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  1. Article ; Online: Hemostatic cover in orthopedic surgery.

    Álvarez Román, María Teresa

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

    2023  Volume 34, Issue S1, Page(s) S22–S25

    Abstract: Bleeding into joints, known as hemarthrosis, is the most common kind of bleeding experienced by patients with hemophilia. Repeat bleeds into the same joint lead to the so-called hemophilic arthropathy. Patients with this condition tend to require surgery ...

    Abstract Bleeding into joints, known as hemarthrosis, is the most common kind of bleeding experienced by patients with hemophilia. Repeat bleeds into the same joint lead to the so-called hemophilic arthropathy. Patients with this condition tend to require surgery earlier and most frequently than the general population. Successful hemostasis is essential to carry out such procedures. Thanks to the advances made in the treatment of hemophilia, most surgical techniques can be performed safely and reliably. The present review shall focus on the international recommendations related to the performance of these surgical procedures. We shall be examining the available treatments, including the way they should be administered as well as the requirements regarding the postoperative period and the subsequent rehabilitation program.
    MeSH term(s) Humans ; Hemophilia A/complications ; Hemophilia A/surgery ; Hemostatics/therapeutic use ; Hemarthrosis/surgery ; Hemorrhage ; Hemostasis ; Orthopedic Procedures
    Chemical Substances Hemostatics
    Language English
    Publishing date 2023-05-31
    Publishing country England
    Document type Review ; Journal Article
    ZDB-ID 1033551-1
    ISSN 1473-5733 ; 0957-5235
    ISSN (online) 1473-5733
    ISSN 0957-5235
    DOI 10.1097/MBC.0000000000001201
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article: Real-world experience of rIX-FP prophylaxis at dosing intervals of up to 14 days in a pediatric patient with hemophilia B during the COVID-19 pandemic.

    Díaz-Jordán, Bolívar Luis / Cebanu, Tamara / García Barcenilla, Sara / Álvarez-Román, Maria Teresa

    Clinical case reports

    2023  Volume 11, Issue 12, Page(s) e8180

    Abstract: Switching to rIX-FP prophylaxis at dosing intervals of up to 14 days in a hemophilia B pediatric patient decreased treatment burden by reducing the number of administrations and hospital visits, without affecting efficacy or treatment adherence. This is ... ...

    Abstract Switching to rIX-FP prophylaxis at dosing intervals of up to 14 days in a hemophilia B pediatric patient decreased treatment burden by reducing the number of administrations and hospital visits, without affecting efficacy or treatment adherence. This is particularly important in contexts of limited mobility and overloaded healthcare services.
    Language English
    Publishing date 2023-11-28
    Publishing country England
    Document type Case Reports
    ZDB-ID 2740234-4
    ISSN 2050-0904
    ISSN 2050-0904
    DOI 10.1002/ccr3.8180
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: Addressing thrombosis concerns in immune thrombocytopenia: the role of fostamatinib in immune thrombocytopenia management.

    Provan, Drew / Thachil, Jecko / Álvarez Román, María Teresa

    Expert review of hematology

    2024  Volume 17, Issue 1-3, Page(s) 55–66

    Abstract: Introduction: Immune thrombocytopenia (ITP), a disease that commonly presents with an increased risk of bleeding, can also paradoxically produce an increased risk of thromboembolic events. The risk of thromboembolism can be associated with patient- ... ...

