35th Hemophilia Symposium Hamburg 2004 : Epidemiology;Risk of Infections and Inhibitors in Hemophilia; Chronic Lic Synovitis and Long-term Results of Orthopedic Treatment;Laboratory Diagnostics:Coagulation Factors; Inhibitors, Monitoring;Pediatric Hemosta

This book contains the contribution to the 35th Hemophilia Symposium, Hamburg 2004. The main topics are epidemiology, risk of infections and inhibitors in hemophilia, chronic hemophilic synovitis and long-term results of orthopedic treatment, laboratory diagnostics and pediatric hemostaseology. The volume is rounded off by numerous free papers and posters on hemophilia and hemorrhagic disorders and inhibitors in hemophilia. Contents......Page 6 Johann Lukas Schoenlein Prize 2004......Page 32 I. Epidemiology......Page 35 HIV Infection and Causes of Death in Patients with Hemophilia in Germany (Year 2003/2004 Survey)......Page 36 Hemophilia Registry of the Medical Committee of the Swiss Hemophilia Association – Update and Annual Survey 2004......Page 46 II. Risk of Infections and Inhibitors in Hemophilia......Page 52 Update on the Safety of Clotting Factors, Mainly Regarding the Risk of Transmission of vCJD (Variant Creutzfeldt Jakob Disease)......Page 53 nvCJD and Blood Products in the UK......Page 61 Mutation Type Dependent Inhibitor Risk – a Single Center Study on 432 Patients with Severe Hemophilia A......Page 65 Inhibitor Incidence in Previously Untreated Patients (PUPs) with Hemophilia A and B. A Prospective Multi-Center Study of the Pediatric Committee of the German, Swiss and.........Page 66 ADVATE Inhibitor Risk Profile: 18 Months post-Licensure......Page 70 EACH-Registry: An European Registry for Acquired Hemophilia......Page 74 Elective Orthopedic Surgery for Hemophilia Patients with Inhibitors......Page 76 III. Chronic Synovitis and Long-term Results of Orthopedic Treatment......Page 79 Results after Total Knee and Hip Replacement in Patients with Hemophilia A – A Single Center Experience......Page 80 Motion Analysis Epidemiology in Hemophilic Children......Page 83 Radiosynoviorthesis in Hemophilic Arthropathy – A Single Center Experience......Page 88 IV. Laboratory Diagnostics: Coagulation Factors, Inhibitors, Monitoring......Page 90 Individual Therapy of Hemophilia – New Laboratory Methods Considering Platelets......Page 91 Epitope Mapping during FVIII Inhibitor Elimination with Rituximab Reveals Conformational Epitopes on FVIII and Identifies Small Molecules Blocking Inhibitor and Targeting B Cells......Page 96 Lack of Factor VIII Expression Represents a Novel Mechanism Leading to Hemophilia A......Page 111 V. Pediatric Hemostaseology......Page 115 Effects of the Factor V G1691A Mutation and the Factor II G20210A Variant on the Clinical Expression of Severe Hemophilia A (< 2%) in Children – Results of a Multicenter Study......Page 116 Continuous Infusion of Factor Concentrates in Children with Hemophilia A in Comparison to Bolus Injections......Page 124 Decreased Clotting Factor Activity (VIII, IX, XI, and XII) due to Inhibitors with Lupus-like Activity in Childhood......Page 131 VI. Free Lectures......Page 136 Effect of Activated Recombinant Coagulation Factor VII on the Function of Glycoprotein IIb/IIIa-Inhibited Platelets in Vitro......Page 137 Mutation Analysis in Hereditary Angioedema Identifies Patients at Risk for Developing Acute and Life Threatening Edema......Page 148 Recurrent Mutation in ADAMTS13 Gene as a Cause of a Hereditary Thrombotic Thrombocytopenic Purpura in the Czech Republic......Page 151 Clinical Manifestations of Patients with Dysfibrinogenemia......Page 154 Case Report of a FXIII Inhibitor in a 77-year-old Patient......Page 158 a) Hemophilia and Hemorrhagic Disorders......Page 161 The ABC’s of Hemophilia: German Working Group of Hemophilia Nurses......Page 162 The Long Journey to Being Diagnosed as a Carrier of Hemophilia A – A Woman with Abnormally Prolonged Bleeding after Myocardial Infarction......Page 163 Clinical Investigation of Orthopedic Outcome in Patients with Severe Hemophilia – Advantage of an Early Prophylactic Treatment?......Page 165 The Relevance of Thrombophilic Risk Factors on Bleeding Tendency in Hemophilia A Patients......Page 169 Magnetic Field Therapy in Patients with Severe Hemophilia – Motion Analysis and Quality Control......Page 171 The Clinical Course of two Patients Receiving High Dose Factor VIII – Replacement Therapy......Page 177 Determination of the Factor VIII Plasma Activity of Hemophilia A Patients Treated with a New Recombinant Factor VIII Concentrate......Page 180 Socio-Economic Aspects of Hemophilia Treatment