LIVIVO - The Search Portal for Life Sciences

zur deutschen Oberfläche wechseln
Advanced search

Search results

Result 1 - 10 of total 162

Search options

  1. Article ; Online: Testing strategies used in the diagnosis of rare inherited bleeding disorders.

    Pruthi, Rajiv K

    Expert review of hematology

    2023  Volume 16, Issue 6, Page(s) 451–465

    Abstract: Introduction: Rare Bleeding Disorders have a low population prevalence and may not be recognized by most clinicians. In addition, knowledge gaps of the indicated laboratory tests and their availability add to the potential for delayed diagnosis or ... ...

    Abstract Introduction: Rare Bleeding Disorders have a low population prevalence and may not be recognized by most clinicians. In addition, knowledge gaps of the indicated laboratory tests and their availability add to the potential for delayed diagnosis or misdiagnosis. The lack of widely available commercial, regulatory body approved esoteric tests limits them to reference laboratories, thus limiting easy access for patients.
    Areas covered: A literature search of PubMed, Medline, and Embase and a review of international society guidelines were performed. Additional references from published articles were reviewed. A patient-centered approach to recognition and evaluation of RBD is discussed.
    Expert opinion: Recognition of RBD relies on obtaining a detailed patient's personal and family hemostatic history. Inquiry into a history of involvement of other organ systems is important and, if present, should lead to suspicion of an inherited platelet disorder or a variant of Ehlers-Danlos Syndrome. Multiple factors contribute to the complexity of developing efficient algorithms for diagnostic testing. Limitations in diagnostic sensitivity and specificity of screening tests, diagnostic tests, and esoteric tests further compound the complexity of establishing a diagnosis. Educational efforts focusing on clinician awareness of RBDs and available testing options are vital for optimal management of such patients.
    MeSH term(s) Humans ; Blood Coagulation Disorders ; Blood Platelet Disorders ; Hemostasis ; Hemorrhage ; Algorithms
    Language English
    Publishing date 2023-05-08
    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 10.1080/17474086.2023.2211257
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

    In stock of ZB MED Cologne/Königswinter

    Zs.A 6943: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular
    ab Jg. 2022: Lesesaal (EG)

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

  2. Article ; Online: Transplacental transfer of emicizumab: Experience with emicizumab in a pregnant female with severe hemophilia A and an inhibitor.

    Kharel, Zeni / Pruthi, Rajiv K / Kouides, Peter / Reid, Robin

    Haemophilia : the official journal of the World Federation of Hemophilia

    2024  

    Language English
    Publishing date 2024-04-22
    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.15022
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

    In stock of ZB MED Cologne/Königswinter

    Zs.A 4249: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (2.OG)
    ab Jg. 2022: Lesesaal (EG)
    Zs.MO 450: Show issues

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

  3. Article ; Online: Platelet genetic testing by next-generation sequencing: A practical update.

    Chen, Dong / Pruthi, Rajiv K

    International journal of laboratory hematology

    2023  Volume 45, Issue 5, Page(s) 630–642

    Abstract: Inherited platelet disorders (IPDs) are a heterogeneous group of disorders characterized by normal or reduced platelet counts, bleeding diatheses of varying severities, and the presence (syndromic) or absence (non-syndromic) of involvement of other ... ...

    Abstract Inherited platelet disorders (IPDs) are a heterogeneous group of disorders characterized by normal or reduced platelet counts, bleeding diatheses of varying severities, and the presence (syndromic) or absence (non-syndromic) of involvement of other organs. Due to the lack of highly specific platelet function tests and overlapping clinical and laboratory features, diagnosing the underlying cause of IPDs remains challenging. In recent years, genetic testing via next-generation sequencing (NGS) technologies to rapidly analyze multiple genes has gradually emerged as an important part of the laboratory investigation of patients with IPDs. A systemic clinical and laboratory testing approach and thorough phenotype and genotype correlation studies of both patients and their family members are crucial for accurate diagnoses of IPDs.
    MeSH term(s) Humans ; Blood Platelets ; Genetic Testing ; Blood Platelet Disorders/diagnosis ; Blood Platelet Disorders/genetics ; Phenotype ; High-Throughput Nucleotide Sequencing
    Language English
    Publishing date 2023-07-18
    Publishing country England
    Document type Journal Article ; Review
    ZDB-ID 2268590-X
    ISSN 1751-553X ; 1751-5521 ; 0141-9854
    ISSN (online) 1751-553X
    ISSN 1751-5521 ; 0141-9854
    DOI 10.1111/ijlh.14136
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

