Old age is a naturally occurring period of age development, The final stage ontogeny. Aging is an inevitable biological destructive process leading to a gradual decrease in the adaptive capabilities of the body; characterized by the development of the so-called age-related pathology and an increased likelihood of death.

The time of onset of old age is conditional. Men and women aged 55-60 to 75 years old are considered old, from 75 years old - old, from 90 years old - centenarians. It is assumed that the species life expectancy of a person is 92–95 years.

It is known that human aging in the vast majority of cases occurs according to the premature (accelerated) type. living conditions modern society, diseases that are widespread in the second half of life (atherosclerosis, ischemic, hypertension etc.), accelerating the aging process, lead to premature aging and death before reaching the species biological limit of life. Therefore, prevention, early detection and treatment of pathological processes is important point in a complex of measures aimed at the prevention of premature aging. At the same time, this problem continues to be actively discussed among scientists to this day; diametrically opposed views are expressed on this issue.

In modern gerontological literature, the terms "physiological" and "premature" aging are widely used, but the issue of these two types of aging has been discussed for almost a hundred years.

The literature contains numerous data on changes in the organs and systems of the body in the process of natural, physiological aging. The situation is completely different with the study of premature aging, which is the main, most common form of aging in people over the age of 40-50 years.

Premature aging is understood as any partial or general acceleration of the rate of aging, leading to the fact that a person is "ahead" of the average level of aging of his or her body. age group. At the same time, age-related changes occur earlier than in healthy people the appropriate age. In other words, with premature aging biological age a person is ahead of his calendar (passport).

Despite the complexity of identifying the syndrome of premature aging, the need to study it is due to clinical practice and the importance of clarifying the causes and mechanisms of premature aging, its main indicators and ways of therapeutic and prophylactic effects.

The WHO seminar on problems of gerontology, held in 1963, was devoted to the criteria of physiological and premature aging. in Kiev. In accordance with its resolution, physiological aging means a natural beginning and gradual development senile changes that are characteristic of this species and limit the body's ability to adapt to the environment. Premature aging is to be understood as any partial or more general acceleration in the rate of aging that results in an individual "ahead" of the average level of aging of the healthy group of people to which he belongs. It was emphasized that the physiological and premature aging due to the influence of both internal factors (including genetic ones) and factors external environment. Naturally, for each of these types of aging, the influence of these factors is completely different.

The complexity and controversy of a clear definition of the syndrome of premature aging is due to the fact that we still do not fully know the mechanisms, the sequence of processes that develop during physiological aging. In any case, it can be assumed that the factors leading to premature aging may be included in different stages development of physiological aging, modifying its mechanisms and manifestations, affecting the pace and nature of the development of senile changes.

The main difficulties arise when trying to determine the essence of premature aging, its mechanisms and ways of development. Two different approaches are possible here. In the first approach, the early development of signs of old age is considered regardless of the cause that caused it. Indeed, clinicians are well aware of various pathological processes leading to fairly rapid decrepitude, early onset external signs, typical for older people, functional and structural changes in organs and systems that accompany the disease and aggravate it. Suffice it to recall how much older than their years, even outwardly, patients with coronary heart disease, COPD, peptic ulcer, diabetes etc. Emotional overstrain, mental stress, irradiation radioactive substances and many other factors are often the cause of premature and rapid development senile changes.

Proponents of a different point of view believe that the emergence early signs old age (accelerated aging) is not associated with any specific disease or external causes, but due to peculiar endocrine-metabolic shifts. However, such an understanding is unlikely to find wide support, since what cannot be detected at present due to limited diagnostic capabilities may later turn out to be quite provable.

It seems to us more productive and having a specific practical significance the study of premature aging in the aspect of those diseases or pathological conditions that modify and complicate the aging process, lead to early and rapid decrepitude and disability.

A characteristic feature of premature aging is a more pronounced limitation of the adaptive capabilities of the organism, which, however, decrease during normal, physiological aging. This, ultimately, can lead to a sharp reduction in the reserve capacity of the functioning of the organs and systems of the body. With premature aging, some structural and functional age-related changes not only accelerate, but sometimes acquire a character opposite to that observed during physiological aging.

From huge number risk factors for premature aging can be identified as endogenous (diseases, heredity) and exogenous, environmental factors (physical inactivity, mental stress, overnutrition, bad habits, pollution environment and etc.).

