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  1. Book: Congenital Bleeding Disorders

    Dorgalaleh, Akbar

    Diagnosis and Management

    2023  

    Author's details Akbar Dorgalaleh is a hematologist and scientific researcher in the field of thrombosis and hemostasis. He has (co-)authored over 120 papers, and is a member of the editorial boards of several international journals. Dr. Dorgalaleh is also a current or past member of several International scientific societies or working parties, including International Society on Thrombosis and Hemostasis (ISTH) Scientific Standardization Committees (SSCs) on Subcommittee on Factor XIII and Fibrinogen and International Council for Standardization in Haematology (ICSH)
    Keywords CommonBleedingDisorders ; VonWillebrandDisorders ; HemophiliaA ; HemophiliaB ; RareBleedingDisorders ; GlanzmannThrombasthenia ; Bernard-SoulierSyndrome ; GrayPlateletSyndrome ; QuebecPlateletDisorder ; CombinedCoagulationFactorDefiencies ; CongenitalFactorIdisorders ; CongenitalFactorIIdeficiency ; CongenitalFactorVdeficiency ; CongenitalFactorVIIdeficiency ; CongenitalFactorXdeficiency ; Common Bleeding Disorders ; Von Willebrand Disorders ; Hemophilia A ; Hemophilia B ; Rare Bleeding Disorders ; Glanzmann Thrombasthenia ; Bernard-Soulier Syndrome ; Gray Platelet Syndrome ; Quebec Platelet Disorder ; Combined Coagulation Factor Defiencies ; Congenital Factor I disorders ; Congenital Factor II deficiency ; Congenital Factor V deficiency ; Congenital Factor VII deficiency ; Congenital Factor X deficiency
    Language English
    Size 480 p.
    Edition 2
    Publisher Springer International Publishing
    Document type Book
    Note PDA Manuell_24
    Format 160 x 241 x 30
    ISBN 9783031431555 ; 3031431553
    Database PDA

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  2. Book ; Online ; E-Book: Congenital bleeding disorders

    Dorgalaleh, Akbar

    diagnosis and management

    2018  

    Author's details Akbar Dorgalaleh editor
    Keywords Medicine ; Hematology ; Pediatrics
    Subject code 616.15
    Language English
    Size 1 Online-Ressource (XIV, 396 Seiten), Illustrationen, Diagramme
    Publisher Springer
    Publishing place Cham
    Publishing country Switzerland
    Document type Book ; Online ; E-Book
    Remark Zugriff für angemeldete ZB MED-Nutzerinnen und -Nutzer
    HBZ-ID HT019791899
    ISBN 978-3-319-76723-9 ; 9783319767222 ; 3-319-76723-2 ; 3319767224
    DOI 10.1007/978-3-319-76723-9
    Database ZB MED Catalogue: Medicine, Health, Nutrition, Environment, Agriculture

    Full text online

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  3. Article ; Online: Novel Insights into Heterozygous Factor XIII Deficiency.

    Dorgalaleh, Akbar

    Seminars in thrombosis and hemostasis

    2023  Volume 50, Issue 2, Page(s) 200–212

    Abstract: The prevalence and clinical significance of heterozygous factor XIII (FXIII) deficiency has long been debated, with controversial reports emerging since 1988. In the absence of large epidemiologic studies, but based on a few studies, a prevalence of 1 ... ...

    Abstract The prevalence and clinical significance of heterozygous factor XIII (FXIII) deficiency has long been debated, with controversial reports emerging since 1988. In the absence of large epidemiologic studies, but based on a few studies, a prevalence of 1 per 1,000 to 5,000 is estimated. In southeastern Iran, a hotspot area for the disorder, a study of more than 3,500 individuals found an incidence of 3.5%. Between 1988 and 2023, a total of 308 individuals were found with heterozygous FXIII deficiency, of which molecular, laboratory, and clinical presentations were available for 207 individuals. A total of 49 variants were found in the
    MeSH term(s) Pregnancy ; Female ; Humans ; Heterozygote ; Factor XIII/genetics ; Factor XIII/metabolism ; Factor XIII Deficiency/genetics ; Factor XIII Deficiency/complications ; Mutation ; Postpartum Hemorrhage
    Chemical Substances Factor XIII (9013-56-3)
    Language English
    Publishing date 2023-03-20
    Publishing country United States
    Document type Journal Article
    ZDB-ID 196901-8
    ISSN 1098-9064 ; 0094-6176
    ISSN (online) 1098-9064
    ISSN 0094-6176
    DOI 10.1055/s-0043-1764471
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: The History of Factor XIII Deficiency.