    In stock of ZB MED Cologne/Königswinter

    Zs.A 1499: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

  4. Article ; Online: The Use of Bypassing Treatment Strategies in Hemophilia and Their Effect on Laboratory Testing.

    Pruthi, Rajiv K / Chen, Dong

    Seminars in thrombosis and hemostasis

    2023  Volume 49, Issue 6, Page(s) 651–660

    Abstract: Factor VIII and IX inhibitors in congenital hemophilia A and B, respectively, neutralize the infused coagulation factor concentrate rendering them ineffective. Bypassing agents (BPAs) that circumvent the block imposed by the inhibitors are used for the ... ...

    Abstract Factor VIII and IX inhibitors in congenital hemophilia A and B, respectively, neutralize the infused coagulation factor concentrate rendering them ineffective. Bypassing agents (BPAs) that circumvent the block imposed by the inhibitors are used for the prevention and management of bleeding. Activated prothrombin complex concentrate was the original BPA, recombinant activated factor VII was then introduced, and more recently nonfactor agents that target the procoagulant and anticoagulant systems have been developed and are in clinical use (e.g., emicizumab, a bispecific antibody for hemophilia A). Other BPAs are in clinical trials (e.g., fitusiran targets antithrombin, concizumab and marstacimab target tissue factor pathway inhibitor, and SerpinPC targets activated protein C). The BPAs have a varied effect on coagulation assays, and as more patients are exposed to these agents, it is important to be aware of the effects. Herein, we present an overview of the effect of BPAs on routine and specialized coagulation assays including thrombin generation and viscoelastic assays.
    MeSH term(s) Humans ; Hemophilia A/drug therapy ; Factor VIII/therapeutic use ; Hemorrhage/prevention & control ; Hemostatics/therapeutic use ; Blood Coagulation Tests ; Thrombin/metabolism
    Chemical Substances Factor VIII (9001-27-8) ; Hemostatics ; Thrombin (EC 3.4.21.5)
    Language English
    Publishing date 2023-05-05
    Publishing country United States
    Document type Review ; Journal Article
    ZDB-ID 196901-8
    ISSN 1098-9064 ; 0094-6176
    ISSN (online) 1098-9064
    ISSN 0094-6176
    DOI 10.1055/s-0043-1768660
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

    In stock of ZB MED Cologne/Königswinter

    Zs.A 1259: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

  5. Article: The Use of Bypassing Treatment Strategies in Hemophilia and Their Effect on Laboratory Testing

    Pruthi, Rajiv K. / Chen, Dong

    Seminars in Thrombosis and Hemostasis

    (Laboratory Diagnostics for Thrombosis and Hemostasis Testing—Part II)

    2023  Volume 49, Issue 06, Page(s) 651–660

    Abstract: Factor VIII and IX inhibitors in congenital hemophilia A and B, respectively, neutralize the infused coagulation factor concentrate rendering them ineffective. Bypassing agents (BPAs) that circumvent the block imposed by the inhibitors are used for the ... ...

    Series title Laboratory Diagnostics for Thrombosis and Hemostasis Testing—Part II
    Abstract Factor VIII and IX inhibitors in congenital hemophilia A and B, respectively, neutralize the infused coagulation factor concentrate rendering them ineffective. Bypassing agents (BPAs) that circumvent the block imposed by the inhibitors are used for the prevention and management of bleeding. Activated prothrombin complex concentrate was the original BPA, recombinant activated factor VII was then introduced, and more recently nonfactor agents that target the procoagulant and anticoagulant systems have been developed and are in clinical use (e.g., emicizumab, a bispecific antibody for hemophilia A). Other BPAs are in clinical trials (e.g., fitusiran targets antithrombin, concizumab and marstacimab target tissue factor pathway inhibitor, and SerpinPC targets activated protein C). The BPAs have a varied effect on coagulation assays, and as more patients are exposed to these agents, it is important to be aware of the effects. Herein, we present an overview of the effect of BPAs on routine and specialized coagulation assays including thrombin generation and viscoelastic assays.
    Keywords bypassing agent ; bispecific antibody ; coagulation assays ; viscoelastic
    Language English
    Publishing date 2023-05-05
    Publisher Thieme Medical Publishers, Inc.
    Publishing place Stuttgart ; New York
    Document type Article
    ZDB-ID 196901-8
    ISSN 1098-9064 ; 0094-6176
    ISSN (online) 1098-9064
    ISSN 0094-6176
    DOI 10.1055/s-0043-1768660
    Database Thieme publisher's database

