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  • 1
    In: Hämostaseologie, Georg Thieme Verlag KG, Vol. 40, No. 04 ( 2020-11), p. 543-545
    Abstract: Hintergrund und Auftrag Mit dem GSAV (Gesetz für mehr Sicherheit in der Arzneimittelversorgung) sollen eine bessere Zusammenarbeit von Bundes- und Länderbehörden sowie eine stärkere Kontrolle von Arzneimittelherstellern und Apotheken erreicht werden. Der Bund soll erweiterte Befugnisse zur Verbesserung der Arzneimittelsicherheit erhalten. Informationen über die Hersteller der Wirkstoffe in Arzneimitteln werden in Zukunft öffentlich zur Verfügung gestellt. Um Lieferengpässen bei Medikamenten entgegen zu wirken, werden Krankenkassen verpflichtet, bei Rabattverträgen künftig die Vielfalt der Anbieter zu berücksichtigen. Schließlich enthält das Gesetz einen Fahrplan zur schrittweisen Einführung des elektronischen Rezepts. Für Arzneimittel zur Versorgung von Patientinnen und Patienten mit hämophilen Gerinnungsstörungen, wird die bisherige Ausnahme vom Apothekenvertriebsweg (Direktvertrieb des Herstellers mit Ärzten und Krankenhäusern) zurückgenommen. Die Neuregelungen zum Vertriebsweg sowie die entsprechende Anpassung der Arzneimittelpreisverordnung und des Apothekengesetzes treten erst am 01.09.2020 in Kraft, auch infolge des vom BDDH initiierten und konzertierten Appells an den Bundesgesundheitsminister und die Gesundheitsbehörden der Bundesländer vom 24.03.2020 zur Verschiebung der Umsetzung des Vertriebsweges von Gerinnungsfaktorenzubereitungen.
    Type of Medium: Online Resource
    ISSN: 0720-9355 , 2567-5761
    Language: German
    Publisher: Georg Thieme Verlag KG
    Publication Date: 2020
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  • 2
    Online Resource
    Online Resource
    Georg Thieme Verlag KG ; 2023
    In:  Hämostaseologie Vol. 43, No. 02 ( 2023-04), p. 155-156
    In: Hämostaseologie, Georg Thieme Verlag KG, Vol. 43, No. 02 ( 2023-04), p. 155-156
    Type of Medium: Online Resource
    ISSN: 0720-9355 , 2567-5761
    Language: English
    Publisher: Georg Thieme Verlag KG
    Publication Date: 2023
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  • 3
    Online Resource
    Online Resource
    Georg Thieme Verlag KG ; 2020
    In:  Hämostaseologie Vol. 40, No. 01 ( 2020-02), p. 133-133
    In: Hämostaseologie, Georg Thieme Verlag KG, Vol. 40, No. 01 ( 2020-02), p. 133-133
    Type of Medium: Online Resource
    ISSN: 0720-9355 , 2567-5761
    Language: German
    Publisher: Georg Thieme Verlag KG
    Publication Date: 2020
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  • 4
    In: Hämostaseologie, Georg Thieme Verlag KG, Vol. 41, No. 02 ( 2021-04), p. 164-166
    Type of Medium: Online Resource
    ISSN: 0720-9355 , 2567-5761
    Language: English
    Publisher: Georg Thieme Verlag KG
    Publication Date: 2021
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  • 5
    In: Hämostaseologie, Georg Thieme Verlag KG, Vol. 42, No. 03 ( 2022-06), p. 210-211
    Type of Medium: Online Resource
    ISSN: 0720-9355 , 2567-5761
    Language: English
    Publisher: Georg Thieme Verlag KG
    Publication Date: 2022
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  • 6
    Online Resource
    Online Resource
    American Society of Hematology ; 2014
    In:  Blood Vol. 124, No. 21 ( 2014-12-06), p. 5068-5068
    In: Blood, American Society of Hematology, Vol. 124, No. 21 ( 2014-12-06), p. 5068-5068
    Abstract: Introduction: It is well know that most of the coagulation factor increase during pregnancy in healthy women. Nevertheless the uninterrupted course of coagulation parameters from the beginning until the end of a pregnancy in healthy women has not been described yet. Only reference ranges for the third month and the sixth month of pregnancy are evaluated. There aren't any data available for the course of coagulation parameters during pregnancy in women with known coagulation disorders. In 2012 we started a study to investigate reference ranges during pregnancy for all coagulation factors, anticoagulants and activation markers of coagulation in 100 healthy pregnant women and 100 pregnant women with a previously known mild bleeding disorders. The study has been approved by the Ethics Committee Nordrhein. Samples and Methods: We analysed samples of pregnant women by conducting the following tests: Blood count, VWF:RCo, VWF:Ag, VWF:CB, Fibrinogen (Clauss), activities of FII, FV, FVII, FVIII (clotting and chromogenic), FIX, FX, FXI, FXII, FXIII. d-dimer, prothrombin fragment 1.2, Quick, partial thromboplastin time, plasma thrombin time , CRP, proteine S, proteine C, antithrombin, Lupus antigoaculant, ACA, ß2-GP in week 10, 16, 22, 28, 34, 40 and 6 weeks post partum (max. +/- 1) Interim Results: Currently 21 pregnant women were included in our study. 