Hypoplasia connecting tissue in children. Connective dysplasia: main clinical manifestations, complex therapy, prevention. Causes of pathology

Connecting tissue - the base of the whole organism. In disruption of its structure, pathological changes arise in all organs. Therefore, the dysplasia of the connective tissue in children is difficult, it can be masked under any other pathology. This makes it difficult to diagnose.

The essence of pathology

Connectant dysplasia in children is not independent pathology - this is a complex of congenital disorders arising in the intrauterine period. The connecting fabric is practically in all organs of the human body. It has the greatest value in building a skeleton, ensuring a motor function. Connecting fabric is mainly made of collagen protein.

Dysplasia occurs if a genetic failure occurs in the formation of collagen fibers. As a result, the connecting tissue loses its elasticity and extensibility.

Interesting!
The incidence rate of the dysplasia varies within 6-9% among all newborn children.

On ICD 10 illness has the designation M35.7.

The reasons

DST in a child is a multifactorous disease. This means that for its occurrence, several reasons should be affected at the same time. The main predisposing factors include:

Bad mother habits during pregnancy;
Poor nutrition;
Unfavorable ecological situation;
Severe flow of pregnant woman's gestosis;
Permanent stress I. physical exercise;
Some infectious diseases of a woman during pregnancy.

The hereditary factor is of great importance.

Manifestations

Symptoms of connective tissue dysplasia in children are very numerous, since this fabric is present in all organs.

Nervous system:

Vegeth-vascular dystonia;
Problems with the development of speech;
Headaches;
Increased sweating;
Urinary incontinence.

The cardiovascular system:

Congenital heart defects;
Aortic aneurysm;
Underdevelopment of vessels;
Lability arterial pressure;
Heart failure.

Respiratory system:

Constant shortness of breath;
Bronchiectatic disease;
Pleurisy;
Underdevelopment of the lungs.

Musculoskeletal system:

Rachiocampsis;
Flatfoot;
Thoracic deformation;
Congenital and usual dislocations;
Increased flexibility of the joints;
Frequent fractures.

Gilitary system:

Renelization;
Anomalies of a cup-making kidney system;
Underdevelopment of ureters;
Urinary incontinence;
Disorders in the development of sex glands;
Violation of the development of uterus and ovaries.

Digestive tract:

Intestinal dysfunction;
Underdevelopment of intestines, esophagus.

Feeling authorities:

Strabismus;
Myopia;
Leakage lens;
Astigmatism;
Reduced hearing.

External manifestations:

High growth;
Long subtle limbs;
Thinness;
Dry and pale leather;
Large asymmetric oars;
Stretching on the skin.

Muscle tissue dysplasia in children leads to the development of miasthenia - muscle weakness, in which the child loses the opportunity to move.

Symptoms may occur separately, only from one group, or from several groups simultaneously. Depending on this, two types of connective tissue dysplasia are distinguished:

Differentiated. There is a defeat of any one group of organs. These diseases include marfan syndrome, sclerodermia, fibergation;
Undifferentiated. Signs of dysplasia cannot be attributed to any one group.

The dysplasia of the musculoskeletal system in children is heavier. This form of the disease leads to a violation of the motor function, a change in the operation of the internal organs.

The dysplasia of the hip joint is manifested by the shortening of one leg of the baby, the asymmetricity of the berium folds. Child can not dilute bending legs to the sides. Children begin to walk late, the gait resembles duck. The child quickly gets tired when walking, it can cause pain.

In the knee dysplasia, the deformation of the knee cup is developing. The child complains of pain when walking, fast fatigue. Often there are dislocations and podlings of the joint. During the defeat of the ankle joint, there is a circuit and flatfoot. The child often turns his legs when walking.

Diagnostics

The process of diagnosing dysplasia in children is quite complex. For diagnosis, various methods are used:

CT and MRI;
Electromyography;
Radiography.

These techniques make it possible to estimate the degree of changes in the connective tissue. To confirm the diagnosis, genetic studies are conducted, revealing disorders in collagen formation.

Treatment

For the treatment of connective tissue dysplasia in children, an integrated approach is used. The possibilities of even modern medicine do not allow to restore genetic disorders, so all treatment is symptomatic.

Diet

Proper nutrition is of great importance in the treatment of connective tissue dysplasia. The purpose of the diet is to stimulate the production of its own collagen, to replenish the body's need in vitamins and minerals. In fact, it meets the principles of healthy nutrition. The diet should include:

Proteins - meat and fish;
Carbohydrates - cereals, bread, pasta;
Vitamins - fruits, vegetables, greens;
Minerals - eggs, dairy products.

Medicinal products

Medical therapy is aimed at eliminating the symptoms of the disease, stimulation of the synthesis of normal collagen. It is known that magnesium deficiency leads to a decrease in collagen production in children. Therefore, the drugs of magnesium and calcium can be prescribed for children during dysplasia of the connective tissue - Magneroth, calchecin.

To replenish the components of the cartilage tissue, preparations containing glucosamine and chondroitin are shown. These include Arters, Teraflex, Pallex.

Ascorbic acid, vitamins A and E. also contribute to the stimulation of own collagen.

