Riassunto:
These proceedings are of a symposium held jointly by the UK Haemophilia Centre Directors and the Royal College of Physicians and Surgeons of Glasgow. The purpose of the meeting was to highlight the growing areas of haemophilia care and research as they serve as a model for the study of other disorders. In particular a major section of these proceedings is devoted to the investigation of liver disease in haemophili- an area which offers unique opportunities for both basic, applied and clinical research. The second section considers modern treatment of bleeding disorders and the potential cost to society. With the advent of better standards of care, the requirements of plasma products have risen to such an extent that future predictions suggest a worldwide shortage may occur. The third section of the book discusses the detailed structure of the Factor VIII molecule and its sub-compon(!nts and also its functional and immunological characteristics. The availability of amniocentesis and its accuracy in predicting which factor is affected has produced new problems for genetic counselling. The fourth section is clinical and describes the experience of procedures in the Nuffield Department of Orthopaedics, Oxford. For all of us looking after such patients this remains the most important unsolved cI inical problem. C. D. Forbes G. D. O. Lowe vii List of Contributors Professor Charles Abildgaard, Dr. Geijlswijk, Department of Paediatrics, Department of Haematology, 4301 X Street, University Hospital, Sacramento, Utrecht, California 95817 The Netherlands Dr. L.
Contenuti:
Section I Liver Disease in Haemophilia.- Opening remarks.- 1 The epidemiology of Factor VIII and IX associated with hepatitis in the UK.- Discussion.- 2 Viruses causing hepatitis.- Discussion.- 3 Clinical, immunological and histological aspects of non-A, non-B hepatitis in haemophiliacs.- Discussion.- 4 Liver biopsy in non-A, non-B hepatitis.- 5 Experience of liver disease in haemophilia.- Discussion.- 6 The development of hepatitis B vaccines and antiviral therapy.- Discussion.- General Discussion.- Section II Therapy for Haemophilia.- Opening remarks.- 7 The cost of care.- Discussion.- 8 The effect of activated prothrombin-complex concentrate (FEIBA) on joint and muscle bleedings in patients with haemophilia A with an inhibitor to Factor VIII.- Discussion.- 9 Therapeutic use of DDAVP in haemophilia and von Willebrand’s disease.- Discussion.- 10 Polyelectrolytes and preparation of Factor VIIIC.- Discussion.- 11 Oral administration of Factor VIII in lipid vesicles.- Discussion.- 12 Use of activated prothrombin complex in Factor VIII inhibitors.- Discussion.- 13 Immune complex disease in haemophilia.- Discussion.- Section III Factor VIII — Structure, Function and Genetics.- 14 Factor VIII — structure and function.- Discussion.- 15 Factor VIIIC — immunology and activity.- Discussion.- Additional Discussion of Chapters 14 and 15.- 16 Genetics of Factor VIII.- Discussion.- 17 Prenatal diagnosis of haemophilia A and Christmas disease.- Discussion.- Section IV Orthopaedic Problems.- 18 Joint surgery in haemophilia.- Discussion.
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