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Proteus syndrome is not for the faint of heart. The Elephant Man, features and treatment of Proteus syndrome. Treatment of Proteus syndrome

Introduction

There were seven miracles: the Great Pyramid of Giza, the Mausoleum at Halicarnassus, the Colossus of Rhodes, Alexandrian lighthouse, Temple of Artemis in Ephesus, statue of Zeus in Olympia, Hanging Gardens of Babylon.

Their number was determined by the magic of the number seven, the limited capabilities of human memory, the limits of the ancient world and, most importantly, the stability of traditions. When, around the third century BC, someone proclaimed this particular seven-flowered pattern as the standard of miracles, part of humanity living around the Mediterranean Sea submitted to authority, and only a few local patriots, without challenging the principle itself, tried to make amendments in particular.

A thousand years after the fall of Rome, when people again had a renewed interest in what was happening outside their small world, the wonders of the world were remembered, and the power of ancient authority was such that the mentioned seven wonders were already perceived as an unshakable whole.

Only one of the miracles has survived to this day, paradoxically, the oldest - the Great Pyramid of Giza.

Great Pyramid of Giza

This grandiose Egyptian pyramid is the oldest of the Seven Wonders of Antiquity. In addition, this is the only miracle that has survived to this day. At the time of its creation, the Great Pyramid was the tallest structure in the world. And she held this record, apparently, for almost 4000 years.

Royal tomb

The Great Pyramid was built as the tomb of Khufu, known to the Greeks as Cheops. He was one of pharaohs, or kings of ancient Egypt, and his tomb was completed in 2580 BC. Later, two more pyramids were built at Giza, for Khufu's son and grandson, as well as smaller pyramids for their queens. Khufu's pyramid, the furthest one in the picture, is the largest. His son's pyramid is in the middle and looks higher because it stands on a higher place.

Construction of the pyramids

The pyramids stand on the ancient cemetery in Giza, on the opposite bank of the Nile River from Cairo, the capital of modern Egypt. Some archaeologists believe that it may have taken 100,000 people 20 years to build the Great Pyramid. It was created from more than 2 million stone blocks, each of which weighed at least 2.5 tons. Workers pulled them into place using ramps, pulleys and levers, and then pushed them together without mortar.

Shiny limestone

When the main structure was completed, it resembled a series of steps. They were then covered with blocks of white limestone with a polished, shiny surface. The blocks were so tightly fitted to each other that it was impossible to insert even a knife blade between them from the outside. Upon completion of the work, the Great Pyramid rose 147 meters. Now its top has collapsed, in addition, at present, only the pyramid of Khufu’s son has retained its limestone cladding at its very top. The base side of the Great Pyramid reaches 230 meters. It occupies more area than nine football fields.

Pharaoh's funeral

The ancient Egyptians believed that when a person died, their body should be preserved so that the spirit could continue to live after death. They removed the internal organs, filled the body with salts and wrapped it in linen shrouds. So the body turned into a mummy. The mummy was then buried along with clothing, food, jewelry and other items useful for the afterlife. Khufu's mummified body was placed in a burial chamber at the heart of his pyramid

The Pyramids of Giza have long been recognized as one of the Seven Wonders of the World. Colossal structures keep centuries-old secrets. Under the giant stone massif secrets of thousands of years are kept, which are still incomprehensible to our consciousness. These are the greatest monuments of world history.

The three main pyramids - Khufu (Pyramid of Cheops), Khafri (Pyramid of Khafre) and Menkaure (Pyramid of Mikerin) are the best preserved and have become famous throughout the world. The majestic tombs of the ancient pharaohs inspire respect. Egypt is the country of the pyramids. A total of 118 structures were found, of which most of lie in ruins. Some have survived a little better, and thanks to them we learn more and more historical details of the kingdom Ancient Egypt.

The word "pyramid" comes from the ancient Greek "pyramis" - fire, since the Greeks believed that the pyramid was shaped like a tongue of flame. Even in geometry textbooks of the 16th century, the pyramid was called a “fiery body.” There are versions that the prototype of the pyramid is a mountain of wheat. The ancient Egyptians had a funeral cake that had a pyramidal shape, and a number of scientists refer to the fact that the pyramids, being a symbol of death, could be identified with this cake. The Egyptians themselves called the pyramids “purama”.

Ancient pyramids are unique in that they have an ideal geometric shape. How the architects of the IV dynasty of Egyptian pharaohs (2613-2439 BC) achieved this is still unclear. The angle of the walls with respect to the horizon is 53°, and the edges are ideally evenly oriented in relation to the cardinal points.