LFK

The obligatory stage of treatment of dysplasia in children is gymnastics. Intensive physical exertion should be limited, since the child has an increased risk of fracture and dislocation. The course of therapeutic gymnastics is made up by a specialist, as the child grows and the restoration of tissues adjust it.

Massage

To restore the elasticity of tissues, the strengthening of the muscular-ligament apparatus is carried out regular massage of the limbs. Used both ordinary classic massage and various non-traditional techniques. It is important that the massage is daily.

Physiotherapy

Physiostaries are carried out with the age of child:

Electrophoresis;
Application of paraffin and ozokerite;
Magnetotherapy;
Dyadynamic currents.

The greatest effect is noted under the coursework.

Effects

Under untreated and inadequate treatment, irreversible disorders in the limbs may be formed. Joint dysplasia leads to the development of contractures, heavy arthritis and arthrosis. In this case, treatment can only be surgical.

The dysplasia of the connective tissue in a child is a complex and poorly studied disease. It requires as much as possible early diagnosis and comprehensive treatment.

The connective tissue dysplasia is the pathology of its development due to genetic mutations. The consequence of the disease is a malfunction in homeostasis at the intestinal level, as well as the whole organism.

A differentiated form, characterized by a certain inheritance option, which has an explicit clinical picture, and often established and long-studied biochemical or gene anomalies. This type of DST has the name of collagenopathy in medicine, as belonging to the hereditary abnormalities of collagen.

The undifferentiated form is diagnosed only when none of the symptoms of the disease cannot be attributed to the differentiated form of DST. Such pathology is especially common.

The reasons

DST is a hereditary disease based on the mutations of genes responsible for the formation of fibers. Such processes are the most diverse, various genes can be involved, which leads to the abnormal formation of collagen and elastin chains, as a result, the structures generated by them are not able to withstand the necessary mechanical loads.

Symptoms

Such innate pathology has a scattered symptomatic picture with a large number of features.

External symptoms:

sweet joints,
increased skin tensile,
deformation of the spine, the presence of scoliosis or kyphosis, violation of vision,
deformation of the sternum, translucent thin skin with a venous mesh on the skin,
asymmetry of the blades,
problems with posture,
fast appearance of bruises,
weak muscles of the peritoneum,
the presence of muscle hypotension,
a curvature or asymmetric nasal partition,
soft skin,
the presence of intervertebral hernia,
problems with increasing teeth.

Internal signs are formed over the years:

the presence of dyskinesia of biliary tract,
omission of internal organs
development of reflux disease,
noise in the heart
frequent constipation,
the presence of hemorrhagic syndrome, the signs of which are nasal bleeding, a tendency to the formation of a hematoma with a slight injury,
mitral valve prolapse,
the presence of the hernia of Schimorl,
varicose veins on the legs,
development of youth osteoporosis,
microtramatic "transit" arthritis, vegetative dystonia,
predisposition to frequent loss of consciousness,
development of vertebobasillary deficiency against the background of problems with the cervical spine,
hyperoportability.

Diagnosis of connective tissue dysplasia

In order to diagnose this disease, a number of studies of the clinical and genealogical plan are carried out.

However, in addition, specialists in order to clarify the diagnosis are used by the following diagnostic methods.

1. Analysis of patient complaints. Preferably, children complain about a number of symptoms, ranging from bloating and dysbacteriosis, and ending with the deviations observed in the work of the respiratory organs, which is associated with the weakness of the bronchi and Alveol. In this case, the implementation of accurate diagnostics contributes to the observation of certain cosmetic defects, as well as violations of the joint joints.

2. In order to conduct diagnostics, the doctor measures the length of all parts of the patient's body. The characteristic "test of the wrist" is used when the child with a thumb or a little finger is capable of grabbing it entirely.

3. The movable joint function is estimated, for which the Beiton criteria apply. Typically, patients have their hypermobility.

4. A daily analysis of urine is taken, in which the definition of oxyproline and glycosaminglikans, proving the fact of the destruction of collagen, has an important value.

In general, to diagnose this ailment for an experienced specialist is not difficult, often enough visual inspection to understand what its problem is.

Complications

Children often have complications in the form of problems with the work of the heart muscle, visual impairment, problems with joints.

Patients with a diagnosis belong to the category of psychological risk. In particular, this is not noticeable in childhood, but with age. Suffering DSTs are prone to low self-esteem, they differ in low levels of claims.

The feeling of increased anxiety and the depressive state leads to a high vanity of patients. Cosmetic features in appearance make patients with dst unsure in themselves, the misinterpretative, constantly dissatisfied with what is happening in their lives who reproach themselves in any mishai.

Treatment

What can you do

When symptoms are found and in the case of a child's complaints on bad state of health, Fast fatigue, you need to consult a doctor. Such a diagnosis as DST can be delivered under the clinic.

What does the doctor do

Therapy for the diagnosis of DST is carried out comprehensively and includes:

Medical treatment based on use medicinesthat will help to activate the formation of collagen.
Non-projected techniques to which rendering psychological assistance, individualization of the day mode, exercises included in therapeutic physical education, massage, physiotherapy procedures, needleflexotherapy, selection of a certain diet.

Prevention

Preventive measures for DST does not exist in principle, since it is a congenital disease due to mutations at the genetic level. But the right way of life, healthy balanced nutrition and doctors recommendations can facilitate the state of the child with such a diagnosis, enhancing the quality of his life.