16 obviously healthy women were used to calculate the reference ranges for pregnancy. Because of strict inclusion and exclusion criteria (no previous spontaneous abortion, no previous placenta haematoma, no previous pre-eclampsia and only natural pregnancies) most of the women are in the group of the no known coagulation disorder so far. Discussion/Conclusion: There are signs that defects in the coagulation system can be associated to complications during pregnancy like child loss, intrauterine haematoma and genital bleeding. The evaluation of reference ranges helps to detect and to value coagulation disorders during pregnancy. It might be possible to explain the higher abortion rate in women with mild bleeding disorders by determination of reference values of all pro- and anticoagulants during pregnancy. If a treatment with coagulation factor concentrates can help to prevent miscarriage is still subject of ongoing studies. Determination of reference ranges for coagulation factors in the third trimenon might help to determine the peripartum bleeding risk of women with mild coagulation disorders and to help to decide whether a women needs coagulation factor concentrate during labour. Limitation: Based on the strict inclusion and exclusion criteria the number of patients is small. Disclosures Halimeh: Biotest: The authors declare that they receive research grant from Biotest AG Other. Rott:Biotest: The authors declare that they receive research grant from Biotest AG Other. Osseiran:Biotest: The authors declare that they receive research grant from Biotest AG Other. Kappert:Biotest: The authors declare that they receive research grant from Biotest AG Other. Siebert:Biotest: The authors declare that they receive research grant from Biotest AG Other.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
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    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2014
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  • 7
    Online Resource
    Online Resource
    American Society of Hematology ; 2013
    In:  Blood Vol. 122, No. 21 ( 2013-11-15), p. 4786-4786
    In: Blood, American Society of Hematology, Vol. 122, No. 21 ( 2013-11-15), p. 4786-4786
    Abstract: In our study 144 children were send to our coagulation centre with a positive anamnesis (such as haematomas or nose bleeding’s) a positive family anamnesis (p.e. Mother with Menorrhagie) or a prolonged aPTT during a standardized preoperative examination. Samples and Methods We analyzed samples of 144 children by conduction the following tests: Blood count, VWF:RCo, VWF:Ag, VWF:CB, Fibrinogen (Clauss), activities of FII, FV, FVII, FVIII (clotting and chromogenic), FIX, FX, FXI, FXII, FXIII. Results In 107 of 144 children (74.3%) a bleeding disorder could be detected. In those with a bleeding disorder the distribution was as followed: 23.6% had a von Willebrand disease, 27.8% had a prolonged bleeding time and 5.6% a factor XIII-deficiency. The remaining 43% hat other bleeding. disorders (e.g. FVIII-deficiency, FVII-deficiency and other mild factor deficiencies). Discussion A standardized preoperative questionnaire can be useful in clinical practice. In our study 74.3% children with one or more positive evidence in the anamnesis suffer from a bleeding disorder. It is specifically noticeable that we found in 68% of the children which mother has a menorrhagia a bleeding disorder. In 31.8% of the children we found a von Willebrand disease. In our more coagulation disorders could be detected if a standardized preoperative questionnaire would be used and if we pay more attention to children with would a mothers with menorrhagia. Conclusions Children from mothers with menorrhagia suffer more frequently from a bleeding disorder. In our patients in 75% of the children with a mother with menorrhagia a bleeding disorder was found. To avoid an unexpected bleeding during a planned surgery or a postoperative bleeding a standardized preoperative questionnaire should be performed. Disclosures: Halimeh: Octapharma AG: Investigator Other, Research Funding.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
    RVK:
    RVK:
    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2013
    detail.hit.zdb_id: 1468538-3
