What is the name of the disease of rapid aging? Child or old man? Progeria - a mysterious genetic defect

This is an extremely rare genetic disease that accelerates the aging process by approximately 8-10 times. Simply put, a child ages 10-15 years in one year. Children with progeria appear normal for 6 to 12 months after birth. After this, they develop symptoms characteristic of old age: wrinkled skin, baldness, brittle bones and atherosclerosis. An eight-year-old child looks 80 years old - with dry, wrinkled skin, a bald head...

Features of such patients include dwarf stature, low weight (usually not exceeding 15-20 kg), excessively thin skin, poor joint mobility, underdeveloped chin, small face in comparison with the size of the head, which gives a person bird-like features. Due to the loss of subcutaneous fat, all blood vessels are visible. The voice is usually high. Mental development appropriate for age. And all these sick children are strikingly similar to each other.

Progeria also causes other problems: in children, for example, a second row of teeth appears in the mouth, and the skin becomes very pale, almost transparent.

These children usually die at the age of 13-14 simply “of old age.” More precisely, from those diseases that are characteristic of old age. For example, they can die from a trivial heart attack. And, as a rule, after several heart attacks and strokes against the background of progressive atherosclerosis, cataracts, glaucoma, complete loss of teeth, etc. Only a few live to 20 years or longer. People call this disease “canine old age.”

There are now about 60 known cases of progeria in people around the world. Of these, 14 people live in the United States, 5 in Russia, and the rest in Europe.

Until recently, doctors were unable to determine the cause of the disease. And only recently, American researchers discovered that the cause of “childhood old age” is a single mutation. Progeria is caused by a mutated form of the LMNA gene. According to the director of the National Genome Research Institute, Francis Collins, who led the study, the disease is not hereditary. A point mutation—when only one nucleotide is changed in a DNA molecule—occurs anew in every patient. A genetic mutation in the Lamin A protein causes accelerated aging of the body. And the young man - with his large protruding ears, bulging eyes and swollen veins on his bald skull - turns into a one hundred and sixteen year old man.

IN Lately some of these patients had a ghostly hope of recovery. American scientists have begun a clinical trial of a drug against Hudchinson-Gilford syndrome. If the tests can be brought to a successful conclusion, the victory over progeria will be a victory for people doing everything to save their children from imminent inevitable death.

Researchers in their work came across a drug - a farnisyltransferase inhibitor; it turned out to be able to block the production of this protein and, at least, stop the development pathological processes, and even reverse some of them.

However, there is a problem in identifying such patients. There are few of them and they are scattered all over the world. The initiative group took on a huge amount of work to find them. Patients live in different countries, you need to get their consent, the consent of their parents. It is finally necessary to bring them, if such consent is obtained, to Boston (tests are being carried out at Children's Hospital Boston). And the life of such children is short. It is believed that the maximum age to which a patient with progeria can live is 27 years But even this is a rare case.

Hussein Khan and his family are unique in their own way: this is the only case known to science when more than one family member suffers from progeria. And thanks to this family, scientists were able to make a real breakthrough in understanding the nature of the disease. Hana's husband and wife are cousins. Neither of them has progeria, nor do their two children, 14-year-old Sangeeta and two-year-old Gulavsa. Their 19-year-old daughter Rehena and two sons: 7-year-old Ali Hussein and 17-year-old Ikramul suffer from this disease. None of them have virtually any chance of living to 25.

Adult progeria manifests itself in the following symptoms. Slowly developing juvenile cataract. The skin of the feet, legs, to a lesser extent, hands and forearms, as well as the face, gradually becomes thinner, the subcutaneous tissue and muscles in these areas atrophy. On lower limbs 90% of patients experience trophic ulcers, hyperkeratosis and nail dystrophy. Atrophy of the facial skin ends with the formation of a beak-shaped nose (“bird nose”), narrowing of the mouth and a sharpening of the chin, reminiscent of a “scleroderma mask.” From endocrine disorders hypogenitalism is noted, late appearance or absence of secondary sexual characteristics, dysfunction of the superior and inferior parathyroid glands (disorder calcium metabolism), thyroid gland(exophthalmos) and pituitary gland (moon face, high voice). Osteoporosis is often observed. Changes in the fingers resemble those seen in sclerodactyly. Most patients with Werner syndrome die before age 40. Trials are currently underway to treat the disease with stem cells.