in Romania......Page 184 Immunosuppressive Treatment in Acquired von Willebrand’s Syndrome......Page 192 HCV-Infection in HIV-Infected and Non-Infected People with Hemophilia – A Retrospective Study: Medical Aspects......Page 195 HCV-Infection in HIV-Infected and Non-Infected People with Hemophilia – A Retrospective Study: Psychosocial Aspects......Page 200 Testing Factor VIII Activity by Using the Chromogenic Assay in Carriers of Hemophilia A......Page 206 Hip Replacement in Hemophilic Patients – A 30 Years Single Center Experience......Page 210 b) Inhibitors in Hemophilia......Page 212 Successful Therapy with anti CD20 Monoclonal Antibody Rituximab in Patients with Acquired Hemophilia against Factor VIII......Page 213 Unusual Prolonged Course of an Immune Tolerance Therapy (ITT) in a Patient with Severe Hemophilia A and a High-Titer Inhibitor Development......Page 216 Successful Major Surgery with Minimal Dosage of rFVIIa in a Hemophilia A Patient with High Level of Alloantibodies to Factor VIII......Page 220 Inhibitor Development after Continuous Infusion of Factor VIII: A Retrospective Study in Germany......Page 224 Inhibitors in PTP’S: A Retrospective Study in Germany......Page 227 Elective Orthopedic Surgery in Inhibitor Patients – the Frankfurt Concept......Page 231 c) Clinic and Casuistics......Page 232 The Frequency of Venous Thromboembolism in Women with FV Leiden in Association with Pregnancy and Puerperium......Page 233 d) Diagnostics......Page 237 Spectrum of Molecular Defects and Mutation Detection Date in Patients with Severe Hemophilia A......Page 238 Hemophilia Patients and Prothrombotic Gene Mutation......Page 242 γAla82Gly Represents a Common Fibrinogen Chain Variant in Caucasians......Page 244 A Novel Mutation (Asp36Tyr) in the Vitamin K Epoxide Reductase Complex Subunit 1 Gene (VKORC1) Causes Increased Phenprocoumon Requirement......Page 249 Denaturing High Performance Liquid Chromatography Represents an Efficient Technique for Detection of Heterozygous Large Deletions in Antithrombin Gene......Page 253 e) Miscellaneous......Page 256 Recurrent Coronary Stent Thrombosis in a Patient with Combined Aspirin and Clopidogrel Resistance......Page 257 Coagulation Factor XIII Mutation Profile: Update 2004......Page 261 Site-Directed Mutagenesis of VKORC1, the Target Protein of Coumarin-Type Anticoagulants......Page 264 Treatment of Dilution Coagulopathy by Fibrinogen and Platelet Concentrates......Page 267 Pathogenesis of Hepatic Veno-Occlusive Disease in Patients Undergoing Hematopoietic Stem Cell Transplantation......Page 269 C......Page 281 F......Page 282 J......Page 283 P......Page 284 X......Page 285 Motion analysis as introduced 2 years ago on a national basis effectively identifies individual functional disorders and provides a means for describing them on the basis of a score. Individual therapy planning is possible. More treatment tends to produce better function scores and thus helps to lower the strain on the joint. Further study is required in order to determine whether this reduces the incidence of bleeding. Knee function is age-dependent. Height, weight and sporting activity seem to be influencing factors. Demeanor, pain, fatigue and parental motivation do not seem to have an impact. The roll-and-glide pattern is not age-dependent and probably shows functional abnormalities of the knee. Functional benchmarking of the sites is possible but d- ficult because each site selects the children differently. Age differences also render an overall assessment difficult. Some sites performed negative screening so as to only test children with more severe problems, while other sites performed no such selection. In other sites, the only children to show up for motion analysis were those with well informed parents and who are always involved in all the other activities on offer too Editors, I. Scharrer, W. Schramm ; Scientific Board: I. Scharrer, W. Schramm ; Chairmen: G. Auerswald ... [et Al.]. Presentation: Epidemiology; Risk Of Infections And Inhibitors In Hemophilia, Chronic Synovitis And Long-term Results Of Orthopedic Treatment; Laboratory Diagnosis: Coagulation Factors, Inhibitors, Monitoring; Pediatric Hemostaseology; Free Lectures. Includes Bibliographical References And Index. Contains the contribution to the 35th Hemophilia Symposium, Hamburg 2004. This book also features papers and posters on hemophilia and hemorrhagic disorders and inhibitors in hemophilia. It covers epidemiology, risk of infections and inhibitors in hemophilia, chronic hemophilic synovitis and long-term results of orthopedic treatment, and more.