The Cheops pyramid is the largest. Its original height was 146 meters. However, they began to build on a 9-meter rock, which is now located at its base. Over time, the facing plates of the pyramid crumbled (this happened as a result of a strong earthquake) and the “growth” of the pyramid decreased to 138 meters. The length of each side is exactly 230 meters. According to mathematicians, the Cheops pyramid consists of approximately 2.5 million stone blocks, each of which weighs 2.5 tons. Another mystery for the builders is how they managed to achieve such a perfect fit for each block. The incredible accuracy of measurement reaches the point that the error in the dimensions of the side of the pyramid is thousandths of a centimeter. Even modern technologies This is not possible. Unfortunately, the facing slabs, which were polished pieces of fine-grained sandstone, have not reached us.

Initially, the entrance to the pyramid was on the north side, at the level of the thirteenth step. However, over time it was walled up. Nowadays, in order to get inside, they use the hole made by ancient looters.

In the minds of many Egyptologists, a pyramid is a monolith that contains several large chambers and an extensive system of corridors. But this opinion is refuted by many scientists, who quite reasonably assume the presence of many other rooms, since only 1% of the entire structure is available for research. In addition, the Egyptian authorities themselves are not in the mood to reveal all the secrets and mysteries of the great buildings of antiquity. All finds pass first of all through their hands, and only then the world community becomes aware of them.

According to Herodotus, more than 100 thousand slaves were collected during the construction of the pyramids. However, after conducting more detailed historical research, modern Egyptologists tend to assume that the people who participated in the construction of the structure simply served a kind of “labor service” to the sovereign. This conclusion was made after a detailed study of the camp in which the builders lived. It was located a few kilometers from Giza and had several bakeries, a smokehouse and even a brewery.

Massive burials of cattle bones were discovered in the surrounding area, which suggests that the builders did not starve, they were cared for and even monitored their health. The skeletons found indicate that almost every worker during construction received one or another work injury, which was well healed. This speaks of high level medicine, which existed in Egypt even then. There is an assumption that groups of builders even waged some competition among themselves.

The mechanism of construction of the pyramids remains a mystery to this day. The most common version was about systems of blocks and counterweights, with the help of which huge pieces of stone were lifted to the top. It has been proven, however, that such a system really existed, but it began to work after the construction of the main structure, when the facing slabs were delivered to the top.

There is another version that the builders created an embankment along which they raised the blocks to the top. However, the version does not stand up to criticism. Indeed, in order for the embankment to be used effectively, its angle of inclination should be no more than 7 degrees. Consequently, the embankment itself should have stretched for two to three kilometers. It would take several years to build such a “ramp”. And then it also needed to be removed.

The French architect Jean-Pierre Houdin suggested that inside the pyramid itself there was a spiral-shaped ramp along which the blocks were pushed upward. An ultrasound examination of the structure showed that there are indeed voids inside. Perhaps the internal ramp still exists today. But in order to find it, you will need to drill through the wall of the structure, which, naturally, no one will allow the architect to do.

In 2006, a number of scientists, after discovering human hair in the thickness of one of the blocks, leaned towards the version that the stone blocks were man-made. Perhaps this is an ancient type of concrete, which consists of limestone with salt, ash and lime. In this case, it becomes clear why the blocks fit together so well.

However, the most important mystery is the purpose of the pyramids. Maybe these are just huge tombs for vain pharaohs? Or messages to descendants, where the secret knowledge of ancestors is encrypted?

It is also known that pyramids were built not only in Ancient Egypt. The Tibetan pyramids, for example, are tens of times larger than the Egyptian ones. The Mayan pyramids are as old as they are. And at the bottom of the Bermuda Triangle, another pyramid was found. Thus, we can assume that this is just part of a planetary plan that will soon come to fruition.

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Pyramids of Giza They are among the oldest tourist attractions on our planet and symbols of ancient Egyptian civilization. The largest of them is the Great Pyramid, which consists of 2.3 million stone blocks.

Until now, no one knows for sure how exactly the multi-ton blocks were installed in their places, but it is known that it was the tallest building in the world for more than 3,800 years, until the Cathedral in Lincoln was built (1300).

Also, the Great Pyramid is the only surviving Seven Wonders of the World. ancient world. It is considered the most massive structure built in the entire history of mankind.

Great Pyramid

The largest of all the pyramids of Ancient Egypt is the Great Pyramid of Khufu at Giza. On Greek Khufu is translated as Cheops. He was the pharaoh of the 4th Dynasty and reigned for 23 years, from 2589 to 2566 BC. e. The Great Pyramid is striking in its size and mathematical precision, and represents the apogee of the construction of the famous monuments of Ancient Egypt.

