Surgical methods of treatment
If you have muscular dystrophy, problems such as contractures may occur. Carrying out surgery on the tendons can significantly reduce this phenomenon.
Also, in some cases, surgery may be required to correct scoliosis. Indeed, in this case, any curvature of the spine can lead to difficulty breathing, as well as disruption of the functioning of the heart muscle.
If, nevertheless, the functioning of the heart is impaired under the influence of muscular dystrophy, then doctors recommend surgical intervention to install a pacemaker. This device allows the heart to beat faster and maintain the desired rhythm.
Etiology and pathogenesis
In the occurrence of Dystrophy in children, exogenous and endogenous factors are important. Exogenous include: nutritional (underfeeding, qualitative violation of the composition of food, the predominance of carbohydrates in it with a small amount of protein and fat, lack of vitamins), infectious (dysentery, pneumonia, etc.) and toxic factors, as well as errors in child care (violation gig regime, lack of a set daily routine, etc.). Among the endogenous causes, anomalies of the child’s constitution, endocrine and neuroendocrine disorders, malformations of various organs and systems (central nervous system, cardiovascular system, gastrointestinal tract, kidneys, lungs, etc.) are important. hereditary metabolic disorders - amino acid, carbohydrate, fat, for example, malabsorption syndrome (see Malabsorption syndrome), galactosemia, etc.
The pathogenesis of Dystrophy is complex. There is a decrease in brain excitability and the regulatory activity of c is disrupted. n. pp., which affects the functions of the gol.-intestinal. tract (the absorption of proteins, fats and vitamins is impaired), the enzymatic energy of the blood decreases, the processes of absorption of nutrients by tissues and cells of the body change. A disorder of nutrition and all types of metabolism develops. The body uses proteins, fats and carbohydrates from its own tissues to maintain vital functions, which leads to exhaustion.
In the formation of intrauterine D., nutrition during pregnancy is of great importance (see Pregnancy, hygiene and nutrition of pregnant women), which may be sufficient in caloric content, but insufficient or excessive in the content of individual food ingredients. If the mother’s diet contains insufficient protein, especially animal protein, and some minerals, the child may be born with stunted height and weight, or with sufficient or even excess weight due to protein-free edema (see Nutritional dystrophy). When carbohydrates predominate in a woman’s diet, the baby is born large due to excess deposition of fat formed from carbohydrates. A lack of vitamins in the expectant mother's diet leads to the birth of a child with signs of hypovitaminosis (see Vitamin deficiency), as well as retardation in weight and height. D.'s development is promoted by toxicosis of pregnant women (see), taking medications, prof. harmfulness. Various hron and diseases of women (heart defects, rheumatism, hron, pneumonia, pyelonephritis, anemia, etc.) that contribute to impaired uteroplacental circulation are also important. This leads to oxygen and protein starvation of the fetus, reduces the amount of blood flowing through the capillaries of the placenta, slows down the rate of blood circulation and thereby worsens the nutritional and metabolic conditions of the fetus and leads to a delay in physical function. its development in the prenatal period.
Symptomatic picture
The distal areas of the lower extremities are damaged in the form of paresis of the feet and legs, and muscle wasting is formed. Gradually, weakness and malnutrition spread to the hands and forearm area; in fairly late stages of the pathology, the proximal parts of the legs may also be involved. Initially, the Achilles reflexes are lost, followed by the knee and upper extremity reflexes. The presence of tendon retractions is not typical. In extremely rare cases, cardiomyopathy develops.
The main manifestation is muscle weakness in the limbs, in the initial stages - such as a slight discomfort and tingling sensation, premature fatigue after light physical activity.
With slow progression, the disease can last for many years, the symptoms increase, the muscles weaken and atrophy, the limbs undergo deformation.
In the later stages of the disease, the patient begins to complain of stiffness; it is difficult for him to grasp any object and hold it. If the leg disease spreads, the activation of the pathological process may result in the patient’s disability - he loses the ability to move independently and lead a normal lifestyle.
The transformation is usually characterized by symmetry; the skeletal muscular system of both extremities (upper and lower) is disrupted simultaneously. The distal parts of the limbs have a hypertrophied appearance; decreased tendon reflexes, loss of muscle tone, and gradual paralysis of the arms and legs contribute to increased myo-weakness.
Therapeutic measures
At the present stage, medications that completely relieve the patient from the manifestations of the pathological process have not been identified. Scientific tests are being carried out, the pathogenetic mechanism of myopathies is at the stage of study, experimental results are being analyzed, however, therapy now remains symptomatic, with the goal of somewhat stopping the development of the pathology and alleviating the patient’s condition.
To normalize the metabolic process in muscle fibers, the following is prescribed:
- vitamin therapy (vitamins B, E);
- amino acids;
- anticholenesterase drugs (ambenonium, galantamine);
- anabolic steroid drugs (methandienone);
- medications K and Ca.
Therapeutic measures are characterized by complexity, an individual selection of drugs is carried out. One therapeutic course lasts approximately four to six weeks, such courses should be carried out at least three times during the year.
In parallel with medications, the following has a fairly good effect:
- Physiotherapy (iontophoresis, electrophoresis, etc.).
- Massotherapy.
- Exercise therapy with minimal physical activity on the limbs (exercises in a swimming pool are recommended).
- Consultation with an orthopedic doctor (in some cases it is necessary to prescribe special orthopedic shoes).
Clinics in Germany
When making a diagnosis, it is important to exclude other diseases that cause muscle weakness. Therefore, multidisciplinary university-level clinics specialize in the diagnosis and treatment of muscular dystrophy in Germany. They help both children with the onset of the disease and adults with severe complications.
Charité University Hospital
, Berlin, traditionally ranks first in the ranking of the best clinics in Germany. The neurology department carries out all the necessary manipulations for a patient with Duchenne dystrophy - from neurophysiological studies to stem cell transplantation.
Beta Clinic
, Bonn. The Department of Neurology and Epileptology provides care to patients of all ages, including young children. In addition to active treatment, rehabilitation is also carried out here.
University Hospital Ulm.
The Department of Neurology is a research center for the development of therapeutic techniques and medications. This allows patients to receive treatment with innovative drugs before they go on sale.
Causal factors
The environment often becomes a kind of “trigger” for the formation of a pathological process; such provoking reasons include:
- Infectious diseases.
- Severe limb injury.
- Dystrophy, anorexic state.
- Intoxication.
- Excessive physical activity.
The disease can be acquired when the patient has endocrine disorders, severe chronic pathologies, is sick with drug or substance abuse, alcoholism and cancer.
