32nd Hemophilia Symposium Hamburg 2001

ISBN-13: 9783540438847 / Angielski / Miękka / 2002 / 318 str.

Scharrer; Inge Scharrer; Wolfgang Schramm

Opis

Seven months following LTX quality of live has increased markedly in the hereby presented patient. A prolonged life expectance can be anticipated due to improved liver function and decreased bleedingtendency as a result of elevated leukocyte and thrombocyte counts. A positive surprise is the patients comment of less joint pain in spite of unchanged hemophilic arthropathy. If this is also true for other haemo- philia patients following LTX this may develop as a new objective towards gene therapy as well. From a clinical perspective bleeding risk ismarkedlyreduced due to nearlynor- mal factor VIII and thrombocyte levels as well as a presumable reduction of blood pressure in esophageal varacies. CD4-Helper cells increased significantly after LTX. However,at the same time a decrease of CD4/CD8 ratio was measured. CD4 count alone may therefore not be appropriate for HIV staging or subsequent decision whether or not immunfunction is sufficient for later LTX. Due to a CD4 count of above 700following livertransplantations the risk of opportunistic infections islow at this time. According to these preliminary results LTX should be considered in a subgroup of haemophilia patients with advanced liver cirrhosis in spite of HIV infection. Dysfibrinogenemia following after Snake Bite M. KRAUSE, J. BOJUNGA, D. MEBS, and I. SCHARRER Introduction A 35 year old snake breeder was bitten on the left foot by a middle American pit viper Crotalus durissus dryinas which had escaped.

