دورية أكاديمية
أعرض في EDS