34th Hemophilia Symposium

I..HIV Infection and Epidemiology

1. HIV Infection and Causes of Death in Patients with Hemophilia in Germany (Year 2002/2003 Survey)
H. Krebs, W. Schramm

2. Hemophilia in Austria — The Annual Survey of the Austrian Hemophilia Centers
H. K. Hartl, P. Bayer, U. Eidher, P. Arends, J. Falger, N. D. Jones, M. Kronawetter, P. Kurnik, I. Pabinger-Fasching, H. Ramschak, E. Reiter, R. Schwarz, W. Streif, H. Türk, H. Wank, W. Zenz, K. Zwiauer

3. Lithuanian Hemophilia Register: Update 2003
V. Ivaskevicius, R. Jurgutis, M. Jurgutis, L. Malciute, J. Schroeder, C. Mueller, E. Seifried, J. Oldenburg

II..Management of Bleedings in Hemophiliacs with Inhibitors

4. Guidelines for Treatment of Patients with Hemophilia and Inhibitors
A. Gringeri

5. Bolus Injection of Recombinant Factor VIIa (NovoSeven) can be More Effective than Continuous Infusion in Inhibitor Patients with Severe Hemophilia A
C. Wermes, H. Pollmann, U. Nowak-Göttl, V. Rolf, M. Depka Prondzinski, K. -W. Sykora

6. The Role of Therapy Regimen and Age at First Exposure on Inhibitor Development in Patients with Severe Hemophilia A
C. Escuriola Ettingshausen, R. Linde, I. Martinez Saguer, A. Zyschka, C. Kessel, H. Stoll, W. Kreuz

7. Presentation of the Inhibitor-Immunology-Study
I. Wieland, C. Wermes, K. Welte, K. -W. Sykora

8. Current Clinical Investigations Involving FEIBA
E. Gomperts, E. Berntorp, J. Astermark, C. Negrier, C. Leissinger, A. Schoppmann, B. Ewenstein

III..Orthopedic Problems and Therapy in Hemophiliacs

9. Major Orthopedic Reconstructions in an Inhibitor Patient — A Case Report
U. Stumpf, C. Eberhardt, M. Krause, W. Miesbach, I. Scharrer, A. A. Kurth

10. Total Hip Replacement in Patients with Severe Bleeding Disorders A 30 Years Single Center Experience
A. A. Kurth, C. Eberhardt, U. Stumpf, I. Scharrer, L. Hovy

11. MRI Staging of Hemophilic Joints in Comparison with the Radiological Score and the Clinical Dates of Children and Young Adults
R. Jentzsch, H. Lenk, D. Weber, F. Schmidt

12. Orthopedic Knee Replacement in Hemophilic Patients
H. H. Wolf, H. Reichel, O. Dorligshaw, H. J. Schmoll

IV..Therapy with Protein C

13. Treatment of Adults with Sepsis-Induced Coagulopathy and Purpura Fulminans with a Plasma-Derived Protein C Concentrate (Ceprotin)
P. Schellongowski, U. Eidher, E. Bauer, P. Schenk, P. Knöbl

14. Comparison of the Anticoagulant Action of Recombinant Human Activated Protein C in Cord with that in Adult Plasma
G. Cvirn, S. Gallistl, M. Köstenberger, K. Baier, P. Fritsch, G. Jürgens, W. Muntean

15. The Relationship between Protein C Activity and the Endogenous Thrombin Potential in Controls and Septic Patients
A. Siegemund, T. Siegemund, U. Scholz, S. Petros, L. Engelmann

V..Pediatric Hemostaseology

16. Antibodies Against Annexin V, Cardiolipin and ß2-Glycoprotein 1 or APC-Resistance in Patients with Recurrent Miscarriage or In-Vitro-Fertilization-Failures
G. Auerswald, H. Lenk, W. M. Halbmayer, W. Feichtinger, C. Kindermann, B. Prendinger, M. Böhm

17. Diagnostics of Platelet Function Disorders by Lumi-Aggregometry — Results and Comparison of Methods
R. Knoefler, G. Siegert, E. Kuhlisch, G. Weissbach

18. Management of a Premature Infant below 1500 g with Hemophilia A
C. Bidlingmaier, A. Garhammer, S. Jenkins, M. Deml, K. Kurnik

