Aging is an inevitable process that affects every person, flowing gradually and continuously. However, there is a disease in which this process develops very rapidly, affecting all organs and systems. This disease is called progeria (from the Greek - prematurely old), it is extremely rare (1 case for 4 to 8 million people), in our country there are several cases of such a deviation. There are two main forms of progeria: Hutchinson-Guilford syndrome (progeria of children) and Werner's syndrome (progeria of adults). About the latter we will talk in our article.
Werner's Syndrome - the mystery of science
Werner's syndrome was first described by the German physician Otto Werner in 1904, but until now, progeria remains an unexplored disease, primarily because of the rare occurrence. It is known that this is a genetic disorder caused by a gene mutation, which is inherited.
For today, scientists have also determined that Werner's syndrome is an autosomal recessive disease. This means that patients with progeria receive simultaneously from the father and mother one anomalous gene located in the eighth chromosome. However, until now it is not possible to confirm or deny the diagnosis through genetic analysis.
The reasons for the progeria of adults
The main cause of the syndrome of premature aging remains unsolved. Damaged genes that are present in the gene apparatus of the parents of the patient with progeria do not affect their body, but when combined lead to a terrible result, condemning the child to suffering in the future and premature departure from life. But what leads to such gene mutations is still unclear.
Symptoms and course of the disease
The first manifestations of Werner's syndrome occur between the ages of 14 and 18 (sometimes later), after a period of puberty. Until this time, all patients develop quite normally, and then in their body the processes of exhaustion of all life systems begin. As a rule, at first the patients turn gray, which is often combined with hair loss. There are senile changes in skin: dryness, wrinkles , hyperpigmentation, skin tightening, pale.
There is a wide range of pathologies that often accompany natural aging: cataracts , atherosclerosis, cardiovascular system disorders, osteoporosis, various types of benign and malignant neoplasms.
Endocrine disorders are also observed: absence of secondary sexual signs and menstruation, sterility, high voice, thyroid dysfunction, insulin-resistant diabetes. Atrophy fatty tissue and muscles, arms and legs become disproportionately thin, their mobility is sharply limited.
Exposed to a strong change and facial features - they become pointed, the chin sharply protrudes, the nose acquires a similarity with the bird's beak, the mouth decreases. At the age of 30-40 years, a person with adult progeria looks like an 80-year-old man. Patients with Werner's syndrome rarely live up to 50 years, dying most often from cancer, heart attack or stroke.
Treatment of adult progeria
Unfortunately, there is no way to get rid of this disease. Treatment is aimed only at getting rid of the emerging symptoms, as well as preventing possible concomitant diseases and their exacerbations. With the development of plastic surgery, it was also possible to slightly correct the external manifestations of premature aging.
At present, tests are carried out for the treatment of Werner syndrome by stem cells. It remains to be hoped that positive results will be obtained in the near future.