Dwarfism Polydystrophic

Dwarfism, or dwarfism, is a congenital genetically determined disease characterized by dysmorphogenesis in the process of human growth and development, manifested in a decrease in body size, corresponding to a delay in its functional development

Polydystrophy is a two-way process. There is central and peripheral damage. Depending on the prevalence of the disease, regional, systemic and local polyorganopathies are distinguished.

Name: Polydystorophic dwarfism or Excessively small human body (Tsereteli-Klimov disease)

Atopic dwarfism is a group of inherited diseases associated with excess growth of the limbs or other parts of the body. The etiology and pathogenesis of the disease are not fully understood, but it is known that approximately one gene, which is a precursor to the synthesis of several proteins, is involved in its development. If this antecedent occurs in an unsatisfactory manner, excessive growth of the limbs occurs. It is widely believed that “atopic” dwarfism occurs due to a congenital, sometimes hereditary, increase in the number of collagen fibers under the influence of growth hormone. Increased collagen synthesis also occurs in the healthy bodies of newborn babies and children, but this does not lead to pathological growth of the limbs - the size of the articular arches, arms and legs in a healthy child is simply proportional to the size of other parts of his body and increases in proportion to age. Adolescents with chronic malnutrition (malabsorption syndrome) also have disproportions between the size of the osteoarticular elements and the size of the body (osteopetrosis). What is the difference between “atypical” and atopic dwarfism? We are talking about various reasons for development, severity, forms of manifestation. It is believed that atopic is accompanied by tall stature, while atypical is accompanied by a proportionally small body with pathologies of the limbs. The cause of these manifestations may be a genetic defect in one or more genes responsible for the synthesis of proteins responsible for the normal formation of tissues, tendons, joints and bones. As a result of these

Polydystrophic dwarfism, or dystrophic dwarfism (from the Greek μείζων - dwarf and -dys - deficiency), is a congenital disproportionately reduced lower part of the body, characterized by a narrowing of the lower part of the body, a flattened pelvis, and a decrease in the size of the lower extremities. The name of the disease “nanism” refers to two diseases associated with decreased growth, regardless of the cause of origin - Shereshevsky-Turner syndrome (this is dysgenesis of the gonads) and congenital dwarfism, which is also called “nanism”. Unlike dystrophy, in which a large body mass goes into the head, and the limbs are slightly underdeveloped, in the case of Dystrophic dwarfism the opposite is true - the limbs are underdeveloped. At the same time, the head remains normal in size. The causes of this form of disease are unknown. But it is the main cause of childhood underdevelopment (neuromuscular, congenital, endocrine pathologies). The main symptom of this disease is underdevelopment of the lower body. This condition involves a reduction in the trunk or lower part in length and weight compared to the average for the child's age. This syndrome is diagnosed only after two years of life.

Dwarfism is an abnormal change in body size and proportions in children.

Forms of dwarfism in children are classified according to a number of parameters:

1. Etiological form, based on the duration of the acquired form: - Acquired dwarfism, caused by internal processes or diseases. - Congenital dwarfism, a congenital form of dwarfism. 2. The kinetic type, as a manifestation of a person’s lifestyle, is based on the role of physical or mental factors in the development of the disease. 3. Severity: - Mild, mild, without clinical symptoms. There is no pronounced deficiency in the vital functions of the body. - Moderate or moderate severity. A complex of clinical signs appears, the degree of which determines the patient’s condition. It is possible that pulmonary pathologies may be added to the overall picture of the disease. Fatigue is pronounced. Dysfunction of the cardiac system and gastrointestinal tract may occur. - Severe form. There is a significant range of symptoms and signs of the disease. Long-term exhaustion occurs, which is accompanied by a general change in the functioning of internal organs and systems. The person experiences significant physical and psychological difficulties. 4. Type of flow. There is an acute course and a chronic course of the pathology. The acute form manifests itself within 3 months from the onset of the disease. The chronic course lasts from three months to 2 years. Classification can also be based on developmental dynamics. Crisis is distinguished between acute and chronic. An acute crisis can develop in a short period of time and determine the transition to the chronic stage of the disease. In chronic crisis, weight gain is not always observed. In any case, there is a slowdown in biological development until normal body size is achieved. 5. The ability to maintain vital functions. All children with dwarfism have the opportunity to fully work and live independently. Patients with mild changes and minimal symptoms are subject to an independent lifestyle. However, there is a need to organize special rehabilitation for these patients. It depends on the intensity of symptoms, and those situations that require the participation of specialists to help people.