19. Elevated Factor VIIIC: Ag in Children with Venous Thrombosis and Stroke — Preliminary Results of a Case-Control Study
R. Schobess, W. Kreuz, K. Kurnik, C. Duering, U. Nowak-Göttl

20. Functional Disorders and Treatment Modalities in Hemophilic Children
A. Seuser, G. Schumpe, H. H. Brackmann, T. Wallny

VI.Free Lectures

21. HLA Profile in Acquired Hemophilia
J. Oldenburg, A. Pavlova, J. Schröder, H. -H. Brackmann, W. Effenberger, R. Zimmermann, A. Huth-Kühne, I. Scharrer, R. Grossmann, E. Seifried, C. Seidl

22. Characterization of Polyclonal Factor VIII-Inhibitory Antibodies
C. Mühle, A. V. Khrenov, S. Schulz-Drost, E. L. Saenko, J. Klinge, H. Schneider

23. Frequency and Inhibitor Risk of the Intron-1-Inversion Mutation in the German Hemophilia Population
J. Schröder, J. Graw, H. -H. Brackmann, B. Kellner, S. Löffler, W. Schramm, R. Schwabb, C. Müller-Reible, J. Oldenburg

24. Effect of FVIII on Clotting Time and Thrombin Potential in Tissue Factor-Activated Hemophilia A Plasma
P. Fritsch, G. Cvirn, K. Baier, B. Leschnik, W. Muntean

VII..Poster

25. Bleeding Tendency in Factor XI Deficiency: Report on two Families and the Detection of a Novel Mutation within the Factor XI Gene
B. Maak, L. Kochhan, J. SchrÖder, J. Oldenburg, P. Heuchel, B. Harwardt, U. Budde

26. Multiple Distal Pseudotumours in a Patient with Severe Hemophilia A and High Titer Inhibitors
C. A. Petrescu, M. Serban, P. Tepeneu, W. Schramm, R. BAdeTi, B. Popescu

27. HIV Resistance to Antiretroviral Therapy in Romania
R. Costa, M. Serban, O. Cristiu, S. Arghirescu, B. Zoica, I. Matziris, W. Schramm, L. GÜrtler

28. Ileopsoas Hemophilic Pseudotumour with Externalized Bowel Fistulation
M. Serban, D. Bordos, M. Siebeck, C. Petrescu, L. Petcana, M. BatAneAnt, A. LAcAtusu, W. Schramm

29. Reduction of Proteoglycan Synthesis in Chondrocytes Depending on Concentration and Duration of Iron Overload
U. Hartl, K. Hochmuth, A. A. Kurth

30. Bleeding Tendency of Carriers of Hemophilia A — Dependent on the Age of the Carriers?
W. Miesbach, J. Oldenburg, I. Stier-Brück, T. Vigh, I. Scharrer

31. Clinical Investigation of Orthopedic Outcome in Patients with Severe Hemophilia Under Prophylactic Treatment. Disadvantage of a late Prophylactic Treatment?
S. Meister, T. Spranger, K. Jersch, G. Auerswald

32. Operative Treatment of Ankle Equinus Deformity in Hemophiliacs
T. A. Wallny, P. Pennekamp, H. -H. Brackmann, P. Hofmann, C. N. Kraft, A. Seuser

33. Spontaneous Empyema of Joints and Staphylococcal Sepsis in a Patient with Severe Hemophilia A
W. Mondorf, L. Hovy, S. Habisreutinger, A. Hümmer

34. The Role of VWF:Ag II in Patients with Acquired von Willebrand Disease
M. Krause, C. Caron, I. Stier-BrÜck, T. Vigh, I. Scharrer

35. Contamination of Coagulation Factor Concentrates with Human Parvovirus Genotype 2 DNA is Less Frequent than Contamination with Genotype 1 (B19) DNA
B. Schneider, M. Becker, U. Reber, H. -H. Brackmann, A. M. Eis-Hübinger

36. The Hemophilia Assistant in Germany
37. “Need” in Hemophilia A — a Qualitative Study
K. Hespe-Jungesblut, M. Depka Prondzinski, T. Schöpke, A. Dörries

38. Fit for Life — Fitness Levels of Young Hemophiliacs Today
A. Seuser, A. Kurme, E. Trunz, P. Böhm, S. Ochs, H. -H. Brackmann, T. Wallny

39. The Austrian “Haemofit-Program” - a two Years work-out Experience of People with Hemophilia (PwH)
H. K. Hartl, T. Kienbacher, H. Ramschak, U. Eidher, M. Fischill, P. Frigo

40. Methods for Testing Pharmacodynamic Variables of Hemophilia and Inhibitor Therapy: Thrombin Generation Assay and Other Tests
K. Váradi, H. P. Schwarz, P. L. Turecek

41. Immune Tolerance in an Inhibitor Patient with Severe Hemophilia A — Comparison of two Different Treatment Schedules Including Rituximab
C. Wermes, I. Wieland, M. Depka Prondzinski, K. W. Sykora

42. The Endogenous Thrombin Potential as a Monitoring Parameter in a Patient with an Acquired Hemophilia A
U. Scholz, A. Siegemund, T. Siegemund, S. Petros, L. Engelmann

43. Case report: 58-Year-Old Hemophilia A Patient with High-Titer Inhibitor Development and Introduction of a Multicenter PTP-Inhibitor Study
Ch. Auer, M. Krause, W. Miesbach, G. Asmelash, I. Scharrer

44. First Data of a Prospective Study About Incidence of Inhibitors During and After Continuous Infusion of Different Factor Concentrates Given During and After Surgical Procedures in Hemophilia A or B and von Willebrand Disease
G. Auerswald, T. Spranger, S. Meister

45. Acquired Thrombophilia in Patients on Hemodialysis with Recurrent Vascular Access Thrombosis
R. Klamroth, O. Eike, F. Seibt, S. Gottstein, H. Rimpler, H. Landgraf

46. Is Travelling a Risk Factor for Venous Thrombosis in Individuals with Factor V Leiden in Heterozygous Form?
P. Dulícek, J. Malý, M. Beránek

47. Popliteal Artery Embolism in Abdominal Aortal Thrombus with Liver Abscesses Caused by Heterozygous Prothrombin Mutation with Protein S Deficiency and Factor VIII Elevation
K. Rifai, I. Schedel, F. Puls, J. Bleck, A. Schneider, M. P. Manns, M. Depka Prondzinski

48. Effect of the new Direct Thrombin Inhibitor Melagatran in Cord and Adult Plasma: an in-vitro Examination
M. Köstenberger, S. Gallistl, G. Cvirn, B. Leschnik, W. Muntean

49. Higher Concentrations of Heparin and Hirudin are Required to Inhibit Thrombin Generation in Tissue Factor-Activated Cord Compared to Adult Plasma
K. Baier, G. Cvirn, P. Fritsch, M. Köstenberger, S. Gallistl, B. Leschnik, W. Muntean

50. Molecular Genetic Analysis in Patients with Inherited Thrombophilia and Antithrombin, Protein C or Protein S Deficiency
A. Pavlova, M. Lim-Eimer, M. Watzka, E. Lindhoff-Last, B. Luxembourg, W. Kreuz, E. Rusicke, E. Seifried, J. Oldenburg

51. Analysis of Thrombophilic Risk Factors in Patients Suffering from Ocular Thrombotic Complications
H. H. Wolf, A. Frühauf, C. Grünauer-Kloevekorn, A. Krause, H. -J. Schmoll

52. Protein C Concentrate for the Treatment of Veno Occlusive Disease in a boy with Nephroblastoma
K. Kentouche, H. J. Mentzel, J. Hermann, F. Zintl

53. Homozygous Factor X Gene Mutation Gly380Arg is Associated to Perinatal Intracranial Hemorrhage
F. H. Herrmann, M. Navarette, L. Salazar-Sanchez, J. M. Carillo, G. Auerswald, K. Wulff

54. Mutation Analysis of the C1 Inhibitor Gene
T. Förster, A. Kocot, J. Schröder, E. Aygören-Pürsün, I. Martinez-Saguer, W. Kreuz, K. Bork, I. Scharrer, C. Müller, J. Oldenburg

55. Novel and Recurrent Mutations in the Gamma-Glutamyl Carboxylase (GGCX) Gene
S. Rost, C. Geisen, A. Fregin, D. Koch, M. Compes, E. Seifried, C. R.Müller, J. Oldenburg

56. First Case of Compound Heterozygous Mutations in the Kininogen Gene Causing Severe High Molecular Weight Kininogen Deficiency
C. Geisen, M. Grünewald, M. Watzka, A. Pavlova, V. Ivaskevicius, E. Seifried, J. Oldenburg

57. Sequence of the rat Factor VIII cDNA
M. Watzka, C. Geisen, E. Seifried, J. Oldenburg

58. Mutation Profiling in Congenital FXIIIA Deficiency: Detection of 6 Novel Mutations
V. Ivaskevicus, R. Seitz, R. Hilgenfeld, T. Sicker, H. -H. Brackmann, W. Eberl, K. Kurnik, U. Göbel, E. Seifried, J. Oldenburg

59. Functional Analysis of the Factor VIII B Domain
O. El-Maarri, J. Schwalbach, U. Herbiniaux, P. Hanfland, J. Oldenburg

60. Expressing Recombinant Coagulation Factors in Yeast and Insect Cells
J. Schwalbach, O. El-Maarri, U. Herbiniaux, P. Hanfland, J. Oldenburg

61. Long Term Optimisation of F8 Gene Mutation Screening by DHPLC
C. Klein, H. Singer, M. Lim-Eimer, M. Watzka, V. Ivaskevicius, J. Schröder, O. El-Maarri, J. Graw, H. -H. Brackmann, W. Schramm, E. Seifried, R. Schwaab, P. Hanfland, C. Müller-Reible, J. Oldenburg

62. Expression Studies of Recombinant FVIII Proteins Exhibiting Mutations in the B-Domain
K. Klempau, H. Singer, C. Klein, H. -H. Brackmann, P. Hanfland, T. Tonn, R. Schwaab, J. Oldenburg

63. Genetic Variability of the Factor VIII Gene in the Normal Population
H. Singer, R. Ahmed, V. Ivaskevicius, M. Watzka, J. Schröder, O. El-Maarri, E. Seifried, R. Schwaab, P. Hanfland, J. Oldenburg

64. Three Novel Microdeletions and the First Insertion / Deletion in Patients with Factor X Deficiency
K. Wulff, M. Gnad, M. Navarette, L. Salazar Sanchez, J. M. Carrillo, A. Batorova, W. Kreuz, R. Strey, F. H. Herrmann

65. SNP Map of the Protein C Gene
A. Pavlova, C. Geisen, M. Lim-Eimer, M. Watzka, E. Seifried, J. Oldenburg

66. Characterization of Factor VIII-Von Willebrand Factor (FVIII-VWF)-Complex Concentrates Under Shear Stress
J. Siekmann, H. P. Schwarz, L. Turecek

67. Procedural Rules of a National Hemophilia Register in Germany
R. Zimmermann, H. -H. Brackmann, M. Depka Prondzinski, H. Lenk, W. Schramm, I. Scharrer

68. Quality Management of Clotting Factor Replacement Therapy in Medical Institutions — Advantage or Obligation
K. H. Beck, M. Mohrmann

69. Proteus Syndrome: Successful Therapy of Severe Migraine Symptoms with low Molecular Weight Heparin
V. Aumann, G. Lutze, A. Neumann, K. Mohnike, U. Mittler

70. Administration of Recombinant Factor FVIIa (NovoSeven, NovoNordisk) in a Patient with Glanzmann Thrombasthenia
A. Zyschka, Z. Elanjikal, C. Escuriola Ettingshausen, I. Martinez Saguer, R. Linde, W. Kreuz

71. Combined Heterozygous Factor XIII-Deficiency in a Family Case Report
E. Rusicke, V. Ivaskevicius, D. Klarmann, C. Escuriola Ettingshausen, J. Oldenburg, W. Kreuz

72. Resistance to Coumarin in a 55-year-old Patient with Chronic Arrhythmia
W. Miesbach, J. Oldenburg, M. Krause, Ch. Auer, I. Scharrer

73. Investigation of the Tendency to Bleeding in Patients with low Activity of Plasminogen Activator Inhibitor-1 (PAI-1)
W. Miesbach, N. Noormalal, T. Vigh, I. Scharrer

74. The Importance of Pre-Analytic Conditions on the Determination of VWF Parameters
S. Täschner, M. Böhm, I. Stier-Brück, G. Asmelash, B. Putz, T. Vigh, C. Rabenstein, V. Meyer, G. Eckert, I. Scharrer

75. Recombinant FVIIa in Chemotherapy Related Thrombocytopenic Bleedings
H. H. Wolf, O. Dorligshaw, H. J. Schmoll

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Keywords: MEDICAL / Public Health MED078000