Humanity has not yet learned to fight all illnesses. To the number incurable diseases Progeria, or premature aging syndrome, should also be included.

What is premature aging syndrome

People started talking about progeria for the first time relatively recently. This is not surprising, because the disease is extremely rare - 1 time in 4–8 million people. The disease occurs at the genetic level. The aging process accelerates approximately 8–10 times. There are no more than 350 examples of the development of progeria in the world.

The disease affects males more than females (1.2:1).

The disease is characterized by severe growth retardation (manifests from an early age), changes in the structure skin, lack of hair and secondary sexual characteristics, as well as cachexia (depletion of the body). Internal organs often not fully developed, and the person looks much older than his actual age.

Progeria is a genetic disease that manifests itself as underdevelopment and premature aging of the body.

The mental state of an individual suffering from progeria corresponds to biological age.

Progeria cannot be cured and is the cause of the development of atherosclerosis ( chronic disease arteries), which ultimately leads to heart attacks and strokes. The result of the pathology is death.

Forms of the disease

Progeria is characterized by premature withering of the body or its underdevelopment. The disease involves:

childhood form (Hutchinson-Gilford syndrome);
adult form (Werner's syndrome).

Progeria in children can be congenital, but most often the first signs of the disease appear in the second or third year of life.

Progeria in adults occurs differently. The disease can suddenly overtake an individual at the age of 14–18 years. The prognosis in this case is also unfavorable and leads to death.

Video: progeria, or young old people

Reasons for the development of progeria

The exact causes of progeria in currently not detected. There is an assumption that the etiology of the development of the disease is directly related to the disruption of metabolic processes in connective tissue. Fibroblasts begin to grow through cell division and the appearance of excess collagen with low glycosaminoglycan levels. Slow formation of fibroblasts is an indicator of the pathology of intercellular matter.

Causes of progeria in children

The cause of the development of progeria syndrome in children is changes in the LMNA gene. It is he who is responsible for encoding lamin A. We are talking about a human protein from which one of the layers of the cell nucleus is created.

Often progeria is expressed sporadically (randomly). Sometimes the disease is observed in siblings (descendants from the same parents), especially in blood-related marriages. This fact indicates a potential autosomal recessive form of inheritance (manifests exclusively in homozygotes who received one recessive gene from each parent).

When studying the skin of carriers of the disease, cells were recorded in which the ability to correct damage in DNA was impaired, as well as to reproduce genetically homogeneous fibroblasts and change the depleted dermis. As a result subcutaneous tissue tends to disappear without a trace.

Progeria is not inherited

It has also been recorded that the Hutchinson-Gilford syndrome being studied is related to pathologies in carrier cells. The latter are simply unable to fully free themselves from DNA compounds caused by chemical agents. When cells with the described syndrome were detected, experts determined that they were not capable of full division.

There are also suggestions that childhood progeria is an autosomal dominant mutation that occurs de novo, or without signs of inheritance. She was ranked among indirect signs development of the disease, the basis of which included measurements of telomeres (the ends of chromosomes) in the owners of the syndrome, their close relatives and donors. In this case, an autosomal recessive form of inheritance is also seen. There is a theory that the process provokes a violation of DNA repair (the ability of cells to correct chemical damage, as well as breaks in molecules).

Reasons for the formation of progeria in adults

Progeria in an adult organism is characterized by autosomal recessive inheritance with the mutation gene ATP-dependent helicase or WRN. There is a hypothesis that in the unifying chain there are failures between DNA repair and metabolic processes in connective tissue.

Since this form of the disease is extremely rare, one can only guess what type of inheritance is inherent in it. It is similar to Cockayne syndrome (a rare neurodegenerative disorder characterized by growth deficiency, disorders in the development of the central nervous system, premature aging and other symptoms) and manifests itself individual signs early aging.