The process of premature modification of cells due to exposure to pathological, genetic or external factors called the disease of premature aging. Pathology is poorly understood, the exact causes of the development of this condition have not been identified. There are a number of external and internal factors that provoke the disease. According to statistics, rapid aging syndrome is extremely rare (1 sick person per 4 million people).

What causes early aging

Premature aging syndrome is a condition where age physiological changes come on a person much earlier than the due date. Aging is a natural process, characterized by a gradual decrease in the entropy (life processes) of all body systems. In addition, changes in various qualities of cells occur: the mechanism of protein synthesis is disrupted and errors gradually accumulate when copying DNA.

Among the first signs of premature aging, there are changes in the skin (deep wrinkles appear, the skin becomes thinner, begins to sag) due to a violation of the synthesis of elastane, collagen. Changes in the functioning of the brain are noted: due to the fact that functional cells(neurons) are destroyed, human cognitive abilities (for example, memory) deteriorate significantly. In addition, Werner's syndrome is characterized by the following violations work of body systems:

• Cardiovascular: destruction of blood vessels occurs, volume decreases cardiac output, the heart muscle thickens, loses its elasticity and ability to regenerate, atherosclerosis develops.
• Immune: Decreased production of antibodies.
• Musculoskeletal system: rapid muscle atrophy, development of osteoporosis, arthritis.
• Sense organs: presbyopia develops ( age decline visual acuity), hearing loss, cataracts, complete hearing loss.
• reproductive system: in women comes early menopause men suffer from erectile dysfunction increases the likelihood of developing malignant neoplasms.

Causes

Many pathological or physiological factors can accelerate the aging process. Non-disease related causes include:

• genetic predisposition;
• environmental factors;
• Lifestyle;
• climate.

Premature aging can be caused early manifestation systemic diseases. In this case, the syndrome manifests itself, as a rule, in early childhood, adolescence or young age. Among pathological causes, leading to early aging, there are:

• Alzheimer's disease;
• diabetes;
• osteoporosis, osteoarthritis;
• Parkinson's disease;
• cardiovascular pathologies;
• hypothyroidism;
• Down syndrome;
• trichothiodystrophy;
• dermopathy.

What is premature aging disease

Pathological process, which is provoked by premature aging and is characterized by a change in the condition of the skin, a violation of the functioning of organs and systems, is called progeria. mental development it is rated as satisfactory. There are two types of the disease: children (Hutchinson-Gilford syndrome) and adults (Werner syndrome). Presumably, the pathology in adults has an autosomal recessive type of inheritance, while in children it occurs spontaneously.

Causes

It is known that the disease of rapid aging is a pathology of genetic origin and occurs as a result of a mutation of the LMNA gene, which encodes the synthesis of lamins, proteins that are part of the shell of the cell nucleus. Genetic disorders provoke instability of cellular structures, which leads to quick start aging mechanisms. A large number of proteins are deposited (accumulates) in cells that lose the ability to divide, renew themselves and die prematurely.

In addition, the mutation provokes the production of a truncated, unstable progerin protein, which is rapidly degraded. It does not penetrate into the plate of the shell of the nucleus, located under the membrane, as a result of which it collapses. This process is key in the pathogenesis of progeria. The disease occurs in children of the same parents (siblings) or in the offspring of consanguineous marriages. In the study of cells of people suffering from such an ailment, they found gross violations DNA repair in cells and fibroblast synthesis. The childhood form of progeria is considered congenital.

Symptoms

Clinical picture in the disease of premature aging, it manifests itself over time. With Hutchinson-Gilford syndrome, the first symptoms of pathology appear at 2-3 years of age, and with Werner syndrome, as a rule, within six months after puberty. The disease captures the entire body at once, the functioning of almost all vital organs is disrupted.

In childhood

For progeria occurring in childhood characterized by a sharp slowdown in the growth of the child, atrophy of the dermis, subcutaneous tissue loss of skin elasticity. The epidermis becomes thinner, becomes dry and wrinkled, scleroderma-like lesions and hyperpigmentation are noted on the body. Large and small veins shine through the pale and thinned skin. In addition, it is noted the following signs Hutchinson-Gilford syndrome:

• atrophy skeletal muscle;
• fragility of teeth;
• fragility of hair, nails;
• pathological changes musculoskeletal system, myocardium;
• underdevelopment of the genital organs;
• violations fat metabolism;
• cataract;
• atherosclerosis.

