Royal affliction. Prince Alexei bled frequently and for prolonged periods.
State Archives of the Russian Federation
descendants of Queen Victoria males were hit by poor health. The son of the British monarch of the 19th century Leopold, Duke of Albany, died from blood loss after he slipped and fell. His grand-son Friedrich bled to 2 years; grandchildren son Leopold and Maurice, 32 and 23, respectively ages. The affliction commonly known as the "royal disease" spread like Victoria's heirs married into royal families across Europe, decimating the thrones of Britain, Germany, Russia and 'Spain. Based on the symptoms, modern researchers concluded that the royals have suffered from hemophilia - a genetic disease that prevents blood clotting - but there was never any concrete evidence. Now a new DNA analysis on the bones of the last Russian royal family, the Romanovs, indicates the Royal disease was indeed hemophilia, a rare subtype known as hemophilia B. name
Hemophilia prevents proteins known under the name of fibrin scab on a cut or blood clots to stop internal bleeding. Even minor injuries can lead to bleeding that lasts for days or weeks and can be fatal. The disease is recessive and is carried on the X chromosome, which means that men are more likely to develop, while women generally act as carriers and have no symptoms.
This was the case with Prince Alexei Romanov, Tsar Nicolas II's son, grand-son of Queen Victoria and heir to the Russian throne. From a young age, Alexei was prone to prolonged bleeding, and her family feared he would not make it through the first month of life, says Evgeny Rogaev, a geneticist at the University of Massachusetts Medical School in Worcester. The disease did not kill Alexei, however: He was killed at age 13 in 1918, and the rest of the Russian royal family after the Russian Revolution. Earlier this year, Rogaev and colleagues reported that according to DNA analysis, the bodies of two children found near the murder site were those of Alexei and his sister Maria. They also confirmed that the other bodies near the site belonged to the remains of the Romanov family. But Rogaev wanted to solve the final puzzle Romanov: Do they really have hemophilia
He and his colleagues analyzed the DNA fragments of royal bones, this time looking for genetic markers of 'haemophilia?. The most common type of the disease, hemophilia A, represents about 80% of cases of hemophilia and is caused by a mutation in a gene called F8 , which encodes a protein involved in the blood clotting. They do not find the mutation. Rogaev then went in search of a rare form of the disease, hemophilia B, which implies another gene F9 . This time, the team found a mutation in F9 , which would have inhibited coagulation, in the bones of Alexei, his sister Anastasia, and their mother Alexandra.
The results, published online today science indicate that Alexei had hemophilia B and actually his mother and Anastasia were carriers of the disease, on the previous speculation. They also confirm that other cases of "royal illness" in the family line were hemophilia, Rogaev said, because they all shared a common genetic heritage. The last carrier of the disease in the royal family was Prince Waldemar of Prussia, who died in 1945.
The disease affected not only the Romanov family, but also probably Russian history, Rogaev said. fragile state of his mother Alexandra Alexei encouraged to keep close company with the Russian mystic Rasputin, who claimed to use healing magic. "There was no medicine to that point," said Rogaev. "She tried to do everything that is possible." According to some historians, when Rasputin used his close relationship with the Romanovs to influence bureaucratic business his favor, the public has grown increasingly suspicious of the regime, perhaps hasten the revolution.
Katherine High, a hematologist who studied blood coagulation at the Philadelphia children's Hospital, said the mutation found in Romanov bones corresponds to an established genetic profile known to cause hemophilia B, further in supporting the conclusions of Rogaev. Tracing this model to the royal family and its history of the disease is "very interesting and exciting," she said.
people affected by the disease of today should be delighted to see as more of hemophilia B stage the shadow of a common type, pediatrician says hematologist Paul Monahan, University of North Carolina, Chapel Hill. "Now it is clear that it has had a huge impact on Western history."
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