of calcium in the blood, hair loss, lack of menstruation in women.These symptoms are combined with episodes of fever and bouts of abdominal pain.In the study of pieces of the intestinal wall tissue, taken at endoscopy, determined characteristic of the disease changes.Since the abdominal form of the disease most often registered in countries where the population suffers from frequent intestinal infections, worm infestations and unbalanced diet, suggests the role of long-term stimulation of foreign proteins.