Symptoms of early aging of the body

The symptoms of progeria manifest themselves in a complex manner. The disease can be recognized by early stage, since its signs are clearly expressed.

Symptoms of early aging disease in children

At birth, children who have the deadly progeria gene are indistinguishable from healthy babies. However, by the age of 1 year, certain symptoms of the disease manifest themselves. These include:

underweight, stunted growth;
lack of hair on the body, including on the face;
lack of subcutaneous fat reserves;
insufficient tone in the skin, causing it to sag and become wrinkled;
bluish skin tone;
increased pigmentation;
strongly visible veins in the head area;
disproportionate development of the bone tissues of the skull, small lower jaw, bulging eyes, prominent ear shells, hooked nose. A child with progeria typically has a “bird-like” grimace. It is the described list of peculiar characteristics that makes children look like older people;
delayed teething, which a short time lose their healthy appearance;
shrill and high-pitched voice;
pear-shaped chest, small collarbones, tight knee joints, as well as the elbows, which, due to insufficient mobility, force the patient to take the “rider” position;
protruding or convex yellow nails;
scleral-like formations or thickening on the skin of the buttocks, thighs and lower abdomen.

Symptoms of progeria in a child most often appear at 1 year of age.

When little patient, suffering from progeria, turns 5 years old, inexorable processes of the formation of atherosclerosis begin to occur in his body, in which the aorta, mesenteric, and also coronary arteries. Against the background of the described failures, heart murmurs and hypertrophy (a significant increase in the mass and volume of the organ) appear in the left ventricle. The cumulative impact of these serious violations in the body is the key reason for the low life expectancy of carriers of the syndrome. The fundamental factor that provokes an emergency fatal outcome Children with progeria are considered to have myocardial infarction or ischemic stroke.

Symptoms of early aging in adults

A progeria carrier begins to quickly lose weight, be stunted in growth, turn gray and soon go bald. The patient's skin becomes thin and loses its healthy tone. Under the surface of the epidermis are clearly visible blood vessels, as well as subcutaneous fat. With this disease, the muscles atrophy almost completely, as a result of which the legs and arms look excessively exhausted.

Progeria in adults occurs unexpectedly and develops quickly

In patients who have crossed the age limit of 30 years, both eyes are destroyed by cataracts (clouding of the lens), the voice becomes noticeably weaker, the skin over the bone tissue loses its softness, and then becomes covered with ulcerative lesions. Carriers of progeria syndrome are usually similar in appearance. They are distinguished:

small height;
moon-shaped face type;
"bird" nose;
thin lips;
a very prominent chin;
a strong, well-built body and dry, thin limbs, which are disfigured by generously manifested pigmentation.

The disease is unceremonious and interferes with the functioning of all body systems:

the activity of the sweat and sebaceous glands is disrupted;
distorted normal function of cardio-vascular system;
calcification occurs;
Osteoporosis appears (decreased density bone tissue) and erosive osteoarthritis (irreversible processes in the joints).

Unlike the child form, the adult form also has a detrimental effect on mental abilities.

Approximately 10% of patients by the age of 40 come into contact with such serious illnesses as sarcoma ( malignancy in tissues), breast cancer, as well as astrocytoma (brain tumor) and melanoma (skin cancer). Oncology is progressing based on high sugar in the blood and malfunctions of functions parathyroid glands. Key reasons Mortality in adults with progeria is most often due to cancer or cardiovascular abnormalities.

Diagnostics

The external signs of the disease are so obvious and vivid that the syndrome is diagnosed based on the clinical picture.

The disease can be detected even before the baby is born. This became possible thanks to the discovery of the progeria gene. However, since the disease is not transmitted through generations (it is a sporadic or single mutation), the likelihood that two children with this disease will be born within the same family a rare disease, is extremely small. After the progeria gene was discovered, detection of the syndrome became much faster and more accurate.

Changes at the gene level are now identifiable. Created special programs, or electronic diagnostic tests. At the moment, it is quite possible to prove and substantiate individual mutational formations in the gene, which subsequently lead to progeria.

