Hemophilia stands out in the history of European empires in the 19th and 20th centuries. Queen Victoria of England, through two of her five daughters, Princess Alice and Princess Beatrice, passes mutations to various royal houses across the continent, including the royal family of Spain, Germany and Russia. Victorian son, Prince Leopold, Duke of Albany also suffered from this disease. For this reason, hemophilia was once popularly called "royal disease". Tests on the remains of the Romanov empire family indicate that the specific form of hemophilia derived by Queen Victoria may be relatively rare Haemophilia B.
Sex-related X chromosome disease manifests almost exclusively in men, although the genetic mutation that causes this disorder lies on the X chromosome and can be inherited from the mother by a boy or from a mother or father by daughters. This is because it is recessive, which means that only one copy of the genes of the correct functioning blood clotting factor is required for normal freezing. Women have two X chromosomes, and therefore an excessive duplicate of the clotting factor genes located on them. A woman who inherits a mutated copy on one X chromosome also inherits a second X chromosome from another parent who tends to carry a copy of an unmutable gene, capable of directing the appropriate clumping. Such females, with normal freezing but have a single copy of a mutated gene, are called carriers. Males have only one X chromosome, inherited from their mother, after receiving Y chromosomes from their father instead of the second X. If their single X chromosome contains hemophilia mutations, they do not have a second copy to provide normal function, as in female carriers. Each carrier child will have a 50% chance of inheriting their mother's mutation, becoming haemophilia (child) or career (daughter). The daughter of a man with hemophilia will always inherit his mutation, while a son will never inherit it. A woman will only be affected with hemophilia in the rare circumstances that she inherits the mutated X chromosome from the father of hemophilia and the carrier mother. There is no double inheritance case as it is known among the descendants of Queen Victoria.
Although individual hemophilia is usually traceable to the ancestor, about 30% of cases have no family history of the disorder, and this condition is speculated as a result of spontaneous mutations in the ancestor. Victoria seems to have become a spontaneous mutation or de novo and she is usually considered a source of disease in modern cases of hemophilia among her offspring. Queen Victoria's father, Prince Edward, Duke of Kent, was not a hemophiliac, and the likelihood of his mother having a lover who suffered from hemophilia was very small given the low life expectancy of the nineteenth-century hemophilia patients. His mother, Victoria, the Duchess of Kent, is not known to have a family history of the disease, although it is possible that he is a carrier but among his only sons Victoria receives a copy of the mutation. The rate of spontaneous mutation is known to increase with the age of the father, and Victoria's father is 51 years old at birth.
The eldest daughter of Queen Victoria, Victoria, Princess Royal, seems to have escaped the hemophilia genes because it does not appear in any of the matrilineal descendants. The fifth child of Victoria, Helena, may or may not be a carrier; two healthy sons survived to adulthood, but two other sons died in infancy and both daughters had no problems. Sixth child Victoria, Louise, died without a problem. The sons of Queen Victoria Edward, Alfred, and Arthur are not hemophiliacs, but their daughters Alice and Beatrice are confirmed as carriers of genes, and Victorian son Leopold is a hemophiliac, making his daughter Alice, Countess of Athlone a career as well..
