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  1. Article ; Online: Gentherapie in der Hämophilie A und B "Ante Portas": Aktuelle Entwicklungen in der ärztlichen Vergütung und Bericht Tagung Ethikrat.

    Koscielny, Jürgen / Kemkes-Matthes, Bettina / Kappert, Günther / Sucker, Christoph

    Hamostaseologie

    2022  Volume 42, Issue 4, Page(s) 274–275

    Language English
    Publishing date 2022-08-24
    Publishing country Germany
    Document type Journal Article
    ZDB-ID 801512-0
    ISSN 2567-5761 ; 0720-9355
    ISSN (online) 2567-5761
    ISSN 0720-9355
    DOI 10.1055/a-1868-1917
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article: Gentherapie in der Hämophilie A und B “Ante Portas”: Aktuelle Entwicklungen in der ärztlichen Vergütung und Bericht Tagung Ethikrat

    Koscielny, Jürgen / Kemkes-Matthes, Bettina / Kappert, Günther / Sucker, Christoph

    Hämostaseologie

    2022  Volume 42, Issue 04, Page(s) 274–275

    Language English
    Publishing date 2022-08-01
    Publisher Georg Thieme Verlag KG
    Publishing place Stuttgart ; New York
    Document type Article
    ZDB-ID 801512-0
    ISSN 2567-5761 ; 0720-9355
    ISSN (online) 2567-5761
    ISSN 0720-9355
    DOI 10.1055/a-1868-1917
    Database Thieme publisher's database

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  3. Article ; Online: Antikoagulation – direkte orale Antikoagulanzien.

    Kemkes-Matthes, B

    Der Internist

    2017  Volume 58, Issue 6, Page(s) 585–597

    Abstract: Since direct oral anticoagulants (DOAC) have become available, use of anticoagulant treatment has become easier and safer-for patients suffering from thromboembolic diseases as well as for patients with atrial fibrillation: Because of constant ... ...

    Title translation Anticoagulation-direct oral anticoagulants.
    Abstract Since direct oral anticoagulants (DOAC) have become available, use of anticoagulant treatment has become easier and safer-for patients suffering from thromboembolic diseases as well as for patients with atrial fibrillation: Because of constant bioavailability, fixed dose regimen treatment is possible, monitoring not necessary and severe bleeding complications-particularly intracranial hemorrhages-rare in comparison to vitamin K anticoagulants. To gain all these advantages, it is essential to give DOAC in the correct dosage. Dose reduction of single DOAC has to be considered depending on underlying disease, body weight and renal function. DOAC are not allowed in patients with artificial heart valves, in pregnancy and in children. In case of severe bleeding complications under DOAC treatment, prothrombin complex concentrates is one treatment option. For dabigatran an antidote is available.
    Language German
    Publishing date 2017-06
    Publishing country Germany
    Document type English Abstract ; Journal Article
    ZDB-ID 2913-0
    ISSN 1432-1289 ; 0020-9554
    ISSN (online) 1432-1289
    ISSN 0020-9554
    DOI 10.1007/s00108-017-0243-y
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: Should emicizumab be used in patients with acquired hemophilia A?

    Tiede, Andreas / Kemkes-Matthes, Bettina / Knöbl, Paul

    Journal of thrombosis and haemostasis : JTH

    2020  Volume 19, Issue 3, Page(s) 637–644

    Abstract: Emicizumab is currently approved to prevent bleeding in patients with congenital hemophilia A with or without neutralizing antibodies (inhibitors) against factor VIII (FVIII). Here, we present a case-based discussion of its potential use in acquired ... ...