- a group of polymorphic clinical terms of pathological conditions due to hereditary or congenital defects of collagen synthesis and accompanied by violation of the functioning of internal organs and the musculoskeletal system. The most commonly connective tissue dysplasia is manifested by a change in body proportions, bone deformations, hypermobility of joints, habitual dislocation, hyperalastic leather, valve heart defects, fragility of vessels, muscle weakness. Diagnostics is based on phenotypic signs, biochemical indicators, biopsy data. Treatment of connective tissue dysplasia includes LFC, massage, diet, drug therapy.

General

Connectual dysplasia is a concept that combines various diseases caused by hereditary generalized collagenopathy and manifested by a decrease in the strength of the connective tissue of all organism systems. The population frequency of the connective tissue dysplasia is 7-8%, but it is assumed that its individual features and small undifferentiated forms may occur in 60-70% of the population. The connective tissue dysplasia falls into the field of view of clinicians working in different medical regions - pediatrics, traumatology and orthopedics, rheumatology, cardiology, ophthalmology, gastroenterology, immunology, pulmonology, urology, etc.

The reasons

The development of a connective tissue dysplasia is based on a synthesis defect or collagen structure, protein-carbohydrate complexes, structural proteins, as well as the necessary enzymes and cofactors. The immediate cause of the pathology under consideration of the connective tissue is the different kind of impact on the fruit, leading to a genetically deterministic change in the fibrillegenesis of the extracellular matrix. Such mutagenic factors include:

unfavorable environmental situation
defective nutrition and bad mother habits,
burdened during pregnancy, etc.

Some researchers indicate the pathogenetic role of the hypomagnemiology in the development of a connective tissue dysplasia, based on the identification of magnesium deficiency in the spectral study of hair, blood, oral fluid. The collagen synthesis in the body is encoded by more than 40 genes, in respect of which over 1300 types of mutations are described. This causes a variety of clinical manifestations of connective tissue dysplasia and complicates their diagnosis.

Classification

Connectual dysplasia is divided into differentiated and undifferentiated. Differentiated dysplasia include diseases with a certain, installed inheritance type, clear clinical picture, known gene defects and biochemical disorders. The most typical representatives of this group of hereditary diseases of the connective tissue serve as Elessa-Danlos syndrome, Martan syndrome, imperfect osteogenesis, mucopolysaccharideoses, systemic elastosis, dysplastic scoliosis, bils syndrome (congenital contracture arachnodactylum), etc. The group of undifferentiated connecting dysplasia is different pathologies whose phenotypic signs No one of the differentiated diseases correspond.

According to the degree of severity, the following types of connective tissue dysplasia are distinguished: small (in the presence of 3 and more phenotypic signs), isolated (with localization in one organ) and actually hereditary connective tissue diseases. Depending on the prevailing dysplastic stigs, 10 phenotypic versions of the connective tissue dysplasia are distinguished:

Marfanopod-like appearance (includes 4 and more phenotypic signs of skeletal dysplasia).
Marfanopodoodnoye phenotype (incomplete set of signs of marfana syndrome).
MSS-phenotype (includes the lesion of aortic, mitral valve, skeleton and leather).
The primary prolapse of the mitral valve (characterized by echo-signs of mitral prolapse, changes from the skin, skeleton, joints).
Classic Ecere-like phenotype (incomplete set of signs of Elessa Dunlos syndrome).
Hypermobile EuremsOther phenotype (characterized by the hypermobility of the joints and related complications - sublifting, dislocation, stretching, flatfoot; arthralgia, bone involvement and skeleton).
The hypermobility of the joints is benign (includes an increased volume of movements in the joints without the interest of the bone-skeletal system and arthralgium).
Untifferentiated connective tissue dysplasia (includes 6 or more dysplastic stigs, which, however, is not enough to diagnose differentiated syndromes).
Increased dysplastic stigmatization with preferential bone-articular and skeletal signs.
Increased dysplastic stigmatization with preferential visceral signs (small heart anomalies or other internal organs).

Since the description of differentiated forms of connective tissue dysplasia is given in detail in the corresponding independent reviews, in the future it will be about its undifferentiated versions. In the case when the localization of the connective tissue dysplasia is limited by one body or system, it is isolated. If the connective tissue dysplasia appears phenotypically and captures, at a minimum, one of the internal organs, this state is considered as the syndrome of the connective tissue dysplasia.

Symptoms of connective tissue dysplasia

Phenotypic signs

External signs of connective tissue dysplasia are represented by constitutional features, abnormalities for the development of skeleton bones, leather, etc. Patients with connective tissue dysplasia have an asthenic constitution: high growth, narrow shoulders, body weight deficiency. Violations of the axial skeleton can be represented by scoliosis, kyphosis, funnel-shaped or cylindic deformations of the chest, juvenile osteochondrosis.

Cranecephalous stigms of the connective tissue dysplasia often include dolikhetiaphalius, bite disorders, teeth anomalies, the Gothic sky, the outstanding of the upper lip and the nose. The pathology of the bone-articular system is characterized by an o-shaped or X-shaped deformation of the limbs, sindactile, the arahnodactyl, the hypermobility of the joints, flat-painting, a tendency to the usual dislocation and sublifting, bone fractures.