    Abstract Introduction: Immune thrombocytopenia (ITP), a disease that commonly presents with an increased risk of bleeding, can also paradoxically produce an increased risk of thromboembolic events. The risk of thromboembolism can be associated with patient-related factors (e.g. co-morbidities, age and history of thrombosis), disease-related factors (e.g. a greater proportion of younger, more reactive platelets, and the presence of microparticles and pro-inflammatory cytokines) and treatment-related factors (e.g. splenectomy, thrombopoietin receptor agonists, and IVIg).
    Areas covered: Aspects of the pathophysiology of ITP and the effects of treatment are discussed with emphasis on individualizing treatment based on the patient's thromboembolic risk, treatment options and preferences.
    Expert opinion: An increased understanding of the pathophysiology of ITP has led to the development of new agents such as fostamatinib, a spleen tyrosine kinase inhibitor. Further research into the factors contributing to the risks for bleeding and thromboembolic events can contribute to the development of more specific therapies for ITP and allow greater individualization of therapy based on each patient's medical history and clinical status.
    MeSH term(s) Humans ; Purpura, Thrombocytopenic, Idiopathic/drug therapy ; Aminopyridines/therapeutic use ; Morpholines/therapeutic use ; Thrombocytopenia/drug therapy ; Pyridines/therapeutic use ; Thrombosis/etiology ; Thrombosis/drug therapy ; Pyrimidines
    Chemical Substances fostamatinib (SQ8A3S5101) ; Aminopyridines ; Morpholines ; Pyridines ; Pyrimidines
    Language English
    Publishing date 2024-02-19
    Publishing country England
    Document type Journal Article ; Review
    ZDB-ID 2516804-6
    ISSN 1747-4094 ; 1747-4086
    ISSN (online) 1747-4094
    ISSN 1747-4086
    DOI 10.1080/17474086.2024.2318345
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  4. Article ; Online: Predicting joint involvement through tailored prophylaxis in severe haemophilia A, is it possible?

    Meijon-Ortigueira, Maria Del Mar / Alvarez-Roman, Maria Teresa / De La Corte, Hortensia / Butta, Nora / Jimenez-Yuste, Victor

    Haemophilia : the official journal of the World Federation of Hemophilia

    2024  

    Abstract: Introduction: Tailored prophylaxis is the current treatment regimen for patients with severe haemophilia A. Recently, published guidelines describe two possible approaches, based on clinical characteristics or estimation of pharmacokinetic parameters. ... ...

    Abstract Introduction: Tailored prophylaxis is the current treatment regimen for patients with severe haemophilia A. Recently, published guidelines describe two possible approaches, based on clinical characteristics or estimation of pharmacokinetic parameters. However, both have strengths and weaknesses, and their characteristics need to be integrated to optimize treatment appropriately. In this paper, we present a model that considers together the characteristics of prophylaxis and the relevance of each.
    Methods: The age at initiation of prophylaxis, number of bleeding events, treatment regimen, therapeutic adherence, FVIII trough levels, and joint status were analyzed in 59 patients followed at La Paz University Hospital between January 2000 and December 2019.
    Results: The mean duration of primary prophylaxis of 113.37 ± 57.79 months. Eighty-three percent (n = 49) had no joint status involvement at the end of follow-up (HJHS and HEAD-US = 0). The median ABR was 0.7 (IQR 0.2 -1.0) and 54.2% presented trough levels of FVIII during follow-up >1 IU/dL. 72,9% engaged in some type of physical activity and overall adherence was over 85% in all patients evaluated. The regression analysis performed, considering all these factors, showed that the initiation of prophylaxis before 21 months of age was the most relevant protective factor against the appearance of joint involvement (OR 88.33 p.031 CI 95% 1.49-5224.40) CONCLUSION: Early initiation of prophylaxis was the most relevant factor in the protection of joint status. More comprehensive analysis models adapted to the characteristics of each population, are needed to adequately individualize treatment.
    Language English
    Publishing date 2024-04-04
    Publishing country England
    Document type Journal Article
    ZDB-ID 1229713-6
    ISSN 1365-2516 ; 1351-8216 ; 1355-0691
    ISSN (online) 1365-2516
    ISSN 1351-8216 ; 1355-0691
    DOI 10.1111/hae.15014
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Arthroscopic ankle surgery in people with haemophilia.

