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:''See main article at ''Hemophilia in European royalty'' | :''See main article at ''Hemophilia in European royalty'' | ||
*In 1924, a Finnish doctor discovered a hereditary bleeding disorder similar to hemophilia localised in the "Åland Islands", southwest of Finland.[36] This bleeding disorder is called '' | *In 1924, a Finnish doctor discovered a hereditary bleeding disorder similar to hemophilia localised in the "Åland Islands", southwest of Finland.[36] This bleeding disorder is called ''Von Willebrand Disease". | ||
==European Royalty== | ==European Royalty== | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]
Overview
Hemophilia was first described by Dr. John Conrad Otto, a Philadelphia physician in 1803 who wrote an account about "a hemorrhagic disposition existing in certain families." He recognised that the disorder was hereditary and that it affected mostly males and rarely females.
Historical Perspective
Scientific Discovery[1]
- The first evidence of hemophilia is in the Talmud, Jewish holy text, which states that males did not have to be circumcised if two brothers had already died from the procedure. In the 12th century, the Arab physician Albucasis wrote of a family whose males died of bleeding after minor injuries. Then, in 1803, Dr. John Conrad Otto, a Philadelphia physician, wrote an account about "a hemorrhagic disposition existing in certain families." He recognised that the disorder was hereditary and that it affected males and rarely females. He was able to trace the disease back to a woman who settled near Plymouth in 1720. His paper was the second paper to describe important characteristics of an X-linked genetic disorder (the first paper being a description of colour blindness by John Dalton who studied his own family). The idea that affected males could pass the trait onto their unaffected daughters was not described until 1813 when John Hay published an account in The New England Journal of Medicine. The first usage of the term "hemophilia" appears in a description of the condition written by Hopff at the University of Zurich in 1828.[2]
- In 1937, Patek and Taylor, two doctors from Harvard, discovered anti-hemophilic globulin. Pavlosky, a doctor from Buenos Aires, found Hemophilia A and Hemophilia B to be separate diseases by doing a lab test. This test was done by transferring the blood of one hemophiliac to another hemophiliac. The fact that this corrected the clotting problem showed that there was more than one form of hemophilia.
- See main article at Hemophilia in European royalty
- In 1924, a Finnish doctor discovered a hereditary bleeding disorder similar to hemophilia localised in the "Åland Islands", southwest of Finland.[36] This bleeding disorder is called Von Willebrand Disease".
European Royalty
Queen Victoria passed hemophilia on to many of her descendants.
- Hemophilia has featured prominently in European royalty and thus is sometimes known as 'the royal disease'. Queen Victoria passed the mutation for hemophilia B[3][4] to her son Leopold and, through some of her daughters, to various royals across the continent, including the royal families of Spain, Germany and Russia. In Russia, Tsarevich Alexei Nikolaevich son of Nicholas II, was a descendant of Queen Victoria through his mother Empress Alexandra and suffered from hemophilia.
- It was claimed that Rasputin was successful at treating Tsarevich Alexei's hemophilia. At the time, a common treatment administered by professional doctors was to use aspirin, which worsened rather than lessened the problem. It is believed that, by simply advising against the medical treatment, Rasputin could bring visible and significant improvement to the condition of Tsarevich Alexei.
- In Spain, Queen Victoria's youngest daughter, Princess Beatrice, had a daughter Victoria Eugenie of Battenberg, who later became Queen of Spain. Two of her sons were hemophiliacs and both died from minor car accidents. Her eldest son, Prince Alfonso of Spain, Prince of Asturias, died at the age of 31 from internal bleeding after his car hit a telephone booth. Her youngest son, Infante Gonzalo, died at age 19 from abdominal bleeding following a minor car accident where he and his sister hit a wall while avoiding a cyclist. Neither appeared injured or sought immediate medical care and Gonzalo died two days later from internal bleeding.
Blood contamination issues
- Ryan White was an American hemophiliac who became infected with HIV/AIDS through contaminated blood products.
- Prior to 1985, there were no laws enacted within the U.S. to screen blood. As a result, many people with hemophilia who received untested and unscreened clotting factor prior to 1992 were at an extreme risk for contracting HIV and hepatitis C via these blood products. It is estimated that more than 50% of the haemophilia population, i.e. over 10,000 people, contracted HIV from the tainted blood supply in the United States alone.
- As a direct result of the contamination of the blood supply in the late 1970s and early/mid-1980s with viruses such as hepatitis and HIV, new methods were developed in the production of clotting factor products. The initial response was to heat-treat (pasteurized) plasma-derived factor concentrate, followed by the development of monoclonal factor concentrates, which use a combination of heat treatment and affinity chromatography to inactivate any viral agents in the pooled plasma from which the factor concentrate is derived. The Lindsay Tribunal in Ireland investigated, among other things, the slow adoption of the new methods.
References
- ↑ "Hemophilia wikipedia".
- ↑ "The History of haemophilia". Retrieved 2007-06-27.
- ↑ Michael Price (8 October 2009). "Case Closed: Famous Royals Suffered From Hemophilia". ScienceNOW Daily News. AAAS. Retrieved 9 October 2009.
- ↑ Evgeny I. Rogaev; et al. (8 October 2009). "Genotype Analysis Identifies the Cause of the "Royal Disease"". Science. Retrieved 9 October 2009.