36th Hemophilia Symposium Hamburg 2005 : Epidemiology; Hemophilia Therapy - Management of Bleedings and Inhibitors; Orthopedic Treatment in Hemophiliacs; Hemostaseologic Diagnosis; Pediatric Hemostaseology; Free Lectures

This book contains the contribution to the 36th Hemophilia Symposium, Hamburg 2005. The main topics are epidemiolgy, hemophilia therapy, orthopedic treatment in hemophiliacs, hemostaseologic diagnosis and pediatric hemostaseology. The volume is rounded off by numerous free papers and posters on hemophilia, inhibitors in hemophilia and diagnostics. Front Matter....Pages I-XXXIV Front Matter....Pages 1-1 HIV Infection and Causes of Death in Patients with Hemophilia in Germany (Year 2004/2005 Survey)....Pages 3-14 Development of the German Hemophilia Register....Pages 15-15 National Hemophilia Registry - Source of Information about the Quality of Hemophilia Medical Care in a Developing Country....Pages 16-20 Front Matter....Pages 21-21 Regulation of Factor VIII Life-Cycle by Receptors from LDL Receptor Superfamily....Pages 23-33 Update of the Inhibitor-Immunology-Study....Pages 34-39 Therapy of Acquired Hemophilia - Immunoadsorption and Rituximab Treatment for Immunosuppression and Substitution of Coagulation Factors....Pages 40-47 Front Matter....Pages 49-49 Osteoporosis and Hemophilia - Is there a Correlation and Is there a Problem?....Pages 51-53 Orthopedic Evaluation of the Lower Extremity in 249 Children: A Multicenter Trial....Pages 54-56 Simultaneous Bilateral Total Knee Arthroplasty in Hemophilic Arthopathy....Pages 57-62 Alternative Therapy such as the Acupuncture of the Skull for Hemophilic Arthropathy....Pages 63-66 Endoprosthetics of Large Joints in Patients Infected with HIV....Pages 67-67 Front Matter....Pages 69-69 Factor VIII Methods: Which Assay Principle for which Indication?....Pages 71-74 Global ECAT Survey on Factor VIII Inhibitor Testing: Results of an Inter-Laboratory Pilot Study....Pages 75-77 A Practical Concept for Pre-Operative Identification and Improved Management of Patients at Risk for Bleeding....Pages 78-89 Front Matter....Pages 91-91 Intravascular Tissue Factor in Cord vs Adult Whole Blood....Pages 93-95 Perioperative Coagulation Screening in Children - Reasons and Results....Pages 96-100 Thrombin Generation in Children....Pages 101-107 Front Matter....Pages 109-109 Working-Group of the German Hemophilia Assistants - History, Purpose and Goals....Pages 111-113 The Endogenous Thrombin Potential as a New Parameter for the Peri-Operative Monitoring in Conjunction with Endo-Prosthetic Supply due to Hemophilic Arthropathy....Pages 114-117 Identification of Inhibitor Epitopes in Acquired Hemophilia by Phage Display....Pages 118-128 Front Matter....Pages 109-109 Spectrum of Molecular Defects and Mutation Detection Rate in Patients with Mild and Moderate Hemophilia A....Pages 129-132 Recombinant Factor VIIa for Major Surgery in Severe Factor XI Deficiency: Pharmacodynamic Monitoring Using Thromboelastometry....Pages 133-136 Front Matter....Pages 137-137 Establishment of a Web-Based Documentation System for Quality Assurance of Hemophilia Treatment in Mecklenburg/Western Pomeranian: a Pilot Study....Pages 139-139 Successful Angiographic Embolization of Recurrent Elbow Joint Bleeds in one Patient with Severe Hemophilia A....Pages 140-143 Evaluation of Thrombotic Events in Hemophiliacs Undergoing Major Orthopedic Surgery Without Thrombosis Prophylaxis....Pages 144-146 Motivating Patients and Parents to Document Treatment Correctly....Pages 147-149 Fit for Life Competition: Everyone’s a Winner....Pages 150-152 Blood Borne Infections in Hemophiliacs in a Developing Country. A Single Center Experience....Pages 153-158 Hemophilia Center Frankfurt - Twinning Center for Nairobi/Kenya....Pages 159-160 Economic Evaluation