Spis treści

I. Epidemiology.- HIV Infection and Causes of Death in Patients with Hemophilia in Germany (Year 2000/2001 Survey).- Hemophilia 2001 — The Annual Survey of the Austrian Hemophilia Centers.- Epidemiology of Hemophilia in Switzerland: A first Insight in the Data Base achieved by the Medical Committee of the Swiss Hemophilia Association.- Overall Blood Supply Strategy with Regard to vCJD.- II. Genetic Diagnosis of Clotting Disorders.- a) Human Genome Project.- 11 novel Mutations in the Factor VIII encoding Gene lead to severe or moderate Hemophilia A.- b) Register and Genetic Diagnosis.- Molecular Analysis of Hemophilia B: »Greifswald Registry FIX Deficiency (Hemophilia B)«.- Gly222Asp and Ser379Lys — Novel Factor X Gene Mutations in severe FX Deficiency — Greifswald Registry of Factor X congenital Deficiency.- c) Gene Therapy.- Hematopoietic Stem Cells as Targets for Gene Therapy of Hemophilia A.- Adenovirus-mediated regulatable Expression of human Factor IX in vitro and in vivo.- III. Hemophilia.- a) Orthopedics.- Experiences with MRI Examination of the Joints of hemophilic Children.- Rhenium-186 Hydroxyethylidenediphosphonate (186Re HEDP) — A novel Treatment for hemophilic Arthropathies?.- b) Monitoring of Substitution Therapy.- Monitoring of Anticoagulant Therapy with the Endogenous Thrombin Potential.- IV. Pediatric Hemostaseology.- First thromboembolic Onset in Children carrying either the heterozygeous FV G1691A Mutation or the Prothrombin G20210A Variant.- UFH Bolus Administration in Comparison to subcutaneous Low Molecular Weight Heparin in pediatrie cardiac Catheterization.- Incidence of Inhibitor Development in consecutively recruited severe Hemophilia A and B Patients — a retrospective Single Centre Study.- V. Free Lectures.- FV Inhibitor and Anti-Phospholipid Antibodies after Treatment with Ciprofloxacin.- Isolated molecular Defects of von Willebrand Factor Binding to Collagen do not correlate with Bleeding Symptoms.- Effects of Tissue Factor Pathway Inhibitor and Antithrombin on Thrombin Generation in Tissue Factor-activated Cord Plasma.- Early and Rapid Diagnosis of acute TTP by Measuring Activity of von-Willebrand Factor Cleaving Metalloprotease (ADAMTS13): A Case Report.- Prions and the Safety of Plasma Proteins: Preventive Measures and Research Activities.- VI. Poster.- a) Clinic and Casuistic.- Transmission of Parvovirus B19 by Heat-treated Coagulation Factor Concentrates.- Course of severe Hemophilia A. Successful Immune Tolerance Therapy (ITT) ten Years after Inhibitor Development.- Increased Resistance to activated Protein C and Protein C Deficiency in the same Family.- A Life-threatening Cardiomyopathy following Port-a-Cath Infection under Immune Tolerance Therapy.- Therapy and Prophylaxis of Bleeding Symptoms in a Patient with Acquired Factor X-Deficiency due to Systemic Amyloidosis (AL-Amyloidosis).- Is there a Correlation between vWF-cleaving Protease-Activity, vWF:Ag, Clinical Course and Number of Relapses in 15 Patients with TTP?.- Life-Threatening Hemorrhage in a Patient with Red Cell Antibodies — Effective Blood Coagulation with rFVIIa.- Liver Transplantation in a HIV/HCV coinfected Hemophilia A Patient.- Dysfibrinogenemia following after Snake Bite.- Bleeding Complications following Tooth Extraction in a Hemophilia A Patient with Inhibitor — A Case Report.- Cerebral Sinus Thrombosis: Recanalization after intravenous Dalteparin Administration.- b) Hemophilia and Hemorrhagic Disorders.- Treatment of FVIII-Autoantibodies by Protein A-Based Immunoadsorption and Immunosuppression: A Regimen without FVIII Substitution.- Factor XI Deficiency caused by a hitherto unknown Mutation in the Factor XI Gene.- Polymorphisms in FV Gene associated with FV Deficiency — First Results.- Influence of Phospholipids of the Platelet Membrane of Newborns on the Thrombin Generation.- Socio-economic Evaluation of Hemophilia Assistance.- Aspects regarding Locomotor Rehabilitation of Hemophiliacs.- c) Thrombophilic Disorders.- Prothrombin and Factor VII Genotypes and Phenotypes in healthy Individuals. Results from the Lugen Study.- Factor V Leiden and Other thrombotic risk factors in CHD and myocardial Infarction.- In vitro Effects of combined Administration of Epitifibatide and Anticoagulants on Thrombin induced Platelet Aggregation after high versus low Coagulant Activation of Platelet Rich Plasma.- Cardiac and cerebral Manifestations of the Antiphospholipid Syndrome.- d) Diagnosis.- Inactivation of Animal Factor VIII by human Factor VIII Inhibitors: Special Methodological Features in Performing the Factor VIII Assay and the Bethesda Assay.- Inactivation of Animal Factor VIII by human Factor VIII Inhibitors: Investigation of Plasma from Patients with Inhibitors in congenital Hemophilia A and from Patients with acquired Hemophilia A.- Factor VIII:C Measurement — Comparison between Chromogenic and Coagulometric Methods in Hemophilia A — Patients with the B Domain-depleted Recombinant F VIII-Preparation ReFacto.- Functional Assessment of fibrinolytic Resistance in whole Blood.- e) Miscellaneous.- Quality Control of Platelet Concentrates during Storage Using Different Forms of Agitation Measuring the Platelet Activation.- Platelet Activation before and after Cryopreservation of Platelet Concentrates with a New Storage Solution.- Flow Cytometric Measurement of CD34+ Cells: How reliable are absolute Cell Counts generated by the Integration of Beads?.- An Innovative Approach to Teach and Learn Diagnostic Skills and Therapeutical Management of Coagulation Disorders: CAMPUS — an Interactive, Computer- and Case-Based Program.- Expression of Protease-activated Receptors in Neuroblastoma Cells.