    Dorgalaleh, Akbar

    Seminars in thrombosis and hemostasis

    2023  Volume 50, Issue 1, Page(s) 34–42

    Abstract: Despite the early discovery of factor XIII (FXIII) in 1944, the diagnosis of FXIII deficiency was not made until 1960, after all the other coagulation factor deficiencies, most likely due to the normality of routine coagulation testing in FXIII ... ...

    Abstract Despite the early discovery of factor XIII (FXIII) in 1944, the diagnosis of FXIII deficiency was not made until 1960, after all the other coagulation factor deficiencies, most likely due to the normality of routine coagulation testing in FXIII deficiency. Although the first case was detected by the clot solubility test and this test has long since been used to detect FXIII deficiency, the test is no longer recommended by experts. Over the past 60 years, knowledge about FXIII deficiency has expanded considerably, between 1992, when the first variant was identified, and 2022, 197 mutations have been reported. Almost all missense mutations have a similar effect on FXIII, leading to instability and faster degradation of mutant FXIII protein. Therapeutic options have evolved from historical fresh frozen plasma (FFP), old plasma, whole blood, and cryoprecipitate, to plasma-derived and recombinant FXIII concentrates, respectively available since 1993 and 2012. These concentrate products were respectively approved by the Food and Drug Administration in 2011 and 2013. This historical review covers various aspects of FXIII related disorders, including the discovery of the FXIII, associated disorders, molecular basis, diagnosis, and treatment of FXIII deficiency.
    MeSH term(s) Humans ; Factor XIII Deficiency/diagnosis ; Factor XIII Deficiency/genetics ; Factor XIII Deficiency/therapy ; Factor XIII/genetics ; Factor XIII/metabolism ; Mutation
    Chemical Substances Factor XIII (9013-56-3)
    Language English
    Publishing date 2023-01-27
    Publishing country United States
    Document type Journal Article
    ZDB-ID 196901-8
    ISSN 1098-9064 ; 0094-6176
    ISSN (online) 1098-9064
    ISSN 0094-6176
    DOI 10.1055/s-0043-1761217
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article: Novel Insights into Heterozygous Factor XIII Deficiency

    Dorgalaleh, Akbar

    Seminars in Thrombosis and Hemostasis

    (Editorial Compilation—Part XIV)

    2023  Volume 50, Issue 02, Page(s) 200–212

    Abstract: The prevalence and clinical significance of heterozygous factor XIII (FXIII) deficiency has long been debated, with controversial reports emerging since 1988. In the absence of large epidemiologic studies, but based on a few studies, a prevalence of 1 ... ...

    Series title Editorial Compilation—Part XIV
    Abstract The prevalence and clinical significance of heterozygous factor XIII (FXIII) deficiency has long been debated, with controversial reports emerging since 1988. In the absence of large epidemiologic studies, but based on a few studies, a prevalence of 1 per 1,000 to 5,000 is estimated. In southeastern Iran, a hotspot area for the disorder, a study of more than 3,500 individuals found an incidence of 3.5%. Between 1988 and 2023, a total of 308 individuals were found with heterozygous FXIII deficiency, of which molecular, laboratory, and clinical presentations were available for 207 individuals. A total of 49 variants were found in the F13A gene, most of which were missense (61.2%), followed by nonsense (12.2%) and small deletions (12.2%), most occurring in the catalytic domain (52.1%) of the FXIII-A protein and most frequently in exon 4 (17%) of the F13A gene. This pattern is relatively similar to homozygous (severe) FXIII deficiency. In general, heterozygous FXIII deficiency is an asymptomatic condition without spontaneous bleeding tendency, but it can lead to hemorrhagic complications in hemostatic challenges such as trauma, surgery, childbirth, and pregnancy. Postoperative bleeding, postpartum hemorrhage, and miscarriage are the most common clinical manifestations, while impaired wound healing has been rarely reported. Although some of these clinical manifestations can also be observed in the general population, they are more common in heterozygous FXIII deficiency. While studies of heterozygous FXIII deficiency conducted over the past 35 years have shed light on some of the ambiguities of this condition, further studies on a large number of heterozygotes are needed to answer the major questions related to heterozygous FXIII deficiency.
    Keywords factor XIII deficiency ; heterozygous factor XIII deficiency ; miscarriage ; postpartum hemorrhage
    Language English
    Publishing date 2023-03-20
    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-0043-1764471
    Database Thieme publisher's database