From the side of the skin, an increased stretchability (hyperacter) or, on the contrary, fragility and dryness of the skin are noted. Often, rain, pigment stains either, or centers of depigmentation, vascular defects (teleangectas, hemangiomas) arise on it without visible reasons. The weakness of the muscular system with a connective tissue dysplasia determines the tendency to the omitting and loss of internal organs, hernias, muscular crying. From other external signs of connective tissue dysplasia, such microanomalies may occur as hypo-or hypertelectricism, burrows, the asymmetry of the ears, the low line of hair growth on the forehead and neck, etc.

Changes from the internal organs

Visceral lesions proceed with the interest of the CNS and the vegetative nervous system, various internal organs. Neurological disorders associated with connective tissue dysplasia are characterized by vegetative-vascular dystonia, asthenia, enuresum, chronic migraine, speech impairment, high anxiety and emotional instability. The syndrome of the connective tissue dysplasia of the heart may include a mitral valve prolapse, an open oval window, aortic hypoplasia and a pulmonary trunk, elongation and excessive mobility of chord, the aneurysms of the coronary artery or the interpresenting partition.

The consequence of the weakness of the walls of venous vessels is the development of varicose expansion of the lower limbs and a small pelvis, hemorrhoids, varicocele. Patients with a connective tissue dysplasia have a tendency to the emergence of arterial hypotension, arrhythmias, atrioventricular and intraventricular blockades, cardialgies, sudden death.

Cardial manifestations often accompanize the bronchopile syndrome, characterized by the presence of cystic hypoplasia of light, bronchiectasis, bullous emphysema, repeated spontaneous pneumothoraxes. Characterized by the lesion of the gastrointestinal tract in the form of omission of internal organs, the diverticulus of the esophagus, gastroesophageal reflux, hernia of the esophageal hole of the diaphragm. Typical manifestations of pathology of the organ of vision, with a connecting tissue dysplasia, serve myopia, astigmatism, hyperopia, nystagm, strabismus, subsidiaries and lens.

From the urinary system, nephroptosis, urinary incontinence, renal abnormalities (hypoplasia, doubling, horseshoe kidney), etc. Reproductive violations associated with connective tissue dysplasia can be represented by the omission of the uterus and vagina, metro- and menorragia, spontaneous abortions, postpartum bleeding; Men have cryptorchism. Persons having signs of connective tissue dysplasia are prone to frequent ORVI, allergic reactions, hemorrhagic syndrome.

The recently recent medical term - dysplasia - means possible any species of pathologies in the development of different parts of the human body, all kinds of organs and a number of tissues. The appearance of pathologies begins to form at the stage of the fetus embryonic development, becomes the result of incorrect maturation or the structure of the cells, their configurations, size. It affects the incorrect creation of tissues, which emerged by the pathology of the body or system of organs.

However, the dysplasia is more often detected by the younger child, examples of the manifestation of pathologies in adults consisted of people. Dysplasia is not a disease, but pathological changes in the structure of organs.

The frequent causes of the appearance of pathologies become genetic deviations of the formation of the fetus. Dysplasia is developing in the case of hormonal failures in the body of a woman during pregnancy or oxygen starvation of vessels, including the inner walls of the vessels with their direct mechanical voltage. An important factor becomes polluted or unfavorable ecology, the atmosphere, water in the terrain of residence of the future mother. Perhaps gynecological difficulties with the health of the future mother, frequent infectious diseases contributed to the development of pathology.

Pathology can be transmitted from generation to generation with less noticeable symptoms, accumulate for future generations. If both parents have a predisposition to dysplasia, gene deviations, children's health are identified. Acquired dysplasia often becomes a consequence of generic injuries or postpartum.

Share a number of displaced dysplasia types:

, or fibrous muscular, is distinguished by the large growth of cells in the walls of the arteries. Pathology is found mainly in carotid arteries or renal. The main trend is the narrowing of the arteries.
Hip joint dysplasia.
or muscle dysplasia.
Dysplasia cervix.
, No tension of the muscular-ligament apparatus of the knee joint.

Difficult pathology is considered soft tissue dysplasia by a child or muscle dysplasia. Pathology is formed by intrauterine, at the genetic level. During the development of the embryo, a failure occurs at the time of the formation of connective tissue cells, provoking the difficulties with the muscles, internal organs - Connecting tissue in the body is the main building material. During muscle dysplasia, anomalies appear in the work of the body as a whole, complex pathology is difficult for setting the correct diagnosis.

Deviations associated with connective tissue dysplasia are becoming noticeable gradually as the child develops and its physical growth. Valgus Structure of the Foot, the overall curvature of the spine, the deformation of the chest, the increased mobility of the joints, are possible violations in the work of the heart, maligation with digestion, falling vision, pathology vascular system. A row is voiced, characterizing muscle dysplasia by a child. Deviations are detected separately either together.

Treatment of muscular dysplasia

Dysplasia is not a disease, but the deviation at the genetic level, the treatment of pathology is not amenable. The main methods of maintaining children are preventive measures that contribute to the decrease in signs of dysplasia, slowing down or stop the syndrome. Mandatory immediate medical intervention will help speed up muscle recovery.