Science is developing rapidly, and scientists are already working on the final scientific method for diagnosing progeria in children. The described development will contribute to even earlier, as well as accurate diagnosis. Today at medical institutions Children with this diagnosis are examined exclusively externally, and then tests and a blood sample are taken for testing.

If symptoms of progeria are detected, you must urgently seek advice from an endocrinologist and undergo a comprehensive examination.

Treatment of progeria

Up to this day effective method no cure for progeria has been found. The therapy is characterized by a symptomatic line, with the prevention of consequences and complications based on the progress of atherosclerosis, diabetes mellitus and ulcerative formations. For an anabolic effect (accelerating the process of cell renewal) it is prescribed growth hormone, which is designed to increase weight as well as body length in patients. The therapeutic course is carried out by several specialists at once, such as an endocrinologist, cardiologist, therapist, oncologist, and others, based on the symptoms prevailing at a particular moment.

In 2006, scientists from America recorded clear progress in the fight against progeria as an untreatable disease. The researchers introduced a farnesyltransferase inhibitor (a substance that suppresses or delays the course of physiological or physicochemical processes), which had previously been tested on cancer patients, into the culture of mutating fibroblasts. As a result of the procedure, the mutation cells acquired their usual shape. The carriers of the disease tolerated the created drug well, so there is hope that in the near future it will become possible to use the drug in practice. Thus, it will be possible to exclude progeria even in early age. The effectiveness of Lonafarnib (a farnesyltransferase inhibitor) lies in the increase in the amount of subcutaneous fat in total mass body, as well as bone mineralization. The result is to reduce the number of injuries to a minimum.

There is an opinion that they can help in curing the disease similar means, as in the fight against cancer. But these are only assumptions and hypotheses, not confirmed by facts.

Therapy for patients today comes down to:

providing ongoing continuity of care;
special diet;
cardiac care;
physical support.

For progeria, treatment is exclusively supportive in nature and is focused on correcting changes occurring in the patient’s tissues or organs. The methods used are not always effective. However, doctors are doing everything they can. Patients are under continuous supervision by medical professionals.

Only by monitoring the function of the cardiovascular system is it possible to timely diagnose the development of complications and prevent their progress. All treatment methods are focused around a single goal - to stop the disease and not give it a chance to worsen, as well as to alleviate general state carrier of the syndrome, as far as the potential of modern medicine allows.

Treatment may include the following:

use of Aspirin in a minimal dosage, which can reduce the risk of developing a heart attack or stroke;
the use of other medications that are prescribed to the patient privately based on the presenting symptoms and his well-being. For example, drugs from the statin group reduce the amount of cholesterol in the blood, and anticoagulants prevent the formation of blood clots. A hormone that can increase height and weight is often used;
the use of physical therapy or procedures designed to work out joints that are difficult to bend, thereby allowing the patient to maintain activity;
elimination of milk teeth. A peculiar feature of the disease contributes to the premature appearance of molars in children, while milk teeth must be removed on time.

Based on the fact that progeria is genetic or random in nature, then preventive measures there are none as such.

Treatment prognosis

The prognosis for carriers of progeria syndrome is unfavorable. Average indicators say that patients most often survive only up to 13 years, subsequently dying from hemorrhages or heart attacks, malignant neoplasms or atherosclerotic complications.

Progeria is incurable. The therapy is only in development. There is no definitive evidence of a cure yet. However, medicine is developing rapidly, so there is a high probability that patients with progeria will have a chance for normal and long life.

Infantile progeria was described independently by two doctors, J. Hutchinson (1886) and H. Guilford (1904). The disease is called Hutchinson-Gilford syndrome in their honor. For 125 years in medical practice More than a hundred cases of childhood disease have been described.

Signs of the disease appear in the first two to three years of a child’s life. Progeria begins unexpectedly: several large age spots. From this moment on, his growth slows down and his weight increases very slowly. The skin becomes thinner to the point where blood vessels can be seen through it. Compared to the body, the head looks very large; the face, on the contrary, is very small with small “bird-like features” and an underdeveloped chin.