Due to the fact that the disease affects all cells of the body and changes their qualitative structure, all human tissues and organs change very much. For people who suffer from progeria, some specific features of appearance are characteristic:

• big head with prominent large frontal tubercles that protrude above a small "bird" face;
• lower jaw severely underdeveloped;
• beak nose;
• secondary sexual characteristics are absent;
• height about 90-130 cm;
• limbs are thin, short.

In adults

First clinical symptoms diseases in adults appear by the age of 14-18. Before puberty, no signs of premature aging disease are observed. Patients begin to lag far behind in physical development, gray and bald. The skin quickly becomes thinner, acquires pallor and pigmented heels. The limbs look very thin due to atrophic changes in the subcutaneous tissue and muscles. By the age of 30, patients develop the following signs of the disease.

The more chromosomal DNA is in an unpacked, active form, the sooner and faster the cell begins to age.

All people age differently, some faster, some slower, but, taken as a whole, signs of old age appear in everyone at about the same age. With one exception: in the event that a person is sick with progeria, he begins to age extremely early. This disease has two variants, children's and adults, children's is called Hutchinson-Gilford syndrome, adult - Werner's syndrome. Children with progeria suffer from diseases characteristic of old age: thinning and wrinkling of the skin, baldness, cardiovascular diseases, lipid metabolism disorders, atherosclerosis, joint problems, etc. Their growth slows down sharply and a characteristic appearance develops: a large head, a small pointed face, an underdeveloped lower jaw. On average, patients with childhood progeria live no longer than 12-13 years.

A normal child and a child of the same age with progeria. (Photo by Ian Tomey/Flickr.com.)

Normal stem cells (left) and cells with premature aging syndrome (right), one of the characteristic features of which is an increase in size. (Photo by Salk Institute for Biological Studies.)

Photo © Andrey Kekälyainen / Lori Photobank.

People with adult progeria live longer, but they also experience age-related changes much earlier than usual - in their 20s, hair begins to gray and fall out, by the age of 30 they develop cataracts, osteoporosis, and other diseases, such as diabetes, and usually a person with Werner's syndrome does not live past 60 years of age. It is known that, at least in the case severe form cells undergo many of the same molecular changes as normal aging, so if we can find a way to slow down progeria, it might give us a tool against aging in general.

The secrets of the disease could be understood by observing the stem cells that were obtained from sick people. Some time ago, researchers were able to turn the skin cells of children with Hutchinson-Gilford syndrome into an analogue of embryonic stem cells, the so-called induced pluripotent stem cells. Further, it was possible to experiment with them, finding out what was wrong in the stem processes in patients with progeria. But when they tried to do the same with the cells of patients with Werner's syndrome, nothing came of it - their cells were too damaged by the disease to withstand a return to the stem, undifferentiated state. Then Juan Carlos Izpisua Belmonte ( Juan Carlos Izpisua Belmonte) along with colleagues from the Chinese Academy of Science and Peking University took a different path - they modeled progeria in initially healthy cells.

It is known that Werner's syndrome is accompanied by mutations in the WRN gene, which is involved in the processes of DNA copying and repair. And so, in order to create a model of the disease, the researchers simply broke this gene in stem cells from a human embryo. Embryonic cells in the course of development turn into more specialized varieties that can later give rise to one or another tissue - for example, into mesenchymal stem cells, the "ancestors" of adipose tissue, cartilage and bones. In an article in Science the authors write that when stem cells with a non-functioning WRN gene turned into mesenchymal ones, they immediately began to age dramatically: a lot of damage accumulated in their DNA, they stopped dividing, and, finally, their telomeres were greatly shortened. This is the name of the ends of chromosomes, which, when copying DNA, protect genes from damage associated with the peculiarities of the work of the protein copier. Telomeres shorten with each cell division, and therefore they are considered something like a molecular clock that measures the lifespan.