    Rodriguez-Merchan, E Carlos / De la Corte-Rodriguez, Hortensia / Gomez-Cardero, Primitivo / Alvarez-Roman, Maria Teresa / Jimenez-Yuste, Victor

    Haemophilia : the official journal of the World Federation of Hemophilia

    2024  Volume 30, Issue 2, Page(s) 286–294

    Abstract: Introduction: People with haemophilia (PWH) not administered primary haematological prophylaxis since childhood, that is, those treated haematologically on demand or not treated at all, often experience the degeneration of the ankles, leading to pain ... ...

    Abstract Introduction: People with haemophilia (PWH) not administered primary haematological prophylaxis since childhood, that is, those treated haematologically on demand or not treated at all, often experience the degeneration of the ankles, leading to pain and functional impairment.
    Aim: To analyse the outcomes and complications of arthroscopic ankle surgery performed on PWH.
    Methods: For this narrative review of the literature, a search was conducted in PubMed on 2, December 2023, using the keywords "haemophilia", "ankle" and "arthroscopy". Of the 29 articles identified, 15 specifically related to ankle arthroscopy in PWH were selected (inclusion criterion). The remaining articles did not meet this requirement (exclusion criterion) and were therefore eliminated.
    Results: Arthroscopic procedures (arthroscopic synovectomy, debridement and arthrodesis of the ankle) are increasingly used in the surgical treatment of haemophilic ankle arthropathy. Although arthroscopic ankle surgery offers good outcomes in patients with haemophilia, the procedure is not free of complications, which range from 7.9% for arthroscopic ankle debridement to 13.1% in arthroscopic ankle synovectomy and 17.8% in arthroscopic ankle arthrodesis, respectively. The non-union rate of arthroscopic ankle arthrodesis is 7.1% (2/28).
    Conclusion: Although arthroscopic interventions in the haemophilic ankle (synovectomy, debridement, arthrodesis) offer good functional outcomes, they are associated with a non-negligible rate of complications. Arthroscopic ankle surgery in PWH is major surgery and should be treated as such.
    MeSH term(s) Humans ; Child ; Hemophilia A/complications ; Ankle ; Hemarthrosis/complications ; Arthroscopy/adverse effects ; Arthroscopy/methods ; Ankle Joint ; Arthritis/complications ; Arthrodesis/adverse effects ; Treatment Outcome
    Language English
    Publishing date 2024-02-20
    Publishing country England
    Document type Journal Article ; Review
    ZDB-ID 1229713-6
    ISSN 1365-2516 ; 1351-8216 ; 1355-0691
    ISSN (online) 1365-2516
    ISSN 1351-8216 ; 1355-0691
    DOI 10.1111/hae.14967
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: Fitusiran prophylaxis in severe haemophilia without inhibitors.

    Jiménez-Yuste, Víctor / Álvarez-Román, María Teresa

    The Lancet. Haematology

    2023  Volume 10, Issue 5, Page(s) e308–e309

    MeSH term(s) Humans ; Hemophilia A/complications ; Hemophilia A/drug therapy ; Acetylgalactosamine/therapeutic use ; RNA, Small Interfering/therapeutic use ; Factor VIII/therapeutic use
    Chemical Substances fitusiran ; Acetylgalactosamine (KM15WK8O5T) ; RNA, Small Interfering ; Factor VIII (9001-27-8)
    Language English
    Publishing date 2023-03-29
    Publishing country England
    Document type Journal Article ; Comment
    ISSN 2352-3026
    ISSN (online) 2352-3026
    DOI 10.1016/S2352-3026(23)00057-1
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article ; Online: The factor VIII treatment history of non-severe hemophilia A: COMMENT. Joint damage in adult patients with mild or moderate hemophilia A evaluated with the HEAD-US system.