The main methods of treating and preventing muscle dysplasia are compliance with balanced diet, physiotherapy and specially designed gymnastic exercises, use only to appoint a doctor of medication drugs. At the stage of prolonged non-interference, operational treatment may be required.

Mandatory psychological support for a child, a frank complicity in procedures will help the baby tune in to the restoration path, it is easier to transfer the procedures. After the correct diagnosis, parents should not relax, believing that tricks on doctors are over, and the treatment is not difficult. Dysplasia is not a terrible pathology, but deviation. For a positive result, all family members will need to participate in the recovery period of the child.

Non-drug treatment consists of a mandatory treatment massage, repeating in several courses, non-professional sports activities - table tennis, swimming or badminton. Customized gymnastic exercises, physiotherapy, including dialing and. Food for children is assumed dense, in order to achieve the required level of collagen. Mandatory use of fish in the daily diet, should be given preference bean products, meat and seafood. Great broths are important for a child, a large amount of quality cheese, a variety of fruits and vegetables. When agreeing with the doctor, you can use biological additives.

With drug treatment, the doctor selects the optimal set of drugs individually for each child. The course of treatment with pills lasts up to 2 months, continues exclusively on the prescription of the doctor. Repeating treatment courses can be extended repeatedly, drugs change to achieve the best result.

Glycine and glutamic acid are taken to standardize the amino acid level. In order to the early development of collagen production, ascorbic acid, magnesium citrate. Alpha cells and osteogenone will advise the normalization of mineral metabolcidol. In order to improve the biodenergetic state of the child, lecithin, riboxin.

The main points for operational intervention are extremely difficult cases of muscle dysplasia. Vividly pronounced vascular pathologies or strong spinal curvature, heavy deformation of the chest. Surgery is obligatory if the syndrome carries a threat to the child's future life, or with developed deviations it is impossible to live normally.

Rehabilitation methods

A clear execution of the listed recommendations will allow the child to adapt to the outside environment, to catch up in the development of peers, with the correct motivation of the baby, it is possible to become better peers and gain full health. Do not forget that the dysplasia detects inheritance. Attracting all family members to uncomplicated procedures will help strengthen health, will create a cozy atmosphere in the house.

Contraindications for children with muscle dysplasia

Heavy or contact sports contribute to the overvoltage of muscle tissue and its destruction;
Psychological loads contribute to nervous overvoltage and insufficiency of oxygen entering the body;
Spine stretching will lead to injuries and development of joint dysplasia.

The connecting tissue, which is about 50% of the entire body weight and bonds together all the tissues of the body, is formed from the first days of the life of the fetus. With a shortage of components from which connective tissue is built, serious developmental anomalies arise. With a pronounced shortage of "building materials" of the connective tissue, these anomalies are incompatible with life already in the intrauterine period (frozen pregnancy, etc.). With a less pronounced deficit, the child is born viable, but is characterized by dysmerfizm and lower body weight and growth.

Therefore, violations of the structure of the connective tissue (or dysplasia of the connective tissue, DST) contribute to the development of a wide variety of diseases, which seemed to have nothing to do with the pathology in children and adolescents: scoliosis and varicose veins, "school" myopia and nephropotosis, a mitral valve prolapse and Flattead, as well as many others. Obviously, all these diseases combines in some kind "weak", insufficiently formed connecting tissue. The mechanically weak connecting tissue is the basis of morphofunctional changes not only with scoliosis, osteoporosis and other pathologies of cartilage and bone tissue, but also in cardiovascular and cerebrovascular diseases. Structural inferiority and reduced regenerative ability of the connective tissue of vessels determine the increase in chronic inflammation levels, less efficiency of traditional treatment regimens, a longer period of recovery, etc.

The connecting fabric differs from any other type of fabric with an excess of extracellular matrix. Extracellular matrix consists of a basic substance (proteoglycans), mechanically reinforced with fibers of three types: 1) collagen fibers (consisting mainly of type I collagen), 2) flexible fibers (consisting mainly of elastin and fibrillins) and 3) mesh (or reticular ) Fibers (type III collagen). It should be noted that magnesium-dependent enzymes participate in the synthesis of these components of the connective tissue. In addition, magnesium regulates the secretion of pararathgamon, vitamin D metabolism and potentiates vitamin D effects in bone tissue, which is important for therapy and prevention of vitamin-D-resistant rickets. Therefore, magnesium deficiency provokes and exacerbates dysplastic processes in the connective tissue, worsening its strength and elasticity. The relationship between dysplastic processes in the connective tissue and magnesium deficiency is particularly relevant in children constantly in the period of active growth.

At present, the data indicate that the Embassibility of the DST depends on the age of the surveyed persons. Growth processes in children flow unevenly. Several periods of the most intensive growth are distinguished: the 1st year of life, the period of preparation for school (5-7 years), the period of the so-called adolescent "jerk" (11-15 years). In each of these transition periods, DST is manifested in different ways. In the 1st year of life, Rakhit, hypotension, sinks, hypermobility of joints are most often found at DST; During the preparation for the school, myopia and flatfoot often start; During the period of adolescent "jerk" - scoliosis, thoracic deformations and spine, stricy of the hips and abdomen, the prolapse of the mitral valve. In adolescence, the increase in the number of signs of dysmorphogenesis of the connective tissue can be more than 300%.