However, cells with Werner's syndrome had another feature that most of all attracted the attention of the authors of the work. It is known that DNA in the cell nucleus is in complex with proteins. Some of them perform some ongoing work on certain genes (for example, they synthesize RNA), while others play a structural role, maintaining rather large fragments of chromosomes in a packed state. The packaged, structured portion of DNA is called heterochromatin. And it turned out that in sick cells there is very little heterochromatin - in other words, DNA with Werner's syndrome comes to a free, "disheveled" state.

The same can be observed in normal aging: when the state of chromosomes was compared in several people different ages, then we saw that older man, the worse DNA is packed in its nuclei. Obviously, with progeria, the same process occurs faster and begins earlier - perhaps already on early stages individual development. Why can a disordered, unpacked state of chromosomes lead to such consequences? If a gene is in a heterochromatic form, this means that it is inactive, turned off, is in a dormant state. If the packaging weakens, then our genes will start to turn on, which should be silent. Just such unnecessary activity can collectively lead to aging. On the other hand, it is known that in a heterochromatic, sealed form there are mobile genetic elements that jump from place to place in DNA, thereby causing unwanted mutations.

Whether the general unpacking and disorder in DNA really entails all those changes that are characteristic of aging cells, and whether this happens in all cases of progeria, both childhood and adult, will be shown by further experiments. But, if this is indeed the case, biologists could focus on DNA packaging as a potential target for drugs that could help delay aging, both premature and normal.

Progeria - genetic disease, in which premature, rapid aging of the body occurs: skin, internal organs and systems. The disease has two forms: childhood (Hatchinson-Gilford syndrome) and adult (Werner syndrome). It should be noted that most often occurs in boys. Girls get sick much less often. The disease is rare. To date, only eighty cases of progeria are known worldwide.

A genetic failure that has occurred in the body accelerates the aging process by about 8-10 times. A child with such a disease, when he turns 8, looks 80. And not only externally. The condition of his internal organs also corresponds to his age. old age. Therefore, such children live for a very short time, about 13 - 20 years.

Today on www.site we will talk in more detail about premature aging of the human body - this is a disease of progeria, the symptoms, causes and treatment of which will essentially interest us further ... Let's start with the causes of this pathology:

Why does progeria disease occur, what are the causes leading to it?

The disease is caused by a genetic mutation in lamin A (LMNA). This is a gene that is directly involved in the process of cell division. Its mutation causes a failure in the genetic system, which deprives the cells of their resistance, starts the process of rapid aging in the body.

Note that unlike many other genetic diseases, progeria is not hereditary, it is not transmitted from parents to children. The mechanism of sudden genetic mutation scientists have not yet studied.

Premature aging symptoms:

In children:

Immediately after birth, the baby looks completely normal. The manifestations of the disease begin closer to the age of 2, when parents notice that the child has stopped developing. Growth retardation has been observed since 9 months. The baby is not gaining weight well, the skin loses elasticity, looks aging, keratinized areas appear on it. The joints lose their elasticity, the subcutaneous adipose tissue. These children often have hip dislocations.

A characteristic appearance takes the shape of the head and face of the child. The head becomes much more face, the lower jaw is small, smaller than the upper. On the scalp, eyelids, veins are clearly visible. Eyelashes fall out, eyebrows thin out, hair falls out intensely. The child's milk teeth do not grow well, they are noted irregular shape. Teeth that have grown in place of milk teeth begin to fall out.

When a child reaches three years old, his growth stops completely, it is noted. mental retardation. The nose takes on a beak-like shape, the skin becomes thinner. Skin undergo typical aging changes.

With the further development of the disease, the elasticity of the arteries is disturbed, atherosclerosis develops, and cardiovascular diseases may have a stroke.

Progeria in adults:

The disease in adults begins to develop suddenly in adolescence(14-18 years old). It all starts with unreasonable weight loss, growth stops. A characteristic sign of the onset of the disease is early graying, increased hair loss, and baldness.

Thinning, dryness of the skin is observed, it becomes pale, acquires an unhealthy shade. blood vessels, rapidly lost subcutaneous body fat limbs, why hands and the patient's legs look very thin.

After 30 years of life, the patient's eyes are affected by cataracts. His voice becomes weak, the skin becomes coarse, ulcerated, there is a violation of the function of sweat, sebaceous glands. The patient's body is deficient in calcium, which causes the development of osteoporosis, erosive osteoarthritis, diseases develop of cardio-vascular system, intellectual abilities decrease.