    detail.hit.zdb_id: 80069-7
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  • 8
    Online Resource
    Online Resource
    American Society of Hematology ; 2018
    In:  Blood Vol. 132, No. Supplement 1 ( 2018-11-29), p. 3522-3522
    In: Blood, American Society of Hematology, Vol. 132, No. Supplement 1 ( 2018-11-29), p. 3522-3522
    Abstract: Background: The development of inhibitors due to infused factor VIII (FVIII) remains a significant challenge in haemophilia treatment, particularly in previously untreated patients (PUPs). Inhibitors in PUPs usually develop within the first 50 exposure days (EDs), with a median time to inhibitor development of around 10-15 EDs. The impact of product type on the risk of inhibitor development remains controversial. Whilst some studies have found no difference in the rate of inhibitor development between plasma-derived (pd) and recombinant (r)FVIII products, other studies have reported higher rates of inhibitor development after treatment with rFVIII. The prospective, randomised SIPPET study found an 87% higher incidence of inhibitors in PUPs treated with rFVIII than in PUPs treated with pdFVIII concentrates containing von Willebrand factor (VWF). Methods The aim of this study was to investigate a personalised treatment approach in PUPs in order to limit both joint damage and inhibitor development. This approach included the use of intensive monitoring from an early age, tailored prophylaxis using pdFVIII concentrates and personalised physiotherapy regimen. FVIII levels and Von Willebrand Antigen (VWF-Ag) were measured after birth (if possible), before start of prophylaxis and during prophylaxis. The inhibitor titre was measured every 3-4 EDs. Our study cohort was compared with a historical cohort (2004 - 2012) treated with early prophylaxis with hamster cell-derived rFVIII or pdFVIII. We enrolled 24 patients, 9 from the historical cohort (7 treated with rFVIII and 2 with pdFVIII) and since 2013 15 patients from in the study cohort (14 treated with pdFVIII and 1 not yet treated). Results: Since 2013 all 14 PUPs started early prophylaxis with pdFVIII/VWF. Initial dose ranged from 25 IU/kg/10 days to 60 IU/kg/week for the first 20 ED and thereafter individual dose escalation was performed. So far, no patient had developed and inhibitor to FVIII. In our historical group 4 out of 7 patients developed a high titre inhibitor (≥ 5 BU) during the first 20 EDs with rFVIII, but none of the patients receiving prophylaxis with pdFVIII (n=2) (p=0.007). All patients who developed an inhibitor later on had a VWF-Ag below 77%, those patients receiving rFVIII and remaining without an inhibitor had an VWF-Ag above 77% (n=3); this difference was highly significant (p 〈 0.001). Conclusion: We found that individually tailored treatment schedules and early prophylaxis seems to minimize the incidence of inhibitors in our cohort. Nevertheless, it has to be investigated if PUPs with a high VWF-level can be treated safely with rFVIII without risking the development of inhibitors. We recommend further prospective studies with a greater number of patients. Disclosures Halimeh: Bayer healthcare, Baxalta Innovations, Biotest, CSL Behring, Novartis, Novo Nordisk, Octapharma, LFB, Pfizer: Honoraria; Bayer Healthcare, Baxalta Innovations, Biotest, CSL Behring, Novo Nordisk, Octapharma, Pfizer: Research Funding.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
    RVK:
    RVK:
    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2018
    detail.hit.zdb_id: 1468538-3
    detail.hit.zdb_id: 80069-7
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  • 9
    In: Blood, American Society of Hematology, Vol. 140, No. Supplement 1 ( 2022-11-15), p. 8472-8473
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
    RVK:
    RVK:
    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2022
    detail.hit.zdb_id: 1468538-3
    detail.hit.zdb_id: 80069-7
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  • 10
    Online Resource
    Online Resource
    Ovid Technologies (Wolters Kluwer Health) ; 2016
    In:  Blood Coagulation & Fibrinolysis Vol. 27, No. 7 ( 2016-10), p. 786-790
    In: Blood Coagulation & Fibrinolysis, Ovid Technologies (Wolters Kluwer Health), Vol. 27, No. 7 ( 2016-10), p. 786-790
    Type of Medium: Online Resource
    ISSN: 0957-5235
    Language: English
    Publisher: Ovid Technologies (Wolters Kluwer Health)
    Publication Date: 2016
    detail.hit.zdb_id: 2035229-3
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