The progeria - causes and features of the course

The progeria is a disease that causes pathological changes in internal organs and the skin from premature aging. There are two basic forms: children and adult.

Children progeria is recorded very rarely. The causes and course of the disease are poorly understood. Worldwide there are only individual cases of manifestations of this disease. There are several episodes of the birth of children with this disease when blood marriages, which may indicate a hereditary type of this disease. Patients were detected changes at the gene level, malnutrition cells of different layers of the skin. In children the disease is considered to be congenital, but it is only in the second year of life. In this case, the child's slowing growth, notable nutritional disorders skin and subcutaneous tissue, in particular, on the face and extremities. The skin becomes dry and thin, there is the formation of dark areas on the skin. Patient's face becomes a bird with a hooked nose, underdeveloped lower jaw and protruding frontal tubercles, the whole head is not proportionately large in relation to the body. There is loss of teeth and hair, underdeveloped muscles, changes in the bone-joint system, the deterioration of vision.

In adults, the disease begins to manifest itself during puberty malnourished skin, slow growth, lack of development of secondary sexual characteristics. The third ten years of the patient significantly deteriorating eyesight, hair falls out, disappears subcutaneous tissue and thin skin, arms and legs become very thin, the nose thin and become the coracoid, and mouth become already. The patient appear trophic ulcers, osteomyelitis, celebrated diabetes, possible malignancy.

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Diagnosis for progeria established on the basis of signs. This disease should be differentiated from scleroderma and congenital poykilodermii. All treatment is aimed at the elimination and prevention of complications of atherosclerosis, venous ulcers, diabetes. The treatment is prescribed by a doctor-internist, endocrinologist or other physician depending on the predominant symptoms. The prognosis is poor: the overwhelming majority of patients die from complications and malignant tumors, osteoporosis. Prevention progeria not developed.

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