It should be noted that the most intensive growth is observed in premature babies and children with low weight at birth. These children constitute a risk group for the development of DST. Given the early formulation of such children registered, the doctor and his developing patient has a large time reserve for organizing a comprehensive rehabilitation program, which includes kinesotherapy, sports and full-fledged nutrition.

Adequate diet necessarily includes sufficient child support by all micronutrients, which are building materials of connective tissue. Taking into account the widespread distribution among children of dietary deficits of micronutrients, first of all magnesium, it becomes obvious the need to use effective and safe drugs of organic magnesium. With DST, magnesium therapy courses must be long enough (at least 6 months).

On the diagnosis of DST

The dysplasia of the connective tissue is a genetically and non-recorded deterministic state due to disorders of the connective tissue metabolism in embryonic and postnatal periods and characterized by anomalies of the structure of the components of the extracellular matrix (fibers and the main substance) with preventive morphofunctional changes in various systems and organs.

An inspection of the child with a neonatologist is already immediately after birth allows you to establish a number of characteristic phenotypic manifestations of DST. Conditionally, they can be divided into groups depending on the localization of organs and systems involved in the dysplastic process. The following signs listed below are not strictly specific for DST and need clinical evaluation and conducting clarifying differential diagnostic analysis if necessary.

1. Kostoy-articular changes:

asthenic type of constitution;
dolichosteniel;
arahanodactilia;
deformation of the chest (funnel-shaped and celoid);
spinal deformations (scoliosis, straight back syndrome, hyperkyphosis, hyperlordosis, spondylolistera);
deformation of the skull (acrocephaliya, arcoid sky, micrognathy, vacifics of teeth);
deformations of the limbs (Valgusny, Varetle);
stop deformations (flatfoot, hollow stop, etc.);
the hypermobility of the joints.

2. Changes in skin and muscles:

stretching;
thin skin;
sluggish
healing in the form of "Papile paper";
keloid scars;
hemorrhagic manifestations (ekkimosis, petechia);
muscle hypotension and / or hypotrophy;
hernia.

3. Signs of the DST of the organ of vision:

myopia;
flat cornea;
submission (dislocation) lens.

4. Signs of DST cardiovascular system:

heart valves prolaps;
mixomatous degeneration of valve structures of the heart;
dilatation of fibrous heart rings;
expansion of the root of the aorta;
the aneurysms of the interpresentation, interventricular septum of the heart;
expansion and aneurysm of vessels (aorta, pulmonary artery, cerebral artery);
varicose veins, phlebopathy.

5. Signs of DST Bronchildren System:

tracheobormy, tracheobronomegaly;
tracheobronchial dyskinesia;
bronchiectase;
apical Bully and Primary Spontaneous Pneumothorax.

6. Signs of DST of the digestive system:

motor-tonic disorders (reflux);
disorders of fixation of the organs (gastroptosis, coloncoatosis);
changes in size and length of hollow organs (Megalolon, Dolichosigma, etc.).

7. Signs of dst urinary system:

nephroptosis, reflux.

8. Signs of the DST of the blood system:

thrombocytopathy, coagulopathy;
hemoglobinopathy.

9. Signs of the Nervous System:

vegeta dystonia.

It should be noted that in the international classification of diseases (ICD-10), undifferentiated DST variants are not highlighted in a separate category, which undoubtedly makes it difficult to work a practical doctor. However, with careful work with the classification, you can find the appropriate code for any manifestation of DST. For example, the diagnoses "I34.1 of the Mutral Valve Prolapse", "I71.2 aneurysm and aortic bundle", "I83 Varicose veins of the lower extremities" in the heading I00-99 "Circulatory System Diseases" are clearly characterized by violations of the structure of the connective tissue. Other examples: "H52.1 Myopia", "H27.1 Submission (dislocation) of a lens", "k07 bite anomalies", "K40 grozhai hernia", "K41 femoral hernia", etc. Therefore, the DST is not limited to the diagnosis in Rubric M00-99 "Diseases of the Muscular System and Connective Tissue" ("M35.7 hypermobile syndrome", "M40.0 Kyphoz position" and others).

Survey of patients with DST is carried out in a strict sequence, in accordance with the following tasks:

identification of small anomalies of development and malformations;
identification of phenotypic signs of DST;
differential diagnosis of syndromic and non-embedrome forms;
assessment of the degree of precursion of the flow;
determination of the risk of developing the complications of the flow, the emergence of associate pathology, sudden death;
assessment of the degree of ability to work.

The search for phenotypic features of the DST should be carried out in a physical examination purposefully and consistently. More detailed information on the diagnosis of DST, extremely important for a practical physician, is given in the monograph of Nechava G. I. et al., 2010.

Molecular biological mechanisms of interconnection of DST and magnesium deficiency

Understanding the role of magnesium in maintaining the structure of the connective tissue is not separated from the molecular cell structure of the connective tissue. In molecular biology, extracellular matrix (VKM) is defined as a complex network formed by numerous structural macromolecules (proteoglycans, collagen, elastin). Interacting with each other and with cells, they maintain the structural integrity of the tissues. The connecting tissue demonstrates excess VKM with a sufficiently small number of cells. It is WCM who helps keep cells together and provides an organized medium, within which migratory cells can move and interact with each other.