of Orthopedic and Surgical Interventions in Hemophiliacs - Not Only Direct Medical Costs Matter....Pages 161-167 The Occurrence of Factor VIII Inhibitor in a Patient with Mild Hemophilia A - During Treatment with Interferon for Chronic Hepatitis C....Pages 171-172 Severe Hemophilia A Patient with High-Titer Inhibitor, use of TGA in the Monitoring of Bypassing Therapy....Pages 173-174 EUREKA - an European Registry for Orthopedic Surgery in Hemophiliacs with Inhibitors....Pages 175-175 Double Balloon Enteroscopy (DBE) with Argon Plasma Laser Coagulation (APC) for a Patient with Heyde’s Syndrome....Pages 179-183 Successful Liver Transplantation in a Patient with Anti-Thrombocyte Antibodies and Severe Hemophilia A....Pages 184-186 Peri- and Postoperative Course of 95 Patients with von-Willebrand’s Disease....Pages 187-189 Pregnancy in a Patient with Congenital Antithrombin Deficiency....Pages 190-194 Successful Inhibitor-Elimination with Rituximab in Acquired Hemophilia A and a Patient with a Carrier Status for Hemophilia A: Two Case Reports....Pages 195-199 An Interesting Family Case of von-Willebrand-Syndrome....Pages 200-200 Clinical Manifestations of Dysfibrinogenemia in Relation to the Fibrinogen Gene Mutation....Pages 201-202 Front Matter....Pages 137-137 Thrombin Generation in a Hemophilic Newborn....Pages 203-209 Rapid and Sensitive Detection of Heterozygous Deletions of one or more Exons in Hemophilia A Females by Multiplex PCR and DHPLC Technique....Pages 213-216 Molecular Genetic Analysis in Patients with Inherited Factor V Deficiency....Pages 217-219 Expression Analysis of C1-Inhibitor Mutants Confirms Causality of Missense Mutations for Hereditary Angioedema....Pages 220-223 Expression of the γ-Glutamyl Carboxylase (GGCX) Containing the Arg485Pro Mutation Found in two Unrelated VKCFD1 Patients....Pages 224-228 Concentration of Soluble Endothelial Protein C Receptor (EPCR) in Plasma in Relation to Age, Sex, BMI and Hemostasis Parameters....Pages 229-234 Thrombin Generation is Age-Dependent in Children as well as in Adults....Pages 235-239 Effects of PFA-100 in Preoperative Screening for von Willebrand Disease in 310 Patients....Pages 240-242 Control of Aspirin Effect in Chronic Cardiovascular Patients Using two Whole Blood Platelet Function Assays: PFA-100 and Multiple Electrode Aggregometry....Pages 243-251 Difficulties in the Interpretation of the Term »Patient-Related« in the Scope of the Introduction of a Remuneration of Additional Payments According to the OPS....Pages 255-256 Factor VIII as Positive Regulator of Activated Platelets....Pages 257-262 Characterization of Three Novel Mutations in the Sodium Binding Site of Coagulation Factor X....Pages 263-271 Characterization of a Mutation in the 5′ Flanking Region and a Novel IVS7 Splice site Mutation in a Patient with Severe FVII Deficiency....Pages 272-278 On the Molecular Basis of Warfarin Resistance in Rats....Pages 279-283 Influence of Factor V HR2 on Endogenous Thrombin Potential and Clinical Phenotype in Factor VII Deficiency....Pages 284-290 Splice Site Mutations Effect on the F8 mRNA Splicing....Pages 291-294 Species-Specific Variation of VKORC1-Activity and Resistance to Warfarin....Pages 295-297 Various Missense Mutations in the Vitamin K Epoxide Reductase Complex Subunit 1 (VKORC1) Cause Hereditary Coumarin Resistance....Pages 298-301 Establishment of an International Registry of Patients with Congenital FXIII Deficiency....Pages 302-304 The Impact of Freezing of Plasma Samples, AB0 Blood Group and Acute-Phase Reaction on Detecting Mild Factor VIII Deficiency and Increased Factor VIII Levels as a Risk Factor for Venous Thromboembolism....Pages 305-309 Front Matter....Pages 137-137 Thrombin Generation in Severely Obese Children....Pages 310-314 Back Matter....Pages 315-320