Ehlers-Danlos Syndrome

Ehlers-Danlos syndrome is the most common hereditary
connective tissue disease. The incidence rate is one case
one hundred thousand newborns. Under this syndrome the group is united
diseases that are based on a defect in collagen, which is the basis
connective tissue. There are more than ten types of the disease, differing among themselves by some features. External signs that have Ehlers-Danlos syndrome are expressed in increased elasticity, skin vulnerability, joint mobility and symptoms of hemorrhagic diathesis. Damage to the respiratory system and the cardiovascular, musculoskeletal, and gastrointestinal organs is also noted.

Skin in patients with a diagnosis of Ehlers-Danlos syndromefragile, thin, weakly fixed to the underlying tissues. At the slightest injury to the skin, there are difficult-healing lacerated wounds, on the site of which scars with a wrinkled surface are formed. Ehlers-Danlos syndrome is characterized by anomalous mobility of the joints, resulting in chronic dislocations and subluxations of the joints. In most cases, the disease is accompanied by hemorrhagic diathesis, hematomas and bruises of various localization easily appear in patients. Typical bleeding from the gastrointestinal tract, gums, uterus.

Retinal detachment and tears are characteristic of some types of the disease.
eyeballs. Ehlers-Danlos syndrome is sometimes accompanied by abnormal
the formation of teeth and their partial absence, the development of periodontitis.
Disorders of the musculoskeletal system are manifested in the form of chest deformity
cells and spinal curvature. Hernias are often found in patients.
various localization. With the defeat of the internal organs the heart suffers,
vessels, bronchopulmonary system, multiple vascular aneurysms are formed
brain and aorta. With the defeat of the heart, various defects are formed.

Ehlers-Danlos syndrome

For accurate diagnosis of the disease, various studies are conducted in
specialized inpatient facilities. Great difficulties bring
obstetric-surgical problems with patients diagnosed with the syndrome
Ehlers-Danlos. Treatment is directed primarily to stabilization and
normalization of metabolic processes in the central nervous system and the cardiovascular system,
musculoskeletal system. Therapy for this disease includes
taking a wide range of vitamins B, A and E. Good results give
carboxylase and ascorbic acid, growth hormone injections (somatotropic
hormone), carnitine chloride. Patients are recommended to take medications that improve brain activity (pantogam, nootropil, riboxin, rutin, panangin). In addition, it is necessary to regularly conduct such physiotherapy procedures as laser acupuncture and magnetic therapy. Patients are also prescribed courses of physical therapy and massage, reflexology sessions.

In the treatment of the disease, great attention must be paid to chronic foci.
infections located in the oral cavity and nasopharynx (adenoids, chronic
tonsillitis, sinusitis, carious teeth). Special treatments for the syndrome for now
not developed. Recommended respect for a gentle lifestyle and, as far as
possibly limiting the load on the body. Symptomatic therapy includes surgical correction of chest deformities, removal of pseudo-tumors, treatment of eye and heart pathologies, etc. Projections for life depend mainly on the type of syndrome. Prevention is medical genetic counseling.