The child’s weight does not exceed 20 kg, growth stops at 110−120 cm. As he “gets older,” he develops muscle atrophy, dental dystrophy, and gradually disappears hairline from the head. In addition, there are pathological changes skeleton and joints. The bones become brittle, the skin becomes very wrinkled, the body becomes hunched and twisted. There are problems with cardiovascular system. A violation occurs fat metabolism, atherosclerosis develops. Vision problems begin, and clouding of the lens is often observed. The mental development of a child corresponds to his physical age.

Childhood progeria affects one child out of 5-7 million newborns. In one year, the baby “lives” 6-8 years of life ordinary person. Very rarely children live to be 18-20 years old.

Causes of the disease

Before beginning of the XXI century special research There has been no research into the causes of progeria. There was an opinion in the medical community that no medicine could cure this incomprehensible and terrible disease.

However, science does not stand still. Medical scientists were interested in the fact that the rapid aging of children with progeria leads to the development of the same diseases that old people die from. To understand the mechanism of aging, researchers are closely studying the causes that cause progeria.

In 2004, scientists at Brunel University (London) discovered the LNMA gene, a mutation of which causes progeria. This gene encodes the lamin A protein, which is part of the cell nucleus envelope proteins. Damaged lamin A causes deformation of cell nuclei. This is fraught with the fact that there is a deficiency in the body of proteins necessary for building the connective tissues of the body.

The normal aging process is explained by the law of cell division. This law was discovered American biologist L. Hayflick in 1961. The main meaning of the law is that the cells of our body can only divide up to certain point, which is called the Hayflick limit. Due to pathological cell division in sick children, the Hayflick limit is significantly reduced, which means premature rapid aging of a young organism.

Currently, scientists are faced with new problem— it is necessary to fully understand how the mutated gene works. If they can solve this riddle, then medicine will have powerful tool, allowing to slow down the aging process of the human body.

Distribution of progeria

Currently, about 80 cases of childhood progeria have been reported on five continents. Interestingly, most of the sick children belong to the white race. Only 12-year-old Onthalametse Phalatse from South Africa is the first black child to be struck down by this terrible disease.

The girl's mother says that her daughter was born a normal baby. She was the first-born in the family, and her parents were happy when she was born. But the happiness did not last long: at three months the child developed an incomprehensible rash, after a year they began to experience age-related changes skin and nails, hair began to fall out. However, at the age of 6, as expected, the girl went to school. Now she looks like an 80-year-old woman, she is half the size of her peers.

Two years ago, Ontalametse and her mother were invited to the United States for examination. After this, the girl travels to the United States every year, where she continues to be monitored and undergoes experimental treatment. Ontalametse herself, an optimist by nature, states: “I call myself the first lady because I am the first black child with this disease... Do you know any other black child with similar disease? Unfortunately, doctors cannot say any comforting words to mother and daughter.

The longest-liver among the “progeria” children is a young man named Denny. IN early childhood his parents abandoned him, but they found him good people who adopted the boy. This year Denny celebrated his 20th anniversary. He looks like a little old gnome: withered body, hunchbacked back, sluggish, uncertain movements, teeth fallen out, bald head. Denny knows that he has few days left in this world. His parents take him to wheelchair. Denny managed to live a “relatively long life” only because he had great willpower, and his wonderful parents surrounded their son with care and love.

How can you survive, and what is being done to overcome this terrible disease?

Progeria translated from ancient Greek means old man. Is rare genetic disease, in which irreversible changes occur in the body, leading to premature aging. There is childhood progeria, called Hutchinson-Gilford syndrome, and adult progeria, known as Werner syndrome.

Mutations in the LMNA gene lead to childhood progeria syndrome. It is this gene that produces the protein lamin, which helps retain the cell nucleus. Scientists believe that a defective lamin protein leads to instability of cell nuclei, which contributes to early aging.

At birth, children with this syndrome appear healthy in appearance and physical appearance. The disease begins to manifest itself at the age of 1.5-2 years. This is expressed by loss of hair and weight, protruding veins are observed, and wrinkled skin is formed. In addition, negative processes are accompanied by complications more common to older people: stroke, cardiovascular diseases, osteoporosis, joint stiffness, generalized atherosclerosis.