    Álvarez Román, María Teresa / de la Corte Rodríguez, Hortensia / Bonanad Boix, Santiago / Mingot-Castellano, María Eva / Fernández Mosteirín, Nuria

    Journal of thrombosis and haemostasis : JTH

    2021  Volume 19, Issue 10, Page(s) 2638–2641

    MeSH term(s) Adult ; Factor VIII/genetics ; Hemarthrosis ; Hemophilia A/diagnosis ; Hemophilia A/drug therapy ; Hemostatics ; Humans
    Chemical Substances Hemostatics ; Factor VIII (9001-27-8)
    Language English
    Publishing date 2021-09-23
    Publishing country England
    Document type Letter ; Research Support, Non-U.S. Gov't
    ZDB-ID 2112661-6
    ISSN 1538-7836 ; 1538-7933
    ISSN (online) 1538-7836
    ISSN 1538-7933
    DOI 10.1111/jth.15475
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  8. Article ; Online: Assessing coagulopathy and endothelial dysfunction in pediatric venous malformation: A thromboelastometry and syndecan-1 study.

    Nava Y Hurtado, Francisco / Monzon Manzano, Elena / Viana-Huete, Vanesa / Triana Junco, Paloma / Alvarez-Roman, Maria Teresa / Arias-Salgado, Elena G / Butta, Nora / Lopez Gutierrez, Juan Carlos

    Pediatric blood & cancer

    2024  Volume 71, Issue 5, Page(s) e30915

    Abstract: Objective: The occurrence of unpredictable pain crises are the principal determinant of the quality of life for patients with venous malformations (VM). A definite coagulation phenomenon, characterized by an increase in D-dimer levels and the presence ... ...

    Abstract Objective: The occurrence of unpredictable pain crises are the principal determinant of the quality of life for patients with venous malformations (VM). A definite coagulation phenomenon, characterized by an increase in D-dimer levels and the presence of phleboliths within the malformation, has been previously reported. By applying Virchow's triad and evaluating intralesional samples, our objective is to delineate the coagulation profile and the extent of endothelial dysfunction within the malformation.
    Methods: With the authorization of the Ethics Committee, a research project was undertaken on intralesional and extralesional blood samples from 30 pediatric patients afflicted with spongiform VM. Thromboelastometry analyses were performed using ROTEM Sigma, and the concentration of syndecan-1 was determined by ELISA.
    Results: In the ROTEM analyses, the A5, A10, and maximum clot firmness (MCF) values were below the established reference ranges in the intralesional samples in both the EXTEM and INTEM assays, indicating that intralesional clots had significant instability. Furthermore, during the investigation of the delayed fibrinolysis phase using recombinant tissue plasminogen activator (rtPA) in EXTEM analysis, widespread hyperfibrinolysis was observed intralesional. Additionally, analysis of syndecan-1 showed significant differences between extralesional and intralesional levels (p < .026) and controls (p < .03), suggesting differences in the state of endothelium.
    Conclusions: For the first time, we developed a comprehensive understanding of the coagulopathic profile of VM and the role of endothelial dysfunction in its pathogenesis. These findings will enable the implementation of targeted therapies based on the individual coagulation profiles.
    MeSH term(s) Humans ; Child ; Thrombelastography ; Tissue Plasminogen Activator ; Syndecan-1 ; Quality of Life ; Blood Coagulation Disorders/etiology ; Blood Coagulation Tests ; Vascular Diseases
    Chemical Substances Tissue Plasminogen Activator (EC 3.4.21.68) ; Syndecan-1
    Language English
    Publishing date 2024-02-18
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2131448-2
    ISSN 1545-5017 ; 1545-5009
    ISSN (online) 1545-5017
    ISSN 1545-5009
    DOI 10.1002/pbc.30915
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  9. Article ; Online: The 2021 guidelines on the diagnosis of von Willebrand disease: A comparison with current clinical practice in Spanish centers.