Extracellular matrix consists of fundamentally necessary components - the main substance, collagen, elastin fibers. The most important element of VKM is the main substance formed by proteo-glycans - extremely stretched polypeptide chains, connected to numerous polysaccharide glucosaminoglycan molecules by means of durable covalent bonds.

Numerous proteoglycan chains are attached to a special type of glucosamineoglycan - polymer hyaluronic acid, Called by Haluronan. Hyaluronan threads help to fasten the structure of the main substance into a single whole. This prevents compression and stretching VKM, and also provides fast diffusion nutrients and hormones to connective tissue cells. Hyaluronan is synthesized by hyaluronancentatase (HAS1, HAS2 and HAS3 genes) and degraded by hyaluronidases (Hyal2, Hyal3, Hyal4 and Hyalp genes). Has1, Has2 and Has3 hyaluronancetase contain magnesium ion in the active center. Magnesium deficiency leads to a decrease in the activity of hyaluronansintytase and, as a result, to deterioration of the mechanical properties of Hyaluronan yarns in the main substance of the extracellular matrix.

Enzymes involved in biochemical modifications and the addition of glucosaminoglycans can also significantly affect the structure of VKM. For example, Xylosyl-beta-1,4-galactosyltransferase-7 (B4GALT7 gene) is associated with one of the forms of DST - Elessa-Dahn Syndrome, which is manifested by the tendency to dislocation, the presence of fragile or hyperalastic leather, fragile vessels, etc.

Collagen fibers give connective tissue strength and durability. Each collagen fiber is several micrometers in diameter and consists of thousands of individual polypeptide collagen polypeptide chains, tightly packed together. It should be noted that the dysplasia of the connective tissue most often occurs not so much due to genetic defects in collagen, as a result of defects in dozens of genes affecting biosynthesis, post-translation modifications, secretion, self-assembly and remodeling of collagen fibers. For example, Lysiloxidaz (LOX gene), as well as lines-like enzymes (LOXL1, LOXL2, LOXL3 and LOXL4 genes), carry out a cross-stitching of the collagen polypeptide chains, thus enhancing the mechanical strength of the fibrils. Lyysocidase activity deficit is detected in patients with Elessa-Danlo syndrome.

It was shown that magnesium contributes to a decrease in the level of activity of matrix metalloproteinases (MMP) (Ueshima K., 2003). Accordingly, magnesium deficiency leads to an increase in the total activity of MMP and more aggressive degradation of collagen fibers, which also worsens the mechanical strength of the connective tissue. Experiments confirm the effect of magnesium on the biological activity of MMP. In mice with an artificially caused magnesium deficiency, the aorta wall is much thinner than that of control animals. These changes correlate with an increase in the total activity of MMP2 and MMP9 metalloproteinase. Probably, the effect of magnesium in reducing the activity of MMP2 is blocked by two tyrosine kinase inhibitors - genisteine \u200b\u200band herbemicin. This suggests that extracellular magnesium reduces MMP secretion through an intracellular signal cascade, which includes a certain tyrosine kinase. The addition of diet with folic acid and magnesium salts reduces the secretion of MMP2 and has a positive effect, in particular, for the course and forecast ischemic Disease Hearts (IBS).

Cells (fibroblasts, chondroblasts, osteoblasts) - active component of the connective tissue. It is the cells that the elements of the extracellular matrix (proteoglycans, collagen, elastin fibers, fibronectin, etc.) and support the structural integrity of the connective tissue support. Cells also secrete all the enzymes necessary for the formation and remodeling of the connective tissue (metalloproteinase, etc.).

It should be noted a significant effect of trace elements, in particular magnesium ions, on the synthesis processes by cells of the connective tissue. In particular, Mg 2+ ions stabilize the structure of transport RNA (TRNA), and magnesium deficiency leads to an increase in the number of dysfunctional TRNA molecules, thus reducing and slowing down the overall speed of protein synthesis. The studies showed that the low content of magnesium stimulates the premature death of endotheliocytes and fibroblasts in culture. Other possible mechanisms of magnesium influence are the increase in the activity of metalloproteinase-elastases (degrading elastic fibers), transglutaminase (forming cross-glutamine-lysine elastin crosslinks), Lysicoxidase (cross-stitching of elastin chains and / or collagen), hyaluronidases (degrading hyaluronan). These mechanisms are summarized in Fig. one.

The positive effect of magnesium on the structure of the connective tissue is confirmed by the results of the experimental study by us on the models of the Russian Academy of Sciences and burns. The results of the experimental study of the effects of organic salts of magnesium (magnesium lactate dihydrate) on the epithelization of the Russian Academy of Sciences and burns showed that the intake of organic magnesium inside stimulates more efficient and rapid healing of wounds compared with standard solk seriflue therapy. According to the results of histological analyzes of the scar tissue in various groups of animals, magnesium receiving prevents the growth of collagen fibrils, promotes the growth of elastin fibers, the increase in the number of fibroblasts of the connective tissue and the formation of a full-fledged basic substance, which in general leads to an increase in the histological quality of the scar.