The Great Pyramid of Giza consists of 2.3 million stone blocks. Average weight one block - 2.5 tons, and the maximum - 15 tons. Each block fit so tightly together that the entire monument was built without the use of any mortar. If we divide the construction time (20 years) by the number of blocks (2.3 million), we can come to the conclusion that a new block was installed every five minutes.

When construction was completed, the Great Pyramid of Giza looked very different than it does today. On the entire surface of the structure there were white polished slabs, which are no longer present. At the top stood a pyramidal stone, which is also no longer there. Having lost the cladding and stone, the height of the pyramid became 138.75 meters (was 146.5), and the length at the base was reduced to 225 meters (was 230.33).

In order to get to the center of Khufu's pyramid, you need to walk through poorly lit narrow corridor. Having crossed halfway, you will enter a gallery 8.5 m high and 47 m long. The gallery leads to the tomb of the pharaoh, in which only the sarcophagus of Cheops remains. The walls in the tomb are made of red granite, and the pharaoh's chamber was isolated from thieves using shifted granite blocks. But, nevertheless, this did not save her from plunder.

Near the pyramid of Khufu, archaeologists discovered two underground vaults in which solar boats were stored. According to legend, after death, the pharaoh on this solar boat must go on a journey along the celestial Nile together with the sun god Ra. Archaeologists discovered one Solar Boat, which consisted of 1224 separate parts. It took 14 years to reconstruct it, and now it stands in the Sun Boat Museum near the Great Pyramid itself. The size of the boat is impressive: length 43 meters and width 6 meters.

Pyramid of Khafre in Giza

Khafre was the son of Khufu and ruled for 27 years, from 2558 to 2532 BC. Khafre tried to make his funeral complex more magnificent than his father's. And in some ways it was successful. Despite the fact that his pyramid is 3 meters shorter and 15% smaller in volume, due to the fact that it is built on a high embankment, it looks significantly higher than his father's.

But inside the pyramid of Khafre is much simpler. Inside, two entrances connect: one goes slightly higher, and the other goes down below the base of the pyramid. A long corridor leads directly to the burial chamber, where there is nothing else except a sarcophagus made of black granite. The lower passage leads to another chamber, which may have been intended for the queen.

Not far from the Pyramid of Khafre there are two sanctuaries: the Welcoming Temple and the Mortuary Temple.

They are connected to each other by a laid embankment about 500 meters long. Both sanctuaries are built from megalithic blocks covered with red granite. The welcoming temple was preserved in good condition, but only ruins remained of the Funeral Church.

Pyramid of Menkaure at Giza

The remaining pyramids of Giza are much smaller in size. For example, the pyramid of Menkaure is 10 times smaller than the Khafre monument. High-quality granite and stone were used for its construction. This distinguishes it from other pyramids, which were built from poor quality limestone. At the end of the 12th century, Sultan Osman ibn Yusuf became convinced of the strength of the walls of this pyramid and tried to destroy the structure for 8 months, but eventually retreated.

The inside of the pyramid of Menkaure is rather uninteresting: if traces of decoration are still visible in the first chamber, then further down there are only stone walls. There is no sarcophagus in Menkaure's burial chamber. It sank along with the ship Beatrice carrying it in 1838 on its way to England.

After the death of Pharaoh Menkaure, the construction of the pyramids still continued, but on a smaller scale, and the center of construction moved from Giza to Abusir and Saqqara.

The Great Sphinx is depicted with the body of a lion and the head of a man. Measuring 73.5 m long, 6 m wide and 20.2 m high, it is the largest monolithic statue in the world.

The Sphinx is the most interesting monument of ancient Egyptian civilization. First of all, because no one can say what the purpose of building such a huge sculpture was. The most likely theory states that the Sphinx served as protection for the two great pyramids of Cheops and Khafre.

Over time, the pharaohs began to view the Sphinx as a holder of royal power, so the rulers of Ancient Egypt constantly presented him with donations. It all started with Prince Thutmose IV, who dreamed that he would become a pharaoh if he cleared the Sphinx of sand. When this happened, he built a donation stele in front of the Sphinx.

Over the past few thousand years, the Sphinx has been covered with sand several times. In 1905, the statue was finally cleared of sand. Today, the monument is in constant need of repairs. The sandstone from which the Sphinx is carved is quite soft and is destroyed by time. Plus, it is rotting from the inside, most likely due to the rising groundwater level. There is a popular theory that the Sphinx’s nose was knocked off by Napoleon’s soldiers, but it is not true: it was lost long before the birth of the famous commander.

