31st Hemophilia Symposium Hamburg 2000: Epidemiology Inhibitors in Hemophilia Therapy and Monitoring of Bleeds in Acute and Intensive Care Medicine Pediatric Hemostaseology Case Reports

303 Pages·2002·15.33 MB·English

by  I. Scharrer (auth.)

Springer
Berlin
Heidelberg
New York
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London
Milano
Paris
Tokyo
31st Hemophilia Symposium
Hamburg 2000
Editors: I. SCHARRER, W. SCHRAMM
Presentation:
Epidemiology
Inhibitors in Hemophilia
Therapy and Monitoring of Bleeds
in Acute and Intensive Care Medicine
Pediatric Hemostaseology
Case Reports
Scientific Board:
I. SCHARRER, Frankfurt/Main
W. SCHRAMM, Munich
Chairmen:
G. AUERSWALD (Bremen); H.-H. BRACKMANN (Bonn); W. KREUZ (Frankfurt/Main)
H. LENK (Leipzig); E.O. MElLI (Ziirich); I. SCHARRER (Frankfurt/Main)
R. SCHNEPPENHEIM (Hamburg); R. SEITZ (Langen); A.H. SUTOR (Freiburg)
R. ZIMMERMANN (Heidelberg)
Springer
Professor Dr. med. INGE SCHARRER
Hemophilia Center, Dept. of Internal Medicine
University Hospital
Theodor-Stern-Kai 7
D-60590 Frankfurt am Main
Germany
Professor Dr. med. WOLFGANG SCHRAMM
Dept. of Hemostasiology
University Hospital
Ziemssenstr. la
D-80336 Miinchen
Germany
Mit 107 Abbildungen
ISBN-13:978-3-540-42131-3
Library of Concress Cataloging-in-Publication Data
31St Hemophilia Symposium: Hamburg, 2000 1 editors, I. Scharrer, W. Schramm.
p.;cm.
Includes bibliographical references and index.
ISBN -13:978-3-540-42131-3 e-ISBN -13: 978-3-642-59383-3
DOl: 10.1007/978-3-642-59383-3
1. Hemophilia-Congresses. I. Title: Thirty-first Hemophilia Symposium. II. Scharrer,
I. III. Schramm, W., 1943-
[DNLM: 1. Hemophilia A-tlIerapy-Congresses. 2. Disease
Susceptibility-Congresses. 3. Hemophilia A-epidemiology-Congresses. 4.
Hemostasis-Child-Congresses. WH 325 H228z 2002]
RC642, H355 2000
616.1'572-dc21
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Contents
Presentation of the Johann-Lukas-Schoenlein-Award . . . . . . . . . . . . . 1
I. Epidemiology
HIV Infection and Causes of Death in Patients with Hemophilia in Germany
(Year 199912000 Survey)
w.
SCHRAMM, H. KREBS, on behalf of the GTH Hemophilia Committee 7
Hemophilia 2000 - the Annual Survey of the Austrian Hemophilia Centers
H.K. HARTL, U. EIDHER, U. KUNZE, P. ARENDS, J. FALGER, N.D. JONAS,
M. KRONAWETTER, P. KURNIK, I. PABINGER, H. RAMSCHAK, E. REITER,
R. SCHWARZ, W. STREIF, H. TURK, H. WANK, W. ZENZ, K. ZWIAUER . . . 15
II. Inhibitors in Hemophilia
Changes in Epitope Specificity and in Distribution of FVIII Antibodies
during Immune Tolerance Therapy (ITT) in Hemophilia A Patients
with FVIII Antibodies - a Case Report
A. KALLAS, T. TALPSEP, H. EVERAUS . . . . . . . . . . . . . . . . . . . . . . . 23
Clinical Experience with the Modified Bonn-Malmo Protocol since 1996
1. HESS, C. UNKRIG, H. ZEITLER, W. NETTEKOVEN, W. EFFENBERGER,
P. HANFLAND, H. VETTER, H.-H. BRACKMANN ................ 41
Course of Inhibitors in mild Hemophilia A
with and without Immune Tolerance Treatment
H. LENK, F. KERTZSCHER .... . . . . . . . . . 47
III. Therapy and Monitoring of Bleeds in Acute-and Intensive Care Medicine
Management of Bleeding in Surgery and Intensive Care
A. CALATZIS, W. SCHRAMM, M. SPANNAGL ........... . 55
VI Contents
IV.. Pediatric Hemostaseology
Frequency and Profile of viral posttransfusional Infections in Patients from
Hemophilia Center Timisoara
M. ~ERBAN, C. PETRESCU, P. TEPENEU, M. POP, S. JENARU, W. SCHRAMM . 69
Symptomatic Onset of severe Hemophilia A in Childhood is dependent
on the Presence of Prothrombotic Risk Factors
S. HALIMEH, C. EscuRIOLA ETTINGSHAUSEN, K. KURNIK, R. SCHOBESS,
C. WERMES, H. POLLMANN, W.D. KREUZ, U. NowAK-GoTTL 77
Differences between Neonates and Adults in Plasmin Inhibitory
and Antifibrinolytic Action of Aprotinin
M. RIEs, M. ZENKER, J. KLINGE .................. . 81
Shorter PFA-100 Closure Times (CT) in Neonates than in Adults:
Role of Red Cells, White Cells, Platelets, and VOn Willebrand Factor
B. ROSCHITZ, K. SUDI, W. MUNTEAN ................. . 91
V. Free Lectures
Efficacy and Safety of a High Purity Protein C Concentrate
in the Management of Patients with severe Congenital Protein C Deficiency
B. MORITZ, S. ROGY, S. TONETTA, H.P. SCHWARZ, H. EHRLICH and the
CEPROTIN Study Group . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10 1
Respective Roles of Factors II, VII, IX, and X in the Pro coagulant Activity
ofFEIBA
S. GALLISTL, G. CVIRN, B. LESCHNIK, W. MUNTEAN ........... 110
Capillary Microscopic and Rheological Dimensions for the Diagnosis
of VOn-Willebrand-Disease in Comparison with other Hemorrhagic Diatheses
J. KOSCIELNY, S. MORSDORF, C. MROWIETZ, H. KIESEWETTER, U. BUDDE,
U.T. SEYFERT, E. WENZEL, E JUNG ..................... 114
Evaluation of Denaturing High Performance Liquid Chromatography
(DHPLC) in the Analysis of Hemophilia A
J. OLDENBURG, O. EL-MAARRI, V. IVASKEVICIUS, S. RosT, B.H.E WEBER,
R. SCHWAAB ..................................... 130
Via. Poster: Clinic and Casuistic
Rheumatoid Arthritis in a Patient with Hemophilia Arthropathy -
a Case Report
H.H. EICKHOFF, G. RADERSCHADT, EW. KOCH, T. WALLNY, H.-H. BRACKMANN 143
Contents VII
Recurrent Fatal Intracranial Hemorrhage (ICH) in 1Wo Non-Identical1Wins
suffering from Hemophilia B (Factor IX Activity <1 %)
S. HALIMEH, H. POLLMANN, M. KXSE, I. HORNIG-FRANZ, H. JURGENS,
U. NOWAK-GOTTL . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 149
Progression of Thrombosis under Low Molecular Weight Heparin without
Heparin-Induced Thrombocytopenia in a young Man: a Case Report
R. KNOFLER, J. WENDISCH, K. NEMAT, G. SIEGERT, S. GEHRISCH, S. SCHELLONG 153
Cardiac Tamponade in a Patient with acquired Factor VIII Inhibitor
and Chronic Renal Failure
E. STRASSER, Y. LINDEMANN, B. NEIDHARDT, I. SCHARRER, R. ZIMMERMANN,
V. WEISBACH, R. ECKSTEIN . . . . . . . . . . . . . . . . . . . . . . . . . . .. 159
Endoscopic Cholecystectomy in a 55-year-old Patient with Heparin-Induced
Thrombocytopenia Type II and Replacement of Mitral and Aortic Valve and
Tricuspidal Valve Anular Plasty
J. GROSS, D. HEITMANN, I. WOJTZYK, G. LUTZE, M.U. HElM . . . . . . . . .. 161
Vlb. Poster: Hemophilia
Successful Orthopedic Operations in Hemophilic Adults
with Inhibitors against Factor VIII
G. LUDWIG, C. BETZ, M. KRAUSE, A. KURTH, L. Hovy, I. SCHARRER ...... 169
Evaluation of Clinical Efficacy of rFVIIa in Pediatrics
M. ~ERBAN, P. TEPENEU, C. PETRESCU, D. MIHAILOV, C. JINCA, W. SCHRAMM . 171
Risk Factors for Thrombosis in Hemophilia - an Analysis
V. AUMANN, G. LUTZE, U. MITTLER ............ . . ...... 179
Status of Pain in Patients with severe Haemophilic Arthropathies
T. WALLNY, L. HESS,A. SEUSER, H.-H. BRACKMANN, C.N. KRAFT . 181
Results of a 5-year Clinical Study with a B-domain Deleted FVIII Concentrate
(rFVIII-SQ)
H. POLLMANN, H. RICHTER and the rFVIII-SQ Study Group . . . . . . . . .. 186
Outpatient Treatment with Radiosynoviorthesis in Hemophilic Arthropathy
D. LASOVIC, G. TONSHOFF, M. FINK, A. ROSENSTOCK, A. GANSER,
M. VON DEPKA PRONDZINSKI ........................... 197
Requirements for Future Hemophilia Treatment
from the Patients' Point of View
G. SCHELLE, W. BREUER, G. BROIL, D. HOHMANN ................ 199
VIII Contents
Vic. Poster: Hemorrhagic Diathesis
Molecular Basis of von Willebrand Disease Type IIC Miami
R. SCHNEPPENHEIM, T. OBSER, J.U. WIEDING, F. BERGMANN, E. DREWKE,
B. LUXEMBURG, U. BUDDE ••••••••••••••••••••• • •• 203
Hemorrhagic Diathesis through acquired Factor XIII Inhibitor
E. STRASSER, R. ZIMMERMANN, B. NEIDHARDT, B. MANGER, I. SCHARRER,
V. WEISBACH, R. ECKSTEIN • • • • • • • • • • • • • • • • • • • • • • • • • • • 208
Liver Transplantation in a Patient with severe von Willebrand Disease Type 3:
Levels of von Willebrand Factor Following Transplantation
E. LECHLER, D. STIPPEL • • • • • • • • • • • • • • • • • • • • • • • • • • • • • •• 210
Clincal Course and Laboratory Findings in a Patient
with a New Mutation Causing Wiskott-Aldrich Syndrome
F. KERTZSCHER, A. MEINDL, V. SCHUSTER, U. SACK, M. KOKSCH, H. LENK • •• 216
Role of acquired and inherited Prothrombotic Risk Factors
in Pediatric Cerebral Venous Thrombosis -
Preliminary Results of a Multicenter Case-Control Study
A. KOSCH, U. SCHULZE-HoRSEL, C. HELLER, R. SCHOBESS, K. KURNIK,
R. STRATER, U. NOWAK-GOTTL • • • • • • • • • • • • • • • • • • • • • •• 219
Analysis of the Fibrinogen Genes of 40 Patients with Suspicion
of Dys-, Hypo-or Afibrinogenemia
A. CZWALINNA, N. BARTKOWIAK, B. CANEPA, A. GANSER,
M. VON DEPKA PRONDZINSKI ••••••••••••••••••••••••••• 225
Vld. Poster: Thrombophilic Diathesis
Hereditary Antithrombin Deficiency - Results of a Family Study
B. MAAK, S. HUTSCHENREITER, F.H. HERRMANN, K. WULFF, A. SIEGEMUND,
W. CHRISTOPH • • • • • • • • • • • • • • • • • • • • • • • • • • • • • • • • • • •• 233
Elevated Factor IX and Factor XI as Risk Factors
for Venous Thrombosis and Stroke
A. SIEGEMUND, T. SIEGEMUND, J. BERROUSCHOT, H. VOIGT, M. KOKSCH ... 239
Thrombophilic Risk Parameters in Juvenile »Idiopathic« Stroke Patients
M. KRAUSE, S. EHRENFORTH, E. AYGOREN-PURSUN, G. LUDWIG, I. SCHARRER 244
Inquiry into the Significance of Constantly Raised FVIII Values
as a Factor in Thrombophilia
M. VON LIEVEN, M. KRAUSE, T. VIGH, C. RABENSTEIN, I. SCHARRER • • • • •• 252
Contents IX
Do Statins Increase the Homocysteine Level?
M. VON LIEVEN, L. ROSSIG, T. VIGH, C. RABENSTEIN, I. SCHARRER . 257
Alpha 2-Macroglobulin Level regulates the Anticoagulant Cofactor Activity
of Protein S in Cord and Adult Plasma
G. CVIRN, S. GALLISTL, M. KOSTENBERGER, J. KUTSCHERA, B. LESCHNIK,
W. MUNTEAN ..•.••••••..•••••.•••••••••.•••••••• 262
Combined Prothrombotic Defects and Contraceptives -
Risk Factor for Deep Venous Thrombosis in Adolescence
R. SCHOBESS, A. JASSOY, S. BURDACH ••••..••••.• 273
Role of 23 bp Insertion in Exon 3 of the Endothelial Cell Protein C Receptor
Gene in Venous Thrombophilia
M. VON DEPKA PRONDZINSKI, A. CZWALINNA, R. EISERT, C. WERMES,
B. CANEPA, I. SCHARRER, A. GANSER, S. EHRENFORTH . . . • • . • • • . . . . • 278
Subject Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 283
List of Participants
ALBERIO, L., Dr.
CH-Bern
ALBERT, T.
Institut fur Experimentelle Hamatologie und Transfusionsmedizin der
Universitat, D-Bonn
ANDERS, 0., Prof. Dr.
Klinik und Poliklinik fur Innere Medizin der Universitat Rostock, D-Rostock
ANDRIPSCHKE, K., Frau
Hamophilieambulanz, Klinikum der Johann-Wolfgang-Goethe-Universitat,
D-Frankfurt/M.
ANSTADT, M., Frau Dr.
DRK Blutspendedienst Sachs en gGmbH, Institut f. Transfusionsmed. Plauen,
D-Plauen
ARENDS, P., Dr.
Arzt flir Kinderheilkunde, A-Gussing
ASPOCK, G., Prim., Dr.
Allg. Osterr. Krankenhaus der Barmherzigen Schwestern yom Heiligen Kreuz,
Labor I, A-Wels
ASPOCK, L., Frau Prim.
AUERSWALD, G., Dr.
Professor-Hess-Kinderklinik, Zentralkrankenhaus St.-Jurgen-Str., D-Bremen
AUMANN, V., Dr.
Zentrum fur Kinderheilkunde, Medizinische Fakultat,
Otto-von-Guericke-Universitat, D-Magdeburg
AYGOREN-PURSUN, E., Frau Dr.
Hamophilieambulanz, Medizinische Klinik I,
Johann-Wolfgang-Goethe-Universitat, D-Frankfurt/Main
XII List of Participants
BALLEISEN, 1., Prof. Dr.
Abteilung Hamatologie u. Onkologie, Innere Medizin,
Evangelisches Krankenhaus, D-Hamm
BARTHELS, M., Frau Prof. Dr.
D-Hannover
BAUM,
Bundesministerium fur Gesundheit, D-Bonn
BAUMGARTNER, CH., Dr.
Facharzt fur Kinder und Jugendliche, spez. Hamatologie, CH -Gossau
BECK, CH., Frau Dr.
A.rztin fur Kinderheilkunde, D-Berlin
BECK, K.H., Dr.
Abteilung Transfusionsmedizin, Klinikum der Albert -Ludwigs-Universitat,
D-Freiburg
BECKER, TH., Dr.
Interessengemeinschaft Hamophiler, D-Bonn
BEESERE, H.P., Prof. Dr.
Institute for Quality Management and Standardization in Transfusion Med.
and Haemostaseology, D-Teningen
BEHA, 1., Frau
D-Oberursel
BENEKE, H., Dr.
Sektion Hamostaseologie, Abteilung Innere Medizin III, Medizinische
Universitatsklinik, D-Ulm
BEREUTER, 1., Dr.
Landeskrankenhaus Feldkirch, Abteilung Padiatrie, A-Feldkirch
BERGMANN, E, Frau Dr.
Gemeinschaftslabor, Dr. Keeser und Prof. Arndt, D-Hamburg
BERGSTRASSER, E., Frau Dr.
Kinderspital, Hamatologie, CH-Zurich
BERTHOLD, B., Dr.
Hamophiliezentrum, Klinik fUr Innere Medizin I, Klinikum Neubrandenburg,
D-Neubrandenburg