    Álvarez-Román, María Teresa / Sierra-Aisa, Cristina / Jiménez-Yuste, Víctor

    Haemophilia : the official journal of the World Federation of Hemophilia

    2023  Volume 29, Issue 3, Page(s) 925–927

    MeSH term(s) Humans ; von Willebrand Diseases/diagnosis ; von Willebrand Factor
    Chemical Substances von Willebrand Factor
    Language English
    Publishing date 2023-03-13
    Publishing country England
    Document type Letter
    ZDB-ID 1229713-6
    ISSN 1365-2516 ; 1351-8216 ; 1355-0691
    ISSN (online) 1365-2516
    ISSN 1351-8216 ; 1355-0691
    DOI 10.1111/hae.14778
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  10. Article ; Online: Prophylaxis therapy with bypassing agents in patients with haemophilia A and inhibitors undergoing surgery: A cost analysis in Spain.

    Mareque, María / Mingot-Castellano, María Eva / López-Fernández, María Fernanda / Álvarez-Román, María Teresa / Oyagüez, Itziar

    European journal of haematology

    2020  Volume 105, Issue 1, Page(s) 94–100

    Abstract: Objectives: This study estimated the cost of prophylaxis with activated prothrombin complex concentrate (aPCC) and recombinant activated factor VIIa (rFVIIa) in surgical patients with haemophilia A and inhibitors in Spain.: Methods: A decision- ... ...

    Abstract Objectives: This study estimated the cost of prophylaxis with activated prothrombin complex concentrate (aPCC) and recombinant activated factor VIIa (rFVIIa) in surgical patients with haemophilia A and inhibitors in Spain.
    Methods: A decision-analytic model was developed to estimate the cost to the Spanish National Health System of providing haemostatic coverage in this haemophilia population, with age distribution and average weight derived from the literature, and the annual number of surgeries (0.33 per patient) from local data. Drug costs were calculated from official ex-factory prices with a 7.5% mandatory deduction and recommended dosing regimens.
    Results: The estimated average costs per patient were €10 100.73 (aPCC) and €14 265.89 (rFVIIa) for dental extraction, €24 043.88 (aPCC) and €62 301.08 (rFVIIa) for minor surgery and €126 595.81 (aPCC) and €347 731.09 (rFVIIa) for major surgery. Assuming an estimated 23 annual surgeries in this population (N = 69), distributed as 19% dental extraction, 50% minor surgery and 31% major surgery, the total annual cost of prophylaxis was €1 209 682.35 with aPCC and €3 221 929.28 with rFVIIa.
    Conclusions: aPCC costs were 62.5% lower than rFVIIa. Assuming potential clinical equivalence, aPCC is a potentially cost-saving option for surgical patients with haemophilia A and inhibitors.
    MeSH term(s) Blood Coagulation Factor Inhibitors/blood ; Clinical Decision-Making ; Cost-Benefit Analysis ; Disease Management ; Drug Costs ; Factor VIIa/administration & dosage ; Factor VIIa/immunology ; Health Care Surveys ; Hemophilia A/complications ; Hemophilia A/drug therapy ; Hemophilia A/epidemiology ; Hemophilia A/surgery ; Hemorrhage/epidemiology ; Hemorrhage/etiology ; Hemorrhage/prevention & control ; Humans ; Isoantibodies/blood ; Isoantibodies/immunology ; Recombinant Proteins/administration & dosage ; Recombinant Proteins/immunology ; Spain/epidemiology ; Surgical Procedures, Operative/adverse effects
    Chemical Substances Blood Coagulation Factor Inhibitors ; Isoantibodies ; Recombinant Proteins ; recombinant FVIIa (AC71R787OV) ; Factor VIIa (EC 3.4.21.21)
    Language English
    Publishing date 2020-04-16
    Publishing country England
    Document type Journal Article
    ZDB-ID 392482-8
    ISSN 1600-0609 ; 0902-4441
    ISSN (online) 1600-0609
    ISSN 0902-4441
    DOI 10.1111/ejh.13414
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