Word: hemophilia
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Dates: during 1960-1969
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...generations of European aristocracy. Her son, James I of England, was affected, as are several living European aristocrats who cooperated in the study but asked that they not be identified. In a simliar fashion, Queen Victoria-whose father, the Duke of Kent, showed signs of porphyria-passed hemophilia on to generations of male European royalty...
...prove the point, Dr. Richard J. Hirschman and his colleagues at NIH went back to an old and seemingly cold trail. In 1952-54, a study was made of hemophilia patients who contracted serum hepatitis from injections of an infected blood-clotting factor. The researchers took weekly blood samples but did not find the culprit; so they deep-froze the samples and stored them. In 1968 the 15-year-old samples were thawed out and tested for the Australia antigen. The viruslike particle was found in the blood of 46 (or 74%) of the patients...
...Hemophilia is not just "the disease of kings," although it was so called after Queen Victoria transmitted the deadly trait to Russia's Romanovs and a dozen other royal-blooded descendants. As many as 40,000 Americans, commoners all, are estimated to suffer from the severe, "classical" form of the ailment. Doctors have learned to control most victims' bleeding episodes with transfusions and intravenous injections. But the techniques involved have been complex, cumbersome and costly. Only recently has medical research advanced sufficiently to simplify the process and cope with the problems of supply...
What the hemophiliac's blood lacks, because of a genetic defect transmitted from mother to son, is a clotting protein known as antihemophilic factor (AHF) or globulin (AHG), also called Factor VIII. Because of this deficiency, the hemophilia victim lives in constant danger of severe bleeding from the most minor wound, such as a finger cut or a tooth extraction. Even with no external injury, he may bleed internally after a bump or a stumble. This is especially likely to happen inside his joints, causing arthritis with progressive deformity and disability...
...Shanbrom foresees a day when patients will enjoy still greater convenience. Some batches of AHF concentrate have been made 1,000 times more potent than plasma. Eventually, Brinkhous is confident, this will become the standard AHF, so safe and stable that hemophilia victims will be able to carry it around and inject it themselves, into muscle, just as diabetics now do with insulin...