One interesting point is observed with this disease. Despite their different ethnic backgrounds, children with this syndrome are externally similar to each other. The most common cause of progeria from which children die is atherosclerosis, and the age to which they live is 13 years. True, the age range ranges from 8 to 21 years.

Adult progeria, according to many years of observations, begins in adolescence, the range is from 15 to 20 years. Naturally, the disease also affects the life expectancy of patients, which is shortened to 40-50 years. Death occurs due to stroke, myocardial infarction, malignant tumors. The cause of the disease is still unknown and to this day occupies the minds of scientists around the world.

You should know that progeria is a genetic disease, not hereditary. It turns out that the parents are not carriers of this disease. Scientists suggest that a sporadic mutation occurs either in the sperm or in the egg even before the moment of conception. It is also worth noting that if parents have a child with CSGP, then the probability of having a second child of the same kind is small and amounts to 1 in 4-8 million. There are some progeria syndromes that are passed down through generations, but this is not the case with classic CSGP.

Before illness, both sexes (female and male) and all races exclusively are equal. The disease is quite rare and occurs in only one in 8 million children worldwide. Known on this moment 42 cases of this disease.

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It happens that at the age of 18 people become decrepit, and at 20-30 they already die

In October 2005, at a Moscow clinic, doctors performed the first operation on a patient suffering from premature aging syndrome. Progeria - very rare disease. Medical luminaries around the world claim that from the moment this disease “awakens” in the body, people live on average only 13 years.

According to statistics, approximately 1 in 4 million people are born with such a genetic defect. Progeria is divided into childhood progeria, called Hutchinson-Gilford syndrome, and adult progeria, called Werner syndrome. In both cases, the genetic mechanism breaks down and an unnatural depletion of all life support systems begins. With Hutchinson-Gilford syndrome there is a delay physical development children with the simultaneous appearance in the first months of life of signs of senile graying, baldness, and wrinkles. By the age of five, such a child suffers from all the ailments of old age: hearing loss, arthritis, atherosclerosis, and does not even live to see 13 years of age. With Werner's syndrome, young people begin to age rapidly at the age of 16-20 years, and by the age of 30-40 such patients die with all the symptoms of extreme old age.

There is no cure for progeria - using all scientific advances, you can only slow down the irreversible process.

Stolen youth

Cases of sudden aging are very prosaic: living in normal conditions At first, the child surprises those around him with his rapid development. At a young age, he looks like an adult, and then he begins to show all the signs of... approaching old age. In 1716 in English city The eighteen-year-old son of Earl William of Sheffield, who began to age at the age of thirteen, died in Nottingham. Young Sheffield looked much older than his father: gray hair, half-lost teeth, wrinkled skin. The unfortunate young man had the appearance of a man battered by life, he suffered greatly from this and accepted death as a deliverance from torment.

There are cases of this kind among representatives of royal families. The Hungarian King Ludwig II, at the age of nine, had already reached puberty and enjoyed having fun with the court girls. At fourteen, he acquired a thick, full beard and began to look at least 35 years old. A year later he got married, and on his sixteenth birthday his wife gave him a son. But at the age of eighteen, Ludwig turned completely gray, and two years later he died with all the signs of senile decrepitude. It is curious that neither the king’s son nor his further descendants inherited such a disease. Among the examples of the 19th century, one can highlight the story of a simple village girl, the Frenchwoman Louise Ravaillac. At the age of eight, Louise, fully formed as a woman, became pregnant by a local shepherd and gave birth completely healthy child. By her sixteenth birthday she already had three children and looked older than her mother; at 25 she turned into a decrepit old woman and, before reaching 26, died of old age.

