Gilbert C. White II – författare

These are the proceedings of the international symposium to honor Dr. Harold R. Roberts, held in Chapel Hill, North Carolina in April of 1999. The volume covers prions and viral transmissions, unexplained aspects of hemophilia and hemostasis, novel approaches to therapy, and inhibitor development in hemophilia patients.

"For the blood is the life . . . . "(Deut. 12 :23) " . . . because the blood, in its value as life, makes atonement" (Lev. 17: 11) HemoPhilia is a rare disease, severe hemophilia rarer still, yet the written history of hemophilia extends back over a millennium and a half. In the ancient Middle East, blood and life were coupled. Blood was the primary substance necessary for life, given to God in sacrifice and forbidden as a food to mortals by Levitical law. Blood was essential for rites of purification and consecration. But the flow of blood during menstruation or parturition rendered a woman unclean. The circumcision of a male child required 33 days of "blood purification" by the mother. '' Circumcision, the visible reminder of the covenant of Abraham lijith Yahweh, was required of newborn Jewish males. It "connote(d) suitability for participation in what God is doing. "2 Hence, free and uncontrolled bleeding of the male child during circumcision, during the ratification of God''s covenant, would be noted with awe and concern by those of the Jewish faith. It should not be surprising that the first genetic counseling offered to families with hemophilia is found in the Babylonian Talmud (compilation of Jewish law dated to about the third century AD) and concerns the necessity for circumcision in families with what we would now call hemophilia.

There should be, and in the best of cases there is, a synergy between basic research and patient care. However, this synergy is hard to develop because the techniques required to be a successful researcher are so different from the skills required to be an outstanding physician. Harold R. Roberts, M.D., of the University of North Carolina at Chapel Hill, is an example of a physician-researcher who has benefited from having his feet in both the world of patient care and the world of the laboratory: he has let clinical problems direct his basic research effort and conversely has adopted research advances in his care of patients. Dr. Roberts's long and continuing career has included many research and clinical advances. He was part of the first group to determine the amino acid sequence of the important thrombin inhibitor hirudin and part of the group that prepared the first cryoprecipitates which were the first alternative to plasma as therapy in hemophilia A. Dr. Roberts has made significant advances in understanding the protein chemistry behind hemophilia B; he was among the first researchers to identify some patients as not being completely deficient but instead as having measurable levels of protein and subsequently demonstrated that this protein was dysfunctional. This important advance led him to a classification scheme for patients into Cross Reacting Material (CRM) positive, negative, and reduced. Dr.

"For the blood is the life . . . . "(Deut. 12 :23) " . . . because the blood, in its value as life, makes atonement" (Lev. 17: 11) HemoPhilia is a rare disease, severe hemophilia rarer still, yet the written history of hemophilia extends back over a millennium and a half. In the ancient Middle East, blood and life were coupled. Blood was the primary substance necessary for life, given to God in sacrifice and forbidden as a food to mortals by Levitical law. Blood was essential for rites of purification and consecration. But the flow of blood during menstruation or parturition rendered a woman unclean. The circumcision of a male child required 33 days of "blood purification" by the mother. ' Circumcision, the visible reminder of the covenant of Abraham lijith Yahweh, was required of newborn Jewish males. It "connote(d) suitability for participation in what God is doing. "2 Hence, free and uncontrolled bleeding of the male child during circumcision, during the ratification of God's covenant, would be noted with awe and concern by those of the Jewish faith. It should not be surprising that the first genetic counseling offered to families with hemophilia is found in the Babylonian Talmud (compilation of Jewish law dated to about the third century AD) and concerns the necessity for circumcision in families with what we would now call hemophilia.

There should be, and in the best of cases there is, a synergy between basic research and patient care. However, this synergy is hard to develop because the techniques required to be a successful researcher are so different from the skills required to be an outstanding physician. Harold R. Roberts, M.D., of the University of North Carolina at Chapel Hill, is an example of a physician-researcher who has benefited from having his feet in both the world of patient care and the world of the laboratory: he has let clinical problems direct his basic research effort and conversely has adopted research advances in his care of patients. Dr. Roberts''s long and continuing career has included many research and clinical advances. He was part of the first group to determine the amino acid sequence of the important thrombin inhibitor hirudin and part of the group that prepared the first cryoprecipitates which were the first alternative to plasma as therapy in hemophilia A. Dr. Roberts has made significant advances in understanding the protein chemistry behind hemophilia B; he was among the first researchers to identify some patients as not being completely deficient but instead as having measurable levels of protein and subsequently demonstrated that this protein was dysfunctional. This important advance led him to a classification scheme for patients into Cross Reacting Material (CRM) positive, negative, and reduced. Dr.