Premature aging of the human body is also manifested by other characteristic symptoms: short stature, round, moon-shaped face, nose like a bird's beak, thin, narrow lips. TO characteristics also includes a thin chin, sharply protruding forward, a dense, short body and thin, dry limbs, abundantly covered with pigmentation.

A large number of patients, by about 40 years, fall ill oncological diseases, diabetes mellitus. They are diagnosed with dysfunction parathyroid glands, there are severe cardiovascular pathologies. It is these serious diseases that cause early death patients with progeria. Which is unlikely to suit anyone ... Therefore, let's talk about how progeria is corrected, what treatment will help in improving well-being and slowing down the processes that have begun.

treatment for progeria

Modern medicine does not yet have methods for treating and preventing this genetic disease. The help of doctors is to slow down its progression, reduce, minimize symptoms.

For example, a patient is prescribed a daily intake of small doses of aspirin, which helps to reduce the risk of heart attacks and prevent stroke.

Use drugs from the group of statins, which reduce cholesterol levels.

Use drugs - anticoagulants, which reduce the risk of blood clots. Also, during therapy, growth hormone is used, which helps the patient's body to restore weight gain, promotes normal growth.

Physiotherapeutic methods are used to help restore the elasticity of the joints, allowing the patient not to lose physical activity. These techniques are very important, in particular for young patients.

In addition, in children with progeria, milk teeth are removed. With this disease, adult teeth erupt very early, while milk teeth deteriorate rapidly. Therefore, they need to be removed in a timely manner.

Progeria treatment requires individual approach to each patient, depending on his condition and age. Currently underway clinical researches drugs created by scientists to treat this genetic disease. Perhaps, effective therapeutic methods will soon appear. Be healthy!

Progeria(Greek progērōs prematurely aged) — pathological condition, characterized by a complex of changes in the skin, internal organs, due to premature aging of the body. The main forms are children's progeria (Hutchinson-Gilford syndrome) and adult progeria (Werner's syndrome).

Childhood progeria is very rare. Etiology and pathogenesis are not known. In most cases, it occurs sporadically, in several families it has been registered with siblings, incl. from consanguineous marriages, which indicates the possibility of an autosomal recessive type of inheritance.

In the skin cells of patients, violations of DNA repair and fibroblast cloning were found, as well as atrophic changes epidermis and dermis, disappearance of subcutaneous tissue. Although children's P. may be congenital, in most patients clinical signs usually appear in the 2nd or 3rd year of life.

The growth of the child sharply slows down, atrophic changes in the dermis, subcutaneous tissue, especially on the face and limbs, are noted. The skin becomes thinner, becomes dry, wrinkled, there may be scleroderma-like lesions on the body, areas of hyperpigmentation. Veins show through the thinned skin. Appearance patient: a large head, frontal tubercles protrude over a small pointed ("bird") face with a beak-shaped nose, the lower jaw is underdeveloped.

Muscle atrophy, dystrophic processes in teeth, hair and nails are also observed; there are changes in the osteoarticular apparatus, myocardium, hypoplasia of the genital organs, impaired fat metabolism, clouding of the lens, atherosclerosis.

Lena ages five years in a year

Yesterday, in a Moscow clinic, doctors performed the first operation on a patient suffering from premature aging syndrome.

At first, my earlobes began to sag in a strange way. Then I noticed amazingly deep wrinkles between the eyebrows, - says the 23-year-old girl.

At the first glance at Lena Melnikova, you even begin to doubt. Well, how is this a cunning bored 40 - 50-year-old lady who desired wide fame and plastic surgery from the best surgeons?! Unfortunately, this has already happened.

This is what she looks like now at 23.

Asking Lena about her personal life doesn’t even turn her tongue ... Although the girl smiles courageously:

Everything is fine.

Lena has almost no chance. Diagnosis: "premature aging syndrome" ("progeria"). medical luminaries around the world claim that people on average live only 13 years from the moment of the disease. And no one knows how to restore youth or at least calm old age ...

Terrible symptoms began to appear in Lena five years ago. First, the face aged, and then the skin of the whole body. Elena then studied at the 1st year of the Mari Polytechnic Institute.

You know, how insulting it was... Guys come up to meet my girlfriend and treat me emphatically politely, they take me for my mother. Almost asked permission to meet with the "daughter".