    More links

    Kategorien

    In stock of ZB MED Cologne/Königswinter

    Zs.A 1259: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

  6. Article: Multiple Simultaneous Infections With Nontuberculous Mycobacteria in the Setting of

    Tabaja, Hussam / Jensen, Kelsey L / Rivera, Christina G / Misra, Anisha / Pruthi, Rajiv K / Vergidis, Paschalis

    Open forum infectious diseases

    2022  Volume 9, Issue 7, Page(s) ofac309

    Abstract: ... ...

    Abstract GATA2
    Language English
    Publishing date 2022-06-28
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2757767-3
    ISSN 2328-8957
    ISSN 2328-8957
    DOI 10.1093/ofid/ofac309
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

  7. Article ; Online: Laboratory monitoring of new hemostatic agents for hemophilia.

    Pruthi, Rajiv K

    Seminars in hematology

    2016  Volume 53, Issue 1, Page(s) 28–34

    Abstract: Prophylactic infusion of factor replacement products results in a reduction in long-term morbidity and mortality in patients with severe hemophilia. However, intravenous access is commonly through central venous access devices, which may result in ... ...

    Abstract Prophylactic infusion of factor replacement products results in a reduction in long-term morbidity and mortality in patients with severe hemophilia. However, intravenous access is commonly through central venous access devices, which may result in complications such as infections and thrombosis. Available clinical data on extended half-life (EHL) factor replacement products indicate the potential for a significant reduction in the need for frequent infusions, eg, once per week for factor (F)IX and twice per week for FVIII. With the current generation of factor replacement products, individualized pharmacokinetics (pK) direct optimal prophylactic dosing. The available data on the EHL factor replacement products also confirm similar individualized variability. Optimal dosing of these therapies relies on accurate assays, of which there is a variety, although performance characteristics vary with the specific product being tested. Herein, the data on clinical trials and laboratory assays are reviewed.
    MeSH term(s) Clinical Trials as Topic ; Drug Combinations ; Factor IX/therapeutic use ; Factor VIII/therapeutic use ; Hemophilia A/drug therapy ; Hemostatics/therapeutic use ; Humans
    Chemical Substances Drug Combinations ; Hemostatics ; Factor VIII (9001-27-8) ; Factor IX (9001-28-9)
    Language English
    Publishing date 2016-01
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 206923-4
    ISSN 1532-8686 ; 0037-1963
    ISSN (online) 1532-8686
    ISSN 0037-1963
    DOI 10.1053/j.seminhematol.2015.10.003
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

    In stock of ZB MED Cologne/Königswinter

    Zs.A 328: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

  8. Article ; Online: Von Willebrand Factor Multimer Analysis and Classification: A Comprehensive Review and Updates.

    Saadalla, Abdulrahman / Seheult, Jansen / Pruthi, Rajiv K / Chen, Dong

    Seminars in thrombosis and hemostasis

    2022  Volume 49, Issue 6, Page(s) 580–591

    Abstract: Von Willebrand factor (VWF) is a multimeric glycoprotein with essential roles in primary hemostasis. Patients with von Willebrand disease (VWD), due to quantitative and/or qualitative defects of VWF usually experience mucocutaneous bleeding. Based on the ...