On therapy DST in children

The contribution of heredity into the development of a multifactorial disease, to which the DST is also of no more than 20%. The share of environmental impacts and the possibility of clinical medicine in improving health accounts for about 30%, and the main value (50%) in the development of the disease has the way of life of the patient. From a clinical and prognostic point of view, non-intromic dysplasia is divided into three distinctly distinguishable groups, which requires a differentiated approach to the implementation of therapeutic and prophylactic technologies (Fig. 2).

In the absolute majority of cases, the main task of observing young patients is to preserve health and prevent the progression of dysplastic processes. The main approaches to the treatment of patients with DST are rational diet therapy, metabolic therapy, physiotherapy, therapeutic massage, individually selected therapeutic physical culture and swimming. In the absence of significant functional disorders, children with DST are shown a general mode with proper alternation of labor and recreation, feasible morning gymnastics, alternation of mental and physical activity, walking in the fresh air, full night sleep, short rest during the day. Dynamic loads (swimming, walking, walking skiing, bicycle, badminton, gymnastics, bicycles, bike, badminton, gymnastics) and inexpedient ballet and dance classes, group gaming sports related to the high probability of injuries.

An important direction of treating patients with DST is rational diet and therapy. Its main goal is considered to provide the body in sufficient micronutrients (vitamins, trace elements, vitamin-like substances, etc.) necessary to maintain healthy metabolism of connective tissue. Diet therapy is complemented medical treatment Using vitamin-mineral complexes and vitamin monoforms (vitamins D, C, etc.) and / or minerals (monoforms of magnesium, zinc, copper, manganese, boron, etc.). Especially it should be noted the role of vitamins C, E, in 6 and D.

Among the microelements of magnesium, copper and manganese are especially important to maintain the physiological metabolism of the connective tissue. For the structure of the connective tissue, the role of magnesium is extremely important, which is one of the main bio-elements that ensure the physiological metabolism of the connective tissue.

With the correction of deep magnesium deficit, it is difficult to do only diets and often requires pharmacotherapy. Bioaccipation studies When using various magnesium preparations gave reason to assert that the bioavailability of magnesium organic salts is almost an order of magnitude higher than inorganic. At the same time, organic salts of magnesium are not only much better absorbed, but is easier tolerated patients. Inorganic salts of magnesium more often give dyspeptic complications, such as diarrhea, vomiting, stomaching. Treatment is more effective if the magnesium and the magnesophyxter (vitamins in 6, in 1, glycine) are introduced simultaneously.

Among the drugs used to correct the magnesium deficit, the drug Magne in 6 has permission to use in pediatrics. The shape of Magne in 6 in the form of a solution for intakes is allowed to receive in children from the first year of life (body weight more than 10 kg) at a dose of 1-4 ampoules per day. Magne Tablets in 6 and Magne in 6 forte are allowed for children over 6 years old (body weight more than 20 kg) at a dose of 4-6 tablets per day.

It should be emphasized that diet therapy in patients with DST is an integral part of a comprehensive treatment program for the corresponding "main" manifestation of the DST among this patient. For example, in the case of a mitral valve prolapse (PMC), orthostatic symptoms (postural hypotension and heartbeat) can be reduced by increasing the consumption of fluid and salt, wearing compression linen, in severe cases of mineralocorticoids. The reception of acetylsalicylic acid (75-325 mg / day) is shown to patients with PMK with transient ischemic attacks in the sinus rhythm and without blood clots in the left atrium. Antibiotics for the prevention of infectious endocarditis with all manipulations accompanied by bacteremia are prescribed to patients with PMK, especially in the presence of mitral regurgitation, thickening of sash, elongation of chord, dialing left ventricle or atrium.

There are literary data on the effectiveness of magnesium preparations for primary PMK. It was shown that after six months of regular intake of organic magnesium, the heart rate and blood pressure level was normalized, the number of episodes of rhythm disorders decreased, but the tremor and the depth of the sash of the mitral valve decreased significantly.

Conclusion

The dysplasia of the connective tissue combines such diseases of children and adolescents, like scoliosis, rickets, flatfoot, disorders of the organ fixation (gastroptosis, nephroptosis, coloncoatosis), a mitral valve prolapse, myopia and others. It deserves special attention to the fact that the DST in childhood is the pathophysiological basis for the formation of cardiovascular and cerebrovascular diseases in adults. Thus, DST in childhood predisposes to reduce the life expectancy and deterioration of the quality of life in adulthood. The available data of fundamental and clinical medicine suggests that the DST is one of the clinical forms of manifestation of the primary magnesium deficiency. Therefore, the use of magnesium preparations can be considered as a means of pathogenetic treatment of DST. The earlier the non-recorded support is started against the background of modern magnesium deficient nutrition, the better.

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RU.MGP.12.01.08

A. G. Kalacheva *, **,candidate of Medical Sciences
O. A. Gromova *, **,
N. V. Kerimkulova *, **,
A. N. Galustyan ***,candidate of Medical Sciences, Associate Professor
T. R. Grishin *, **,doctor of Medical Sciences, Professor

* Russian Satellite Center of the Institute of Microelements UNESCO,Moscow
** GBOU VPO IGIM MINDRESSOVSDIVE OF RUSSIA,Ivanovo.
*** GBOU VPO SZGMU. I. I. Mechnikov Ministry of Health and Social Development of Russia,St. Petersburg