Plunder of the pyramids

Even at the design stage, Egyptian architects tried to protect the Giza pyramids from thieves. To achieve this, passages were blocked with large granite blocks, and false entrances and chambers were created. But the robbers turned out to be smarter and all the tombs were plundered.

Pyramids of Giza: useful information

  • Almost every hotel in Giza offers the opportunity to purchase tickets for tours to the pyramids.
  • To purchase tickets, there are two ticket offices near the pyramids: one next to the main entrance, and the second near the Sphinx.
  • In order to enter the pyramids, you will need an additional ticket. Also, the number of visits inside the pyramids of Khafre and Cheops is limited to 300 tickets: 150 of them at 8:30 am and 150 after 13:00.
  • Inside the pyramids it is very humid and hot, the corridors are dusty, and it is difficult to move. Therefore, it is not advisable for those who have problems with the lungs or heart to enter. Well, for those who are not afraid of such conditions, visiting the pyramids will be very educational and interesting.
  • Photography is prohibited inside the pyramids.
  • The most best time for visiting - in the morning at the time of opening. Later, crowds of tourists begin to arrive, and during the day it becomes very hot.
  • The Pyramids of Giza attract not only millions of tourists, but also a large number of scammers. Even as you approach, people may come up to you and say that they won’t let you in without a guide (don’t worry, they will let you in), or that you need a camel or a horse, which you can’t do without (you can easily get by).
  • Prices for a trip around the pyramid on a camel are very high; similar tours in other places in Egypt will cost you much less.
  • Before you take a camel or horse ride, agree on a price, and don't pay in advance until you get what you agreed on. if you pay in advance, your horse will go lame after a few meters and you will be offered another one, but for a separate fee. Discuss all the details of the trip in detail. The arrogance of animal owners knows no bounds. For example, after a trip, you may be asked to pay extra for help getting off the camel.
  • Cases of theft are also not uncommon. For example, if you give your camera to a stranger, there is a chance of losing it.
  • Due to security reasons, it is currently officially prohibited to climb the Pyramids of Giza, but in some places, the guards turn a blind eye to this for tips.
A rare genetic disorder that leads to the overgrowth of bone and other tissues.

Proteus syndrome is an extremely rare genetic disorder that causes overgrowth of body tissues such as bones. The growth often occurs disproportionately and can seriously change a person's appearance. The condition was named after the Greek god Proteus, who could take various shapes optional.

Symptoms of proteus syndrome

Each person with Proteus is affected differently. Some people may have overgrown limbs, while others experience excessive skull growth. While this condition can affect any part of the body, the most common are bones, fat, and connective tissue. Blood vessels and internal organs may also be affected.

Symptoms and features of Proteus syndrome are absent at birth. As the child begins to grow, the condition becomes apparent - usually between 6 and 18 months of age. The condition becomes more severe as the child continues to grow.


Although the specifics of the condition vary, certain symptoms are commonly observed in all patients diagnosed with Proteus syndrome.

General symptoms of proteus syndrome

Three characteristics common to people with Proteus syndrome include:
  • Some parts of the body, such as the limbs or head, grow rapidly and are disproportionately large compared to other parts of the body. The growth pattern usually occurs in an asymmetrical and "spotted" (mosaic) formation. Which parts of the body are affected will vary from person to person.
  • Over time, overgrowth becomes more severe and can change a person's appearance. Over time, a person may experience excessive growth in new areas.
  • No one in the person's family has the condition or any symptoms indicating it.
Other signs and symptoms of Proteus syndrome may include:
  • Noncancerous (benign) tumors
  • Abnormal curvature of the spine (scoliosis)
  • Fatty growths (lipomas) or areas of tissue loss (atrophy)
  • Deformed blood vessels, which increase the risk of blood clots and pulmonary embolism (the most common cause of death in people with Proteus syndrome)
  • Thick, rough, raised grooves in the skin, especially in the lower legs (a condition called connective tissue neurosis cerebri, which is almost exclusively seen in people with Proteus syndrome)
Some people with Proteus syndrome develop problems with the central nervous system. nervous system which can lead to mental retardation or seizures.

People with Proteus syndrome may also have certain facial features, such as a long face with wide nostrils, squinting (strabismus), and drooping eyelids (ptosis). Some studies show that people with characteristic features Individuals associated with Proteus syndrome are more likely to have neurological problems associated with this disease. Although the connection between these two aspects has been noted, it is not yet fully understood.

Causes of proteus syndrome

Proteus syndrome is not believed to be hereditary. Of the several hundred cases in the medical literature, not a single person known to have Proteus syndrome had a child with the condition.