Of no less interest are the fates of those who lived in the 20th century. Some of them were a little luckier than others. For example, Michael Sommers, a resident of the American city of San Bernardino, born in 1905, matured and aged early, and was able to live to the age of 31. At first, a super-fast entry into adult life he was even pleased. But when, at seventeen, Michael realized with horror that he was beginning to age, he began to make desperate attempts to stop this destructive process. But the doctors just shrugged their shoulders, unable to do anything to help. Sommers managed to slow down his decrepitude a little after he, having moved permanently to the village, began to spend a lot of time on fresh air. But still, by the age of 30, he turned into an old man, and a year later he was finished off by an ordinary flu. Among other similar phenomena, one can highlight the Englishwoman Barbara Dahlin, who died in 1982 at the age of 26. By the age of 20, having been married and given birth to two children, Barbara quickly and irreversibly aged. That is why her young husband left her, who did not want to live with the “old wreck.” At the age of 22, due to deteriorating health and the shocks she had suffered, the “old woman” became blind and until her death she moved by touch or accompanied by a guide dog, given to her by the authorities of her native Birmingham.

Paul Demongeau from the French city of Marseille is twenty-three years old. At the same time, he looks 60 and feels like an old man. However, he has not yet lost hope that a miracle will happen and a remedy will be found that will stop his rapid decrepitude. His brother in misfortune, a Sicilian from the city of Syracuse, Mario Termini, is not even 20 years old, but he looks much older than 30. The son of wealthy parents, Termini does not deny himself anything, meets with local beauties and leads a riotous lifestyle.

What do we have?

“Precocious” people also lived in our country. Even during the time of Ivan the Terrible, the son of the Mikhailov boyars, Vasily, died at the age of 19 as a decrepit old man. In 1968, at the age of 22, Nikolai Shorikov, a worker at one of the factories, died in Sverdlovsk. He began to age at the age of sixteen, which greatly puzzled the doctors. The luminaries of medicine just shrugged: “This can’t be!” Having become an old man at the age when everything is just beginning, Nikolai lost all interest in life and committed suicide by swallowing pills... And thirteen years later, the 28-year-old “old man” Sergei Efimov died in Leningrad. His youthful period ended at the age of eleven, and he began to noticeably age after twenty and died as a decrepit old man, having almost completely lost the ability to think sensibly a year before his death.

Genes are to blame for everything

Many scientists believe that the main cause of this disease is genetic mutation, leading to accumulation large quantity protein in cells. Psychics and magicians claim that there are special techniques for sending “damage” in order to make a person grow old.

By the way, this disease occurs not only in humans, but also in animals. They also have life cycles and periods sometimes follow the scenario of a year in three, or even ten years. Perhaps a solution to the problem will be found after many years of experiments on our smaller brothers.

As researchers from the University of California have established, a drug called farnesyltransferase inhibitor significantly reduces the rate of symptoms of premature aging in laboratory mice. Perhaps this medicine will be suitable for treating people.

Here is how Candidate of Biological Sciences Igor Bykov characterizes the symptoms of the disease in children: “Progeria occurs suddenly with the appearance of large pigment spots on the body. Then people begin to suffer from real senile illnesses. They develop heart disease, vascular disease, diabetes, hair and teeth fall out, and subcutaneous fat disappears. Bones become brittle, skin wrinkles, and bodies become hunched. The aging process in such patients proceeds approximately ten times faster than in healthy person. Evil is most likely rooted in genes. There is a hypothesis that they suddenly stop giving the command to cells to divide. And they quickly become unusable.”

Genes stop giving the cell the command to divide, seemingly due to the fact that the ends of the DNA in the chromosomes are shortened - the so-called telomeres, the length of which presumably measures the length of a human life. Similar processes are taking place in normal people, but much slower. But it is completely unclear as a result of what kind of disorder telomeres are shortened and aging begins to accelerate by at least 10 times. Now scientists are using enzymes to lengthen telomeres. There were even reports that American geneticists managed to extend the life of flies in this way. But we are still far from results that can be applied in practice. People cannot be helped even at the level of experiments. Fortunately, the disease is not inherited.

It is assumed that a malfunction in the genome occurs even during the period intrauterine development. So far, science cannot monitor and manage this failure: it can only state a fact, but perhaps in the near future gerontology will answer this question to the world.