Inhibitors to Coagulation Factors, Volume 286

Front Cover
Louis M. Aledort, Leon W. Hoyer, Jeanne M. Lusher, Howard M. Reisner, Gilbert C. White II
Springer Science & Business Media, 1995 - Medical - 308 pages
"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.
 

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Contents

Factors VIII IX and von Willebrand factor their molecular and antigenic structure An overview
1
Structure and function of Factor VIII
3
Molecular etiology of factor VIII deficiency in hemophilia A
19
The incidence of factor VIII inhibitors in patients with severe hemophilia A
35
Epitope specificity and functional characterization of factor VIII inhibitors
47
Immunogenetics of the human immune response to factor VIII
65
Molecular structure epitopes and mutations associated with inhibitor formation
79
Antibodies to von Willebrand factor in von Willebrand disease
87
An hypothesis
157
Clinical update on the use of recombinant factor VII
163
Apheresis
175
IVIg in the treatment of patients with factor VIII inhibitors
185
Tolerance to coagulation factors
191
A clinical perspective
193
The international registry data
201
Novel approaches for unresponsiveness
209

The molecular basis of the human immune response
93
Fact and fiction
95
Antigen analogs as therapeutic agents
109
Genetic variation and the immune response
119
Tcell receptors in immune responses
121
Immunoglobulin heavy chain variable region gene usage in human autoimmune diseases
133
Treatment of inhibitors
141
Porcine factor VIII therapy in patients with factor VIII inhibitors
143
Clinical presentation and management of patients with circulating anticoagulants
153
New approaches to the inhibitor problem
221
Variable V regionmediated control of autoreactivity to factor VIII
223
Hepatic gene therapy for hemophilia B
229
Who and why?
235
Today and tomorrow
247
Poster Session Abstracts
263
Index
299
Copyright

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