    Abstract Von Willebrand factor (VWF) is a multimeric glycoprotein with essential roles in primary hemostasis. Patients with von Willebrand disease (VWD), due to quantitative and/or qualitative defects of VWF usually experience mucocutaneous bleeding. Based on the laboratory results of VWF antigen, various VWF activities, factor VIII activity, and VWF multimer patterns, VWD can be categorized as type 1, 2, and 3 VWD. VWF multimer analysis by either manual or semi-automated electrophoresis and immunoblotting is a critical part of the laboratory testing to differentiate type 1, type 2 VWD, and subtypes of type 1 or 2 VWD. The multimer distribution patterns can also help to understand the underlying molecular mechanism of VWF synthesis, multimerization, and clearance defects in VWD. This review will cover VWF synthesis, multimerization, secretion, VWF multimer analysis, and VWF multimer interpretation of various types and subtypes of VWD.
    MeSH term(s) Humans ; von Willebrand Factor ; von Willebrand Diseases/diagnosis ; von Willebrand Disease, Type 2 ; Hemostatics
    Chemical Substances von Willebrand Factor ; Hemostatics
    Language English
    Publishing date 2022-09-29
    Publishing country United States
    Document type Review ; Journal Article
    ZDB-ID 196901-8
    ISSN 1098-9064 ; 0094-6176
    ISSN (online) 1098-9064
    ISSN 0094-6176
    DOI 10.1055/s-0042-1757183
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

    In stock of ZB MED Cologne/Königswinter

    Zs.A 1259: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

  9. Article ; Online: Use of crushed tranexamic acid tablets in water for paediatric patients with bleeding disorders.

    Al-Huniti, Ahmad / Marshall, Linda / Rusk, Dawn / Pruthi, Rajiv K / Rodriguez, Vilmarie / Ferdjallah, Asmaa / Kuhn, Alexis

    Haemophilia : the official journal of the World Federation of Hemophilia

    2024  

    Abstract: Background: Ε-Aminocaproic acid oral solution (EACA OS) is the only commercially available antifibrinolytic for patients who cannot swallow tablets. Insurance denials and high costs remain barriers to its use.: Objectives: To determine the safety and ...

    Abstract Background: Ε-Aminocaproic acid oral solution (EACA OS) is the only commercially available antifibrinolytic for patients who cannot swallow tablets. Insurance denials and high costs remain barriers to its use.
    Objectives: To determine the safety and efficacy of crushed tranexamic acid tablets in water (cTXAw) for children with bleeding disorders.
    Methods: We retrospectively reviewed records of children (<10 years) with bleeding disorders who received cTXAw or EACA OS from 1 December 2018, through 31 July 2022, at Mayo Clinic (Rochester, Minnesota). Bleeding outcomes were defined according to ISTH criteria.
    Results: Thirty-two patients were included (median age, 3 years; male, n = 23). Diagnoses were VWD (n = 17), haemophilia (n = 5), FVII deficiency (n = 3), inherited platelet disorder (n = 4), ITP (n = 2), and combined FV and FVII deficiencies (n = 1). Thirty-two courses of cTXAw (monotherapy 24/32; mean duration 6 days) and fifteen courses of EACA (monotherapy 12/15; mean duration 5 days) were administered. No surgical procedures (n = 28) were complicated by bleeding. Of the 19 bleeding events, 16 had effective haemostasis, two had no reported outcome, and one had no response. cTXAw and EACA were equally effective in preventing and treating bleeding (p value > .1). No patients had adverse effects. Eight of 19 patients (42%) who were initially prescribed EACA OS did not receive it because of cost or insurance denial. The estimated average wholesale price of one treatment was $94 for cTXAw and $905 for EACA OS.
    Conclusions: CTXAw appears to be an effective, safe, and low-cost alternative option to EACA OS for young children with bleeding disorders.
    Language English
    Publishing date 2024-03-20
    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.14996
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

    In stock of ZB MED Cologne/Königswinter

    Zs.A 4249: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (2.OG)
    ab Jg. 2022: Lesesaal (EG)
    Zs.MO 450: Show issues

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

  10. Article ; Online: C3+ and C3- warm autoimmune hemolytic anemias: A comparison of clinical characteristics and treatment outcomes.

    Nunnelee, Jordan / Abeykoon, Jithma Prasad / Ashrani, Aneel A / Elliott, Michelle Ann / Hook, C Christopher / Pardanani, Animesh / Pruthi, Rajiv K / Rouse, Rachelle L / Sridharan, Meera / Wolanskyj-Spinner, Alexandra P / Go, Ronald S

    European journal of haematology

    2024  

    Language English
    Publishing date 2024-04-15
    Publishing country England
    Document type Letter
    ZDB-ID 392482-8
    ISSN 1600-0609 ; 0902-4441
    ISSN (online) 1600-0609
    ISSN 0902-4441
    DOI 10.1111/ejh.14212
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

    In stock of ZB MED Cologne/Königswinter

    Zs.A 323: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)

    Order via subito

    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

To top