Video Haemophilia in European royalty
Queen Victoria's family tree
Children
Victoria, German Empress (1840 - 1901) Problem: Wilhelm II of Germany, Charlotte, Duchess of Saxe-Meiningen, Prince Henry of Prussia, Prince Sigismund of Prussia, Viktoria, Adolf Princess of Schaumburg-Lippe, Prince Waldemar of Prussia, Sophia, Queen Hellenes, Margaret, Landgravine of Hesse-Kassel
Edward VII, King of the Kingdom of England (1841 - 1910) Problems: Prince Albert Victor, Duke Clarence and Avondale, George V, Louise, Princess Royal, Princess Victoria, Maud, Queen Norway, Prince Alexander John
Alice's daughter, Grand Duchess of Hesse and Rhine (1843 - 1878) Problems: Victoria Mountbatten, Marchioness Milford Haven, Grand Duchess Elizabeth Feodorovna from Russia, Irene, Princess Henry Prussia, Ernest Louis, Great Duke of Hesse and by Rhine, Count of Friedrich, Alexandra Feodorovna, Empress of Russia, Princess Marie of Hesse and by Rhine
Prince Alfred, Duke of Saxe-Coburg and Gotha (1844 - 1900) Problems: Alfred, Prince of Saxe-Coburg and Gotha, Marie, Queen of Romania, Victoria Melita, Great Duchess Russia, Alexandra, Princess Hohenlohe-Langenburg, Princess Beatrice, Duchess of Galliera
Helena, Princess Christian of Schleswig-Holstein (1846-1923) Problem: Prince Christian Victor, Albert, Duke of Schleswig-Holstein, Princess of Helena Victoria, Princess Marie Louise, Prince Harald
Princess Louise, Duchess of Argyll (1848 - 1939) No problem
Prince Arthur, Duke of Connaught and Strathearn (1850 - 1942) Problems: Margaret, Crown Princess of Sweden, Prince Arthur of Connaught, Princess Patricia, Lady Ramsay
Prince Leopold, Duke of Albany (1853 - 1884) Problems: Princess Alice, Count of Athlone, Charles Edward, Duke of Saxe-Coburg and Gotha
Beatrice, Princess Henry of Battenberg (1857 - 1944) Problems: Alexander Mountbatten, 1st Marquess Carisbrooke, Victoria Eugenie, Queen of Spain, Lord Leopold Mountbatten, Prince Maurice of Battenberg Maps Haemophilia in European royalty
Princess Alice
Alice (1843-1878), Victoria's third child, and wife of the future Grand Duke of Louis IV of Hesse and by Rhine (1837-1892), left her at least three children: Irene, Friedrich, and Alix.
- Princess Victoria of Hesse and by Rhine (1863-1950), then Marchioness Milford Haven, wife of Prince Louis of Battenberg (1854-1921) and maternal grandmother to Prince Philip, Duke of Edinburgh,.
- Princess Elizabeth of Hesse and by Rhine (1864-1918), then Grand Duchess Elizabeth Feodorovna of Russia through her marriage to Grand Duke Sergei Alexandrovich (1857-1905), may or may not be a carrier. After the murder of her husband, she became a nun and had no children when killed by the Bolsheviks in 1918. Princess Irene of Hesse and by Rhine (1866-1953), then Princess Heinrich of Prussia, through her marriage to Prince Heinrich of Prussia (1862-1929), left it to two of her three sons:
- Prince Waldemar of Prussia (1889-1945). Persisting up to age 56; has no problem.
- Prince Heinrich of Prussia (1900-1904). Died at age 4.
- Prince Friedrich of Hesse and by Rhine (1870-1873). Died before the third anniversary of bleeding in the brain from falling from a third floor window (which would almost certainly be fatal even if he did not have hemophilia).
- Princess Alix of Hesse and by Rhine (1872-1918), then Empress Alexandra Feodorovna of Russia through her marriage to Tsar Nicholas II (1868-1918). Alix had a marriage proposal from his first cousin, Prince Albert Victor (1864-1892), the eldest son of the Prince of Wales (later King Edward VII); if he accepts it, hemophilia may return to the immediate line of succession in Britain.
- Grand Duchess Maria (1899-1918), the third daughter of Nicholas and Alexandra, was regarded by some as a symptom because she had bleeding during a tonsillectomy. Romanov family DNA tests still in 2009 showed that one of the four daughters, considered Maria by American researchers and Anastasia by Russian researchers, was a carrier.
- Tsarevitch Alexei (1904-1918) was murdered with his family by the Bolsheviks at the age of 13. Alexei's hemophilia was one of the factors causing the collapse of the Russian Empire during the Russian Revolution of 1917.
- Princess Marie of Hesse and by Rhine (1874-1878), the seventh and last child of Alice, may or may not be a carrier. He died of diphtheria at the age of four.
Prince Leopold
Leopold (1853-1884), the eighth child of Victoria, was the first member of the family to show hemophilia; he died at the age of 30 because of bleeding after a small fall, just two years after marrying Princess Helena of Waldeck and Pyrmont. (1861-1922)
He gave the gene to his only daughter, because all daughters of haemophiliac's father would inherit the gene:
- Princess Alice of Albany (1883-1981), then Countess of Athlone, who in turn handed her over to her eldest son:
- Prince Rupert of Teck (1907-1928), who died at the age of 20, bleeds to death after a car accident.
The younger son of Alice, Prince Maurice of Teck died in infancy, so it is not known whether he was a sufferer. His daughter Lady May Abel Smith (1906-1994), granddaughter of Leopold, has a living descendant who is unknown or has hemophilia.
