Haemophilia figured prominently in the history of European royalty in the 19th and 20th centuries. Britain's Queen Victoria, through two of her five daughters (Princess Alice and Princess Beatrice), passed the mutation to various royal houses across the continent, including the royal families of Spain, Germany and Russia. Victoria's son Prince Leopold, Duke of Albany suffered from the disease. For this reason, haemophilia was once popularly called "the royal disease". The sex-linkedX chromosome disorder manifests almost entirely in males, although the gene for the disorder is located on the X chromosome and may be inherited from either mother or father. This is due to the fact that, although the trait is recessive, males only inherit one X chromosome, from their mothers, thus if the haemophilia gene is transmitted on it, there is no possibility for the male to inherit a haemophilia-free gene from his father to mask or dilute the symptoms.That's because fathers determine the sex of the child, either by passing them another "X" chromosome, in which case the child will be female or by passing them a "Y" chromosome in which case the child will be a boy. Now, the "X" chromosome from the mother will dominate the "Y" chromosome from the father. The mother has two "X" chromosomes and the father has an "X and "Y" chromosome.
If the mother has one defective chromosome and one good chromosome, the odds become 50-50 that the girls will be carriers and the boys will have the disease. Since the hemophilia chromosome is recessive, the "X" chromosome from the father will mask the disease, making the daughter a carrier but not ill. /p> Â Females who inherit the gene for haemophilia from both parents would be expected to manifest full symptoms, similar to those seen in affected males, but this is extremely rare. Despite frequent inter-marriage among royalty, no case of such double inheritance is known among Queen Victoria's descendants. However, it is possible that such cases have gone undocumented because of the potentially higher mortality affected females would likely experience, especially upon onset of menarche. Although an individual's haemophilia can usually be traced in the ancestry, in about 30% of cases there is no family history of the disorder and the condition is speculated to be the result of spontaneous mutation in an ancestor.[1] Victoria appears to have been a spontaneous or de novomutation, and is considered the source of the disease in all known modern cases of haemophilia among royalty. Â Her mother, Victoria, Duchess of Kent, was not known to have a family history of the disease. Victoria's maternal half-brother, Carl, 3rd Prince of Leiningen, and the descendants of her half-sister, Feodora, Princess of Hohenlohe-Langenberg, are not known to have suffered from the disease. Queen Victoria's father, Prince Edward, Duke of Kent, was not a haemophiliac. The probability of her mother having had a lover who suffered from haemophilia is minuscule, primarily due to the low life expectancy of 19th century haemophiliacs. Queen Victoria's eldest daughter, Victoria, apparently escaped the haemophilia gene as it did not appear in any of her descendants. Victoria's fifth child, Helena may or may not have been a carrier; two healthy sons survived to adulthood but two other sons died in infancy and her two daughters did not have issue. Victoria's sixth child, Louise, died without issue. Her sons King Edward VII, Alfred, and Arthur were not haemophiliacs. However, her son Leopold was a sufferer of haemophilia and her daughters Alice and Beatrice were confirmed carriers of the gene.
I've studied this "line" before. Most interesting.
And I wonder what the current royals have and/or are dealing with?
I love reading medical related articles. This one was most interesting.
almost forgot about the hemophilia.Have that got this cured so to speak. Loads of good information there.Along with febreize. Thank you for the history on this.I hope that more people read this.
Victoria is renowned for having infected Royal Europe with the illness - including Czar Nicholas son Alexi (can't spell his name). Victoria hated pregnancy including labor (although she loved, her children). I think it was when she was delivering her third child, that she decided to try the new fan-dangle 'chloroform' - much against the advice of the medical profession of the time! Once the people found out that she had used it, everyone did (at least those who could afford it).
We don't hear much about it in our daily life, I guess maybe here in America it's not a high priority subject, aside from hearing about people getting AIDS as a result of the blood transfusions required to help with their hemophilia, and haven't heard much about that lately, either.