After graduating from the Mari Polytechnic University, the girl decided on plastic surgery. But banal circular lift facial skin did not help. Only left scars on the neck and temples. The mysterious process of aging of the organism continued. Local doctors could advise Elena only one thing - to take vitamins and be constantly monitored.

The girl - by the way, a certified engineer-architect - did not despair and went to Moscow. Melnikova became interested in an expensive metropolitan clinic plastic surgery Beauty Plaza. Its experts decided to help the provincial woman in trouble. And completely free.

We decided to try. If it is generally recognized that nothing can be done, then you should at least try, - said the leading surgeon of the clinic, Doctor of Medical Sciences Professor Alexander TEPLYASHIN, on the eve of the operation. - Although it is unsafe for Elena to operate, because the disease could also affect the state of internal organs.

She is so young! She needs to live normally, communicate with young people. First, we will make a face, and then we will begin to fight the disease at the genetic level, - Professor Teplyashin is determined.

“I really believe the professor,” Elena Melnikova persistently convinces us. It looks like she's also convincing herself.

Elena arrived at the clinic yesterday morning. She was being prepared for surgery. Allocated a separate room, where she waited. So far, Professor Teplyashin is also preparing for his very difficult work. A quarter of an hour before the operation, Elena is calm.

I'm not afraid of anything, - she repeats and repeats everything. And in the end it still sobs. Some time ago, the girl was seriously thinking about ending her life.

The time has come for the operation. Lena gets up and, looking straight ahead, walks with a deliberately firm gait into the bowels of the clinic. Suddenly, she stops for a minute and turns clearly more to herself than to others: “I was very afraid of this first operation, and now I have a second one. And I have no choice. My last hope". - And resolutely steps to the anesthesiologist.

The doctors of the clinic allowed the photographer into the holy of holies - the operating room aesthetic surgery. The first stage of the operation is the breast. The doctor cuts the skin on the chest and prepares a special bio-implant. The composition is one of the secrets of the clinic. The main thing - no alien silicone. Like dough, Professor Teplyashin vigorously kneads the implant so that the pliable material almost seeps between his fingers. And finally puts in the body. The second and main stage is the face. And the first difficulty here is to eliminate the scars and imperfections of the previous plastic surgery. The sight is not for the faint of heart. But everything seems to be going well...

After Lena Melnikova undergoes a special rehabilitation course at the clinic, geneticists and cell biologists will develop an individual biotechnological treatment program especially for her, which should end with an injection of stem cells. These cells are supposed to expel old age from a young body...

Once upon a time, a beautiful and smart 18-year-old student Melnikova had many fans. But when the disease began to develop, there was only one who really loves. The girl does not name him, but she is sure that he is very worried and is waiting for her in Yoshkar-Ola. In the meantime, in Moscow, the unemployed engineer-architect Melnikova lives with her brother.

For the first time, the syndrome of premature aging was discussed 100 years ago. And not surprisingly, such cases occur once in 4-8 million babies. Progeria (from the Greek pro - earlier, gerontos - old man) is an extremely rare genetic disease that accelerates the aging process by about 8-10 times.

Simply put, a child ages 10-15 years in one year. An eight-year-old looks 80 years old - with dry, wrinkled skin, a bald head ... These children usually die at the age of 13-14 after several heart attacks and strokes against the background of progressive atherosclerosis, cataracts, glaucoma, total loss teeth, etc. And only a few live to 20 years or longer.

Now only 42 cases of people with progeria are known in the world ... Of these, 14 people live in the United States, 5 in Russia, the rest in Europe ...

Among the features of such patients are dwarf growth, 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 the person as if a bird's features. Due to the loss of subcutaneous fat, all vessels are visible. The voice is usually high. Mental development age appropriate. And all these sick children are strikingly similar to each other.

12 year old Seth Cook looks like an 80 year old man. He has no hair, but he has a full range of diseases that affect older people. Therefore, every day the boy takes aspirin and other drugs that thin the blood. With a height of 3 feet (a little over a meter), Seth weighs 25 pounds (11.3 kg).

Oury Barnett was born on April 16, 1996. Already at the age of five, poor Ouri began ischemic disease hearts. Attacks followed one after another. The kid often ended up in the hospital, but he had to be treated with the means that are usually prescribed for older people.

Ouri looked like a stroke survivor: his legs were weak and he began to stumble like a decrepit old man. His eyes faded upper lip did not move, saliva flowed, speech became illegible.

Ouri's mother did a lot to convey to people her experience and her observations of the unfortunate child. From the age of three, the baby was taken to the shooting of television programs and scientific conferences. The only condition that the mother set for sensational journalists was that they should not write that the baby was dying of progeria.

The most famous case of progeria described in the Russian press is the story of Alvydas Gudelauskas, who suddenly began to age at the age of 20. Literally in a matter of months, Alvidas turned into a 60-year-old man before our eyes. And only after plastic surgery did he begin to look like a mature man. In the photo on the left - this is how he looked before the operation, on the right - after. Now Alvydas is only 32 years old.

Until recently, doctors could not determine the cause of the disease. And only recently, American researchers discovered that only a single mutation is the cause of "childish old age" or Hutchinson-Gilford progeria.

According to director National Institute genome study by Francis Collins, who led the study, this disease is not hereditary. A point mutation - when only one nucleotide is changed in a DNA molecule - occurs anew in each patient. People suffering from progeria die mainly from precisely those ailments that are characteristic of old age. A mutated form of the LMNA gene has now been found to cause progeria.

Seven year old man and his family

Khan children. Rehena, Ali Hussein and Ikramul suffer from rare disease. He is only seven years old, and he is already going bald. This is the most noticeable of the many symptoms of the disease that Ali Hussein Khan suffers from. He is still a boy, but he is already in middle age. This progeria is extremely rare disease, due to which Ali's body ages prematurely.

Neither he nor his sister and brother - 19-year-old Rehena and 17-year-old Ikramul - have practically no chance of living to 25.

This disease greatly accelerates the development of children. However, it also causes other problems: in their mouth, for example, a second row of teeth appears, and the skin becomes very pale, almost transparent.

These children get sick ordinary people suffer in old age. Last year, their sister Ravena, who also had progreria, died of pneumonia. She was 16.

As soon as Ali Hussein begins to speak, it becomes clear that he is seized with childish enthusiasm and absorbed in hopes that are not characteristic of an adult.

“I would like to be an actor, drive cars and planes, be an action hero,” he says. “Well, then I would like to become a doctor, because doctors check me all the time, and I would like to check myself, and therefore I wanted someday I would like to be a doctor."

Hana is unique in this sense: this is the only case known to science when more than one family member suffers from progreria. And thanks to this family, scientists were able to make a real breakthrough in understanding the nature of the disease.

Scientists led by pediatrician Chandan Chattopadhyaya observed the Khans for two years and concluded that the disease is hereditary and recessive. This means that her gene can be in both parents. In this case, Hana's husband and wife are cousins ​​to each other. Neither of them has progreria, nor do their other two children, 14-year-old Sangita and two-year-old Gulavsa.

V last years looking after the family Charity organization from Calcutta. The head of the Bisul Khan family says that life has treated him and his wife Rajia cruelly. Both of them are natives of one of the villages Indian state Bihar. The locals called their children aliens, and as a result they had to grow up in complete isolation.

“When we lived there, in Bihar, every evening we sat in a room, unable to sleep, because one of the children was tormented by something, then the other,” recalls Khan. “And we thought, me and my wife, we sat down side by side and thought: how can we continue to live? We even thought about putting an end to all this in one fell swoop ... "

“But now the children live,” says the father. “They are energetic, they are happy, they live normal life as much as possible, of course."

For the past two years, Khanami has been looked after by Sekhar Chattopadhyay, head of the S-bi Devi Charitable House in Calcutta. Now they live in this city, although their the exact address kept secret.

The charitable organization helped my father find a job as a security guard, but his salary is low, so they are also helped financially. But no less important than money are those normal human contacts that children have acquired with the help of a charitable organization.

"We support them and we've become friends," says Chattopadhyay, tossing Ali Hussein on his lap.

Thanks to his support, the Khans say they now live much more full life than before. They smile when they talk about their interests and hobbies.

Rehena says she loves Indian films, especially passionate love songs. When I ask if she sings herself, she says that she is shy, but it is still clear that she wants to demonstrate her abilities, and, having received approval, she agrees to try.

"I love loving you and when I don't see you, I can't wait until we meet again," she sings in Hindi.

According to various sources