Leopold's son, Charles Edward (1884-1954), was unaffected, because father could not pass the gene on to a son.
Princess Beatrice
Beatrice (1857-1944), Victoria's ninth and lastest son, and the wife of Prince Henry of Battenberg (1858-1896) bequeathed to at least two, if not three, of his four children:
- Princess Victoria Eugenie of Battenberg (1887-1969), then Queen Victoria Eugenia of Spain through her marriage to King Alfonso XIII (1886-1940), who left her to
- Infante Alfonso of Spain, Prince Asturias (1907-1938). Dead at age 31, bleed to death after a car accident.
- Infante Gonzalo (1914-1934). Died at the age of 19, bleed to death after a car accident.
- The two daughters of Victoria Eugenie, Infantas Beatriz (1909-2002) and Maria Cristina of Spain (1911-1996), both of whom had unknown or hemophilia living births.
- Count Leopold of Battenberg (1889-1922), then, Lord Leopold Mountbatten. Died at age 32 during knee surgery.
- Prince Maurice of Battenberg (1891-1914). Killed in action in World War I at the age of 23 years. Maurice's hemophilia is disputed by various sources: It seems unlikely that known hemophilia would be allowed to serve in combat.
Today
No member living from the present or past European dynasties is known to have symptoms of hemophilia or is believed to carry the gene for it. The last descendant of Victoria who is known to suffer from this disease is Infante Don Gonzalo, born in 1914, although tens of descendants of Queen Victoria (including men only descended through women) have been born since 1914. However, since hemophilia genes usually remain hidden in women who only inherited the genes of one parent, and the descendants of Victorian women have left many offspring in noble and noble families, there is still a small chance that the disease could reappear, especially among the descendants of female descendant daughter Beatrice.
- The two sons of Infanta BeatrÃÆ'z are not affected by the disease. Beatriz's oldest daughter, Sandra, has two children, a son and a daughter. His son was unaffected, and his daughter had two sons, who seemed unaffected. BeatrÃÆ'z's youngest daughter, Olimpia, has six children; two of his eldest daughters, Beatrice and Sibilla were both married to children, none of whom, in the case of their sons, were seen as hemophiliacs. If the descendants of Sibilla express or transmit genes, however, another ruling dynasty in Europe, in the 21st century, will join the rest of the ruling family who inherited the disease from Queen Victoria. Young Olimpia girls are unmarried, but there is still the possibility that they can become carriers. Another daughter, Laura, died as a child, like her only son, Paul, the latter apparently not a hemophiliac.
- Infanta Maria Cristina has four daughters, all potential carriers. His eldest daughter, Vittoria Eugenie, has a daughter and three sons, the latter all seemingly unaffected. Infanta's second daughter, Giovanna, has only one child, an unaffected son. Her two youngest daughters, Donna Maria Teresa and Donna Anna Sandra, also have only daughters. Of this number, only one, Maria Teresa's second daughter, Isabel, married, but she also only had a daughter. There is a possibility that this disease may remain in the offspring of Princess Beatrice.
Chronological order
Queen Victoria died in 1901 so she lived to see her youngest son and grandson die of the disease. A great-grandchild is also diagnosed with this disease. The genes can be passed down the female line without the born child of haemophiliac, but as the family line continues and no sons of hemophilia are born, it is unlikely that a particular ancestor has a gene and passes it through the female line.
Type of hemophilia found
Since the last known descendant of Queen Victoria with hemophilia died in the 1940s, the type of hemophilia found in this family remained unknown until 2009. Using genetic analysis of the remains of Romanov dynasty, and especially Tsarevich Alexei, Rogaev et al. able to determine that the "Kingdom Disease" is actually a hemophilia B. In particular, they found a single nucleotide change in the gene for freezing Factor IX that led to incorrect RNA splicing and resulting in a truncated nonfunctional protein.
Note
References and external links
- Potts, D. M. Queen Victoria's Genes . Sutton Publishing, 1999. ISBNÃ, 0-7509-1199-9.
- "Hemophilia: The Royal Disease"
- Queen Victoria's family tree and her offspring
- Hemophilia on Queen Victoria's Descendants. Archived 2006-11-18 at the Wayback Machine.
Source of the article : Wikipedia
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