Marfan syndrome or how are genias

Marfan syndrome - constant danger syndrome

Defects of some genes affecting the formation and development of connective tissue in humans, often lead to disproportionate giantism. This disease was described in 1896. French Pediatrician A.Marfan. With the most vivid manifestation of this dominant feature, people with very long hands and legs and a relatively short torso appear. Their elongated fingers resemble the paws of a huge spider. Hence the figurative name of this imbalance - the Arahanodactile (from Greek. Dactyl - Finger and Arachna - Woman, on legend, converted by Athena in spider). People with similar defects are extremely thin, their chest is deformed, the crystal eye is shifted.

Such an anomaly is called Marfan's syndrome and is considered half-liters, since it is associated with heart defects. The syndrome is caused by the hereditary defect of the development of connective tissue and is also characterized by the lesion of the musculoskeletal system, eye and internal organs. The root causes of such vices are not sufficiently studied.

Often, people with Martan syndrome die from the aortic aneurysm - the largest vessel coming out of the right ventricle of the heart, does not withstand the pressure thrown into it. People who do not have this syndrome with all cruelty, wait to mature years. Fortunately, Marfan's syndrome is rare enough. Specialists estimate the likelihood of its appearance as 1/50000.

«Genius» - Compensation for the disease

The only compensation, which people with Martane syndrome get from fate for their vice, - increased blood adrenaline content. As you know, this hormone is produced by adrenal glands and is thrown into the bloodstream at the time of danger. As a result, many parameters of the human body (heart patent, blood pressure) are given, so to speak, in combat readiness. Thus, people with Martan syndrome all their lives are in an excited state: adrenaline constantly spurst the nervous system and makes them incredible workaholics.

Historical examples: Fame and Martan Syndrome

Marfan's syndrome suffered from several world famous personalities, distinguished extraordinary performance. Such was the Lincoln Lincoln Lincoln, which, thanks to constant self-education, outstanding abilities and, most importantly, stunning hardworking became president of the United States. He possessed high height - 193 cm, huge footsteps and hands, a small chest and long flexible fingers - a typical physique with Martan syndrome.

Very similar to Lincoln on the physical warehouse was the son of the midnight shoemaker, which later became the great writer XIX in., Hans Christian Andersen. His extraordinary hardworking manifested himself in school. He rewrite his literary works to ten times, seeking ultimately virtuoso accuracy and at the same time ease of style.

Contemporaries so described its appearance: «He was high, thin and extremely peculiar in posture and movements. His hands and legs were disproportionably long and thin, brushes are wide and flat, and the feet of the legs of such huge sizes that he probably never had to worry that someone replaced his Kalosh. His nose was the so-called Roman shape, but also disproportionally great and somehow especially issued forward».

Nervous tension in which, apparently, this talented person was constantly located, he gave rise to many fears. He was afraid to get a cholera, suffer from a fire, to get into an accident, lose important documents, take not the dose of medication...

The story knows the case when the long, thin fingers of a man with Martan syndrome along with impressive performance helped their owner to make a fantastic career. We are talking about the famous violinist Niccolo Paganini. Goethe and Balzac so describe his appearance in their memories: Deadly pale, as if a face-winged face, deeply spawned eyes, thin, angular movements and, most importantly, thin ultra-flexing fingers, some incredible length, as if halve it longer, than ordinary people. This purely morphological feature allowed him to create a violin of real wonders.

In the crowd, who listened to the improvisation of Paganini on the Roman streets, alone said that he was in collusion with the devil, others - that his art is the music of heaven, in which angel votes sound. He played so that the listeners seemed to be somewhere hidden the second violin playing at the same time from the first. Many up to XX. They believed rumors that in the youth of Niccolo resorted to the help of a surgeon who made him an operation to increase the flexibility of the hands. Now, we know that, most likely, he has been obliged to rare genetic deviation.

For the first time, Paganini's skill with Martan syndrome was indicated by the American doctor Mairon Schenfeld in an article published in «Journal of the American Medical Association». He indicated that the description of the appearance of Paganini - pale leather, deeply planted eyes, thin body, awkward movements, «Puachge» Fingers - absolutely accurately coincides with the description of the appearance of people with Martan syndrome. As you know, at the end of my life, a great musician almost lost voices. This is an extra certificate in favor of the fact that Paganini had a Martan syndrome, since severely complication of this disease is a strong hoarse or even a voice loss caused by paralysis of the upper gentle nerve.

Preserved Diary of the doctor who treated Paganini. The recordings made in it confirm the classic symptoms of Marfan syndrome: asthenic addition, expressly expressed scoliosis, «Bird» Facial expression, narrow skull, protruding or cut chin, eyes with blue gluers, sinking, disproportions in the size of the body and limbs, brushes and feet are long with thin «spider-shaped» fingers. It is not surprising that not only the game of Paganini, but his unusual appearance itself made an impression on his contemporaries, giving birth at once the most unthinkable legends about the musician.

It should be noted that marfan's syndrome itself does not have a musical giftedness. With the exception of Paganini, among patients with this syndrome there were no outstanding musicians. As for Paganini, the disease only gave him great technical capabilities, and a great musician, with a huge creative heritage, including, in addition to works for violin with other tools and the orchestra, also more than 200 plays for the guitar, he began thanks to his great talent and hardworking, Also indirectly associated with Martan syndrome.

Surely you remember two more famous long, awkward and talented «Nosecha». This is Charles de Gaulle and the roots of Ivanovich Chukovsky. The active nature of the future president of France was so brightly manifested in the youth that many of his colleagues in the army until the Second World War were then read him into generalssimises. De Gaulle's head always towering over the sea of ​​caskens and testers marching soldiers. At the same time, sitting at the table, he seemed quite ordinary man. The secret was covered in its disproportionate addition, so characteristic of Marfan's syndrome.

Above all in the crowd was the favorite by the children «Fly tracked», «Moydodyra» and «Cockroach». Its long-growing, long-beaches, the most and the overall hopping of the figure were repeatedly played in cartoons. «I work all my life. Like no! As a tractor!» - I wrote about myself the roots of Ivanovich. And it really was so, although his titanic performance for many readers of children's poems writer, not familiar with his numerous special literary articles and translations, remained hidden. Like Hans Christian Andersen, Chukovsky repeatedly reworked every line. «I never watched to someone else with such work was given by the Scripture technique itself», - He noticed about himself.

From our contemporaries, Martan syndrome may have suffered a biologist.IN. Nikolsky. By the end of the end of Moscow University, he had five printed works. In the afternoon of work, the number of its printed publications exceeded 300, and among them there were about ten books. Such a stunning performance can boast of not every even a very capable scientist! Is it possible to argue that any disorders due to genes due to harmful genes?