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  6. Article: The History of Factor XIII Deficiency

    Dorgalaleh, Akbar

    Seminars in Thrombosis and Hemostasis

    (Celebrating 50 Years of Seminars in Thrombosis and Hemostasis—Part III)

    2023  Volume 50, Issue 01, Page(s) 34–42

    Abstract: Despite the early discovery of factor XIII (FXIII) in 1944, the diagnosis of FXIII deficiency was not made until 1960, after all the other coagulation factor deficiencies, most likely due to the normality of routine coagulation testing in FXIII ... ...

    Series title Celebrating 50 Years of Seminars in Thrombosis and Hemostasis—Part III
    Abstract Despite the early discovery of factor XIII (FXIII) in 1944, the diagnosis of FXIII deficiency was not made until 1960, after all the other coagulation factor deficiencies, most likely due to the normality of routine coagulation testing in FXIII deficiency. Although the first case was detected by the clot solubility test and this test has long since been used to detect FXIII deficiency, the test is no longer recommended by experts. Over the past 60 years, knowledge about FXIII deficiency has expanded considerably, between 1992, when the first variant was identified, and 2022, 197 mutations have been reported. Almost all missense mutations have a similar effect on FXIII, leading to instability and faster degradation of mutant FXIII protein. Therapeutic options have evolved from historical fresh frozen plasma (FFP), old plasma, whole blood, and cryoprecipitate, to plasma-derived and recombinant FXIII concentrates, respectively available since 1993 and 2012. These concentrate products were respectively approved by the Food and Drug Administration in 2011 and 2013. This historical review covers various aspects of FXIII related disorders, including the discovery of the FXIII, associated disorders, molecular basis, diagnosis, and treatment of FXIII deficiency.
    Keywords factor XIII deficiency ; factor XIII ; bleeding ; diagnosis ; management
    Language English
    Publishing date 2023-01-27
    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-0043-1761217
    Database Thieme publisher's database

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  7. Article ; Online: Bleeding and Bleeding Risk in COVID-19.

    Dorgalaleh, Akbar

    Seminars in thrombosis and hemostasis

    2020  Volume 46, Issue 7, Page(s) 815–818

    MeSH term(s) Betacoronavirus ; COVID-19 ; Coronavirus ; Coronavirus Infections ; Humans ; Pandemics ; Pneumonia, Viral ; Prognosis ; SARS-CoV-2
    Keywords covid19
    Language English
    Publishing date 2020-06-08
    Publishing country United States
    Document type Journal Article ; Comment
    ZDB-ID 196901-8
    ISSN 1098-9064 ; 0094-6176
    ISSN (online) 1098-9064
    ISSN 0094-6176
    DOI 10.1055/s-0040-1713434
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article ; Online: Erratum: Laboratory Diagnosis of Activated Protein C Resistance and Factor V Leiden.

    Bahraini, Mehran / Fazeli, Alieh / Dorgalaleh, Akbar

    Seminars in thrombosis and hemostasis

    2023  

    Language English
    Publishing date 2023-10-25
    Publishing country United States
    Document type Journal Article ; Published Erratum
    ZDB-ID 196901-8
    ISSN 1098-9064 ; 0094-6176
    ISSN (online) 1098-9064
    ISSN 0094-6176
    DOI 10.1055/s-0043-1776324
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Article ; Online: Bleeding and Bleeding Risk in COVID-19

    Dorgalaleh, Akbar

    Seminars in Thrombosis and Hemostasis

    2020  Volume 46, Issue 07, Page(s) 815–818

    Keywords Hematology ; Cardiology and Cardiovascular Medicine ; covid19
    Language English
    Publisher Georg Thieme Verlag KG
    Publishing country de
    Document type Article ; Online
    ZDB-ID 196901-8
    ISSN 1098-9064 ; 0094-6176
    ISSN (online) 1098-9064
    ISSN 0094-6176
    DOI 10.1055/s-0040-1713434
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

    Full text online

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