Familial bleeding disorders affecting males have been described in religious and historical texts for thousands of years. Hemophilia A (classic hemophilia) and B (Christmas Disease) are clinically indistinguishable and discussed together in this chapter. Some of most famous individuals affected with hemophilia have been the descendents of Queen Victoria of England and the Empress of the Indies, including Czar Alexis. The downfall of the Russian aristocracy is partially attributed to the poor health of Czar Alexis and the royal family’s dependence upon their physician, Rasputin, for advice. Analysis of Czar Alexis’ DNA revealed a substitution in exon 4 of the factor IX gene. Schonlein termed the disease hemophilia in the 1820s. In the early twentieth century, experiments by several investigators determined that the plasma and, more specifically, the “globulin fraction” of blood plasma could correct the clotting defect of the blood of affected patients in vivo and in vitro, leading to...
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Yaish, H.M., Chang, E. (2012). Bleeding Disorders. In: Elzouki, A.Y., Harfi, H.A., Nazer, H.M., Stapleton, F.B., Oh, W., Whitley, R.J. (eds) Textbook of Clinical Pediatrics. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-02202-9_335
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