Hemophilia historical perspective
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Historical Perspective
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. The first usage of the term "hemophilia" appears in a description of the condition written by Hopff at the University of Zurich in 1828.[1] 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
Hemophilia figured prominently in the history of European royalty and thus is sometimes known as "the royal disease". Queen Victoria passed the mutation to her son Leopold and, through several of her daughters, to various royals across the continent, including the royal families of Spain, Germany, and Russia. Tsarevich Alexei Nikolaevich, son of Nicholas II, was a descendant of Queen Victoria and suffered from hemophilia.
Prior to 1985, there were no laws enacted to screen blood, even though the technology existed. Corporations decided that the deaths of thousands of young men were more cost efficient than the installation and usage of the screening equipment. As a result, many haemophilia patients 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. At a rate of over 90% of the Hemophilia population, over 10,000 people contracted HIV from the tainted blood supply in the United States alone.The first medical professional to describe a disease was Abulcasis. In the tenth century he described families whose males died of bleeding after only minor traumas.[32] While many other such descriptive and practical references to the disease appear throughout historical writings, scientific analysis did not begin until the start of the nineteenth century.
In 1803, Dr. John Conrad Otto, a Philadelphian physician, wrote an account about "a hemorrhagic disposition existing in certain families" in which he called the affected males "bleeders".[33] He recognised that the disorder was hereditary and that it affected mostly males and was passed down by healthy females. 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). Otto was able to trace the disease back to a woman who settled near Plymouth, NH in 1720. The idea that affected males could pass the trait onto their unaffected daughters was not described until 1813 when John Hay published an account inThe New England Journal of Medicine.[34][35]
In 1924, a Finnish doctor discovered a hereditary bleeding disorder similar to Haemophilia localised in the "Åland Islands", southwest of Finland.[36] This bleeding disorder is called "Von Willebrand Disease".
The term "haemophilia" is derived from the term "haemorrhaphilia" which was used in a description of the condition written byFriedrich Hopff in 1828, while he was a student at the University of Zurich.[33][37] In 1937, Patek and Taylor, two doctors from Harvard, discovered anti-haemophilic globulin.[38] In 1947, Pavlosky, a doctor from Buenos Aires, found haemophilia A and haemophilia B to be separate diseases by doing a lab test. This test was done by transferring the blood of one haemophiliac to another haemophiliac. The fact that this corrected the clotting problem showed that there was more than one form of haemophilia.
References
- ↑ "The History of haemophilia". Retrieved 2007-06-27.