Proteus syndrome, however, is caused by a genetic mutation. In 2011, researchers identified the gene responsible for proteus syndrome: AKT1. The gene is involved in how cells grow and divide (proliferation) and can also prevent cells from dying when they should (apoptosis). Because it affects these cellular mechanisms, the gene is also involved in some forms of cancer.

Even though Proteus syndrome is associated with a gene, it does not mean that the parent "gives" the condition to the child. Mutations occur spontaneously (sporadic) and early stages fetal development.

A mutation in the AKT1 gene affects how cells grow, but the mutation will not affect all cells (called mosaicism). This causes some tissues to grow normally, while others develop the growth characteristic of Proteus syndrome.

The specific symptoms a person has and their severity are usually proportional to the ratio of normal to mutated cells they have.

How they mutate and cause disorders

Diagnosis of proteus syndrome

Proteus syndrome is very rare, with only a few hundred cases diagnosed worldwide, and many researchers believe the condition may be overdiagnosed. Other conditions that can cause asymmetric or abnormal growth patterns may be misdiagnosed as Proteus syndrome, meaning the true incidence of the disorder may be even rarer.

Doctors use a very specific set of guidelines to make a diagnosis of Proteus syndrome. Understanding the role that gene mutations play in the development of the condition has helped make the diagnostic process more accurate.

Along with diagnostic criteria, which include the presence physical characteristics, signs and symptoms, diagnosing Proteus syndrome usually requires a thorough workup, including tissue biopsy, laboratory tests, imaging studies, and genetic testing.

Proteus syndrome was not described in the medical literature until 1979. Because the disease is so rare, many doctors have never seen it. Even with strict diagnostic criteria, it can be difficult to correctly diagnose the condition. Proteus syndrome appears to affect men more often than women and occurs in people of all racial and ethnic groups.

One of the most notable cases of Proteus syndrome is that of Joseph Merrick, better known as the "Elephant Man." Throughout his life in the early 19th century, Merrick was thought to have neurofibromatosis. Many decades later, researchers now believe that he actually had Proteus syndrome.

Why the medical mystery "The Elephant Man" has baffled doctors for 100 years

Treatment of proteus syndrome

Does not exist from Proteus syndrome. Each person who has the condition will have different symptoms, and these symptoms will vary in severity. All patients with Proteus syndrome will need to work with a team of healthcare professionals to manage their medical care.

Health needs

Some common health needs of people with Proteus syndrome include:
  • Surgery to remove growths or excess skin
  • Dental problems, including malocclusion
  • Poor vision, squinting (crossed eyes), or vision loss due to neurological disorders
  • Cosmetic procedures to correct areas of the body deformed due to excessive growth
  • Monitoring of affected organ systems (kidneys, brain, spine, etc.)
  • Mental health, social and educational support
  • Referral to specialists who deal with body systems that are more severely affected or that are at higher risk for complications (for example: a vascular doctor who can monitor a person for blood clots, or an orthopedist who can help treat problems related to excessive bone growth)
Some complications of Proteus syndrome can be serious, even life-threatening. Blood clots, emboli and damage internal organs may endanger human life. Rapid and steady bone growth may require a person with Proteus syndrome to undergo many complex orthopedic procedures.

However, surgery comes with its own risks, especially for patients at risk of developing blood clots, as most with Proteus syndrome do. Because of this risk, deciding when, if at all, to act can be a difficult decision for the clinician.

Other potential problems, although they may not shorten life expectancy, can certainly affect a person's quality of life. Excessive height or growth caused by Proteus syndrome can seriously change a person's appearance. When one or more limbs are affected, a person's mobility can be greatly affected. This, combined with neurological effects, can limit a person's ability to live independently. Social stigma associated with being “other” appearance, may cause emotional, anxiety and depression.

While people with Proteus syndrome usually need a team of specialists to manage their care, as most doctors (such as general practitioners or internists) are unlikely to have encountered such cases earlier in their careers, the management of a person's care is usually supervised. specialist such as a geneticist.

Need in regular care, follow-up, and close monitoring with specialists will depend on what areas of the body are affected, as well as the severity of the person's symptoms and the progression of the condition.

A word from Health-Ambulance

Because Proteus syndrome is so unusual, it can be difficult to properly diagnose. Once a person has been diagnosed, the primary focus is on managing symptoms (which can range in severity from mild to debilitating and can be physical, medical, and cosmetic) and preventing potentially life-threatening complications (such as pulmonary embolism or blood clots). ).

Managing Proteus syndrome can be difficult, but is most successful when a person with the condition has the support of a team of health care professionals, including specialists, genetic counselors and mental health professionals, who can address all aspects of a person's life that have been affected by the condition.