    Abstract Emicizumab is currently approved to prevent bleeding in patients with congenital hemophilia A with or without neutralizing antibodies (inhibitors) against factor VIII (FVIII). Here, we present a case-based discussion of its potential use in acquired hemophilia A (AHA), a severe bleeding disorder caused by autoantibodies against FVIII. State-of-the-art management is based on bypassing agents (recombinant factor VIIa, activated prothrombin complex concentrate) and recombinant porcine FVIII; immunosuppressive therapy (corticosteroids, rituximab, cyclophosphamide) is used to suppress autoantibody formation. Case reports and one series suggest that emicizumab can reduce the risk of bleeding and the requirement for hemostatic therapy until remission of AHA is achieved. Further, it may allow to postpone the start of immunosuppressive therapy or to use less intense regimens. However, the risk-benefit assessment of emicizumab in AHA is difficult because demographic and clinical characteristics are different compared with congenital hemophilia. Prospective clinical trials are needed before the use of emicizumab can be recommended in AHA.
    MeSH term(s) Animals ; Antibodies, Bispecific/therapeutic use ; Antibodies, Monoclonal, Humanized/therapeutic use ; Factor VIII ; Hemophilia A/diagnosis ; Hemophilia A/drug therapy ; Humans ; Prospective Studies
    Chemical Substances Antibodies, Bispecific ; Antibodies, Monoclonal, Humanized ; emicizumab (7NL2E3F6K3) ; Factor VIII (9001-27-8)
    Language English
    Publishing date 2020-12-10
    Publishing country England
    Document type Case Reports ; Journal Article
    ZDB-ID 2112661-6
    ISSN 1538-7836 ; 1538-7933
    ISSN (online) 1538-7836
    ISSN 1538-7933
    DOI 10.1111/jth.15208
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  5. Article: Inhibitor-immunology-study. Evaluation of inhibitor development in haemophilia B.

    Wieland, I / Wermes, C / Eifrig, B / Holstein, K / Pollmann, H / Siegmund, B / Eberl, W / Kemkes-Matthes, B / Bidlingmaier, C / Kurnik, K / Lischetzki, G / Nimtz-Talaska, A / Eisert, R / Bogdanova, N / Doerk, T / Sykora, K-W

    Hamostaseologie

    2011  Volume 31 Suppl 1, Page(s) S57–60

    Abstract: Unlabelled: The development of inhibitors in haemophilia B is one of the most important ... results of the haemophilia B group of our Inhibitor-Immunology study.: Patients, methods: So far ... we have analysed 15 patients with haemophilia B. Four of them developed a high titre inhibitor ...

    Abstract Unlabelled: The development of inhibitors in haemophilia B is one of the most important complications of replacement therapy, affecting mortality and morbidity. Inhibitor development is based on complex immunological factors, and to date, only little is known about its underlying mechanisms. Here, we present first results of the haemophilia B group of our Inhibitor-Immunology study.
    Patients, methods: So far we have analysed 15 patients with haemophilia B. Four of them developed a high titre inhibitor; the remaining 11 had no inhibitor. We evaluated 9 SNPs in 8 genes (CD40, CTLA-4 , IL-1β, IL-10, TLR2 , TLR4, TLR9, TNF-α). We compared the distribution of these alleles between inhibitor and non-inhibitor haemophilia B patients and between haemophilia B patients and a normal male control population. HLA typing was performed in all patients. Results, discussion: There appears to be a trend towards a skewed distribution of TLR 9, IL-10 and CTLA4 alleles in haemophilia B patients. Due to the limited number these differences are, however, not statistically significant. The t-test of all patients with inhibitor versus without inhibitor was significant for HLA-A*03 and DPB1*0401 and borderline for DRB1*0201.
    MeSH term(s) Adolescent ; Blood Coagulation Factor Inhibitors/blood ; Blood Coagulation Factor Inhibitors/genetics ; Child ; Child, Preschool ; Female ; Genes, MHC Class II/genetics ; Genetic Predisposition to Disease/genetics ; Hemophilia B/blood ; Hemophilia B/genetics ; Humans ; Male ; Polymorphism, Single Nucleotide/genetics ; Young Adult
    Chemical Substances Blood Coagulation Factor Inhibitors
    Language English
    Publishing date 2011-11
    Publishing country Germany
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 801512-0
    ISSN 0720-9355
    ISSN 0720-9355
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  6. Article ; Online: Direct oral anticoagulants in patients with antiphospholipid syndrome: a retrospective study in a real-life patient cohort.

    Franke, Benjamin / Luxembourg, Beate / Heidinger, Kathrin / Kemkes-Matthes, Bettina / Sachs, Ulrich J

    Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis

    2021  Volume 33, Issue 3, Page(s) 184–187

    Abstract: The current mainstay for the treatment of thrombotic antiphospholipid syndrome (APS) is anticoagulation with vitamin K antagonists (VKAs). The use of direct oral anticoagulants (DOACs) is under debate. We aimed to assess whether DOACs would be safe in ... ...

    Abstract The current mainstay for the treatment of thrombotic antiphospholipid syndrome (APS) is anticoagulation with vitamin K antagonists (VKAs). The use of direct oral anticoagulants (DOACs) is under debate. We aimed to assess whether DOACs would be safe in APS patients presenting to the thrombosis clinic. A retrospective cohort study was conducted. All patients presenting to our thrombosis clinic between 2010 and 2017 with a diagnosis of APS taking either VKAs or DOACs were included. APS diagnosis was based on the revised Sapporo criteria. Clinical and laboratory data were collected from the electronic and physical patient files. Out of 200 patients, 81 received VKAs, and 119 DOACs. The two cohorts did not differ with regard to their initial clinical manifestation or additional prothrombotic risk factors. Only a small minority of patients was antiphospholipid antibody triple positive (VKA, 7.0% vs. DOAC, 4.2%). Numberofon-treatment events was low (3 vs. 2). The hazard ratio for any thromboembolic event for patients taking DOACs was 0.78 (95% confidence interval, 0.12-5.19). Treatment with DOACs was not associated with an increased risk of recurrent thromboembolism in comparison with VKAs in this retrospective study. Our observation supports the assumption that in nontriple positive (low risk) APS patients, DOACs might be safe. Prospective data are urgently needed.
    MeSH term(s) Administration, Oral ; Anticoagulants/adverse effects ; Antiphospholipid Syndrome/complications ; Antiphospholipid Syndrome/drug therapy ; Humans ; Prospective Studies ; Retrospective Studies ; Thromboembolism/chemically induced ; Thrombosis/drug therapy
    Chemical Substances Anticoagulants
    Language English
    Publishing date 2021-02-09
    Publishing country England
    Document type Journal Article
    ZDB-ID 1033551-1
    ISSN 1473-5733 ; 0957-5235
    ISSN (online) 1473-5733
    ISSN 0957-5235
    DOI 10.1097/MBC.0000000000001021
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article ; Online: Impact of double heterozygosity for Factor V Leiden and Prothrombin G20210A on the thrombotic phenotype.

    Luxembourg, Beate / Henke, Franziska / Kirsch-Altena, Anette / Sachs, Ulrich / Kemkes-Matthes, Bettina

    Thrombosis research

    2021  Volume 200, Page(s) 121–127

    Abstract: Introduction: Because of the rarity of double heterozygosity for Factor V Leiden (FVL) and Prothrombin (FII) G20210A, little is known about the thrombotic phenotype in double heterozygotes.: Material and methods: In a retrospective cohort study of ... ...

    Abstract Introduction: Because of the rarity of double heterozygosity for Factor V Leiden (FVL) and Prothrombin (FII) G20210A, little is known about the thrombotic phenotype in double heterozygotes.
    Material and methods: In a retrospective cohort study of patients referred for a thrombophilia work-up, we investigated whether double heterozygotes (n = 138) exhibit a more severe thrombotic phenotype compared with single FVL or FIIG20210A heterozygotes, single FVL homozygotes, or wildtype carriers.
    Results: The risk of venous thromboembolism (VTE) was higher for female but not male double heterozygotes compared with single heterozygotes (FVL: 2.51, 95%CI 1.55-4.08, FIIG20210A: 1.75, 95%CI 1.14-2.68) and wildtype carriers (HR 2.53, 95%CI 1.58-4.05) but not compared with FVL homozygotes (HR 1.31, 95%CI 0.94-1.83). Female double heterozygotes developed VTE nearly a decade earlier than wildtype carriers and FVL heterozygotes (mean 44.2 vs. 52.6 and 52.2 years), most often in association with oral contraceptives. Spontaneous VTE and arterial thromboembolic events were not more frequent in double heterozygotes compared with the other genotype groups. Deep vein thrombosis (DVT) of the lower limb was the predominant VTE location in double heterozygotes, atypical vein thrombosis was rare. A phenomenon that has been described as the FVL paradox, a higher proportion of isolated DVT than pulmonary embolism, was also found for double heterozygotes.
    Conclusion: The thrombotic phenotype in double heterozygotes resembles the appearance of the thrombotic phenotype in FVL carriers but the thrombotic risk is aggravated by women-specific risk factors.
    MeSH term(s) Factor V/genetics ; Female ; Humans ; Phenotype ; Prothrombin/genetics ; Retrospective Studies ; Risk Factors ; Thrombophilia/genetics ; Thrombosis
    Chemical Substances factor V Leiden ; Factor V (9001-24-5) ; Prothrombin (9001-26-7)
    Language English
    Publishing date 2021-02-02
    Publishing country United States
    Document type Journal Article
    ZDB-ID 121852-9
    ISSN 1879-2472 ; 0049-3848
    ISSN (online) 1879-2472
    ISSN 0049-3848
    DOI 10.1016/j.thromres.2021.01.022
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  8. Book: Blutgerinnung und Thrombose

    Kemkes-Matthes, Bettina / Oehler, Gerd

    50 Tabellen

    2001  

    Author's details Bettina Kemkes-Matthes ; Gerd Oehler
    Keywords Hämostaseologie
    Language German
    Size XI, 175 S. : Ill., graph. Darst.
    Edition 3., neu bearb. Aufl.
    Publisher Thieme
    Publishing place Stuttgart u.a.
    Publishing country Germany
    Document type Book
    HBZ-ID HT012940358
    ISBN 3-13-104822-0 ; 978-3-13-104822-6
    Database Catalogue ZB MED Medicine, Health

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  9. Article: Inhibitor-Immunologie-Studie - Evaluation der Inhibitorentwicklung der Hämophilie B. Inhibitor-Immunology-Study - Evaluation der Inhibitorentwicklung der Hämophilie B

    Wieland, I. / Wermes, C. / Eifrig, B. / Holstein, K. / Pollmann, H. / Siegmund, B. / Eberl, W. / Kemkes-Matthes, B. / Bidlingmaier, C. / Kurnik, K. / Lischetzki, G. / Nimtz-Talaska, A. / Eisert, R. / Bogdanova, N. / Doerk, T. / Sykora, K.-W.

    Hämostaseologie

    2011  Volume 31, Issue 4a,Suppl.1, Page(s) 57

    Language German
    Document type Article
    ZDB-ID 801512-0
    ISSN 0720-9355
    Database Current Contents Medicine

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  10. Book: Blutgerinnung und Thrombose

    Kemkes-Matthes, Bettina / Oehler, Gerd

    49 Tabellen

    1998  

    Author's details Bettina Kemkes-Matthes ; Gerd Oehler
    Keywords Blood Coagulation Disorders ; Thrombosis / drug therapy ; Hämostaseologie
    Language German
    Size XIII, 162 S. : Ill., graph. Darst.
    Edition 2., völlig neubearb. Aufl.
    Publisher Thieme
    Publishing place Stuttgart u.a.
    Document type Book
    Old title 1. Aufl. u.d.T. Oehler, Gerd: Blutgerinnung und Thrombose
    HBZ-ID HT008289750
    ISBN 3-13-104821-2 ; 978-3-13-104821-9
    Database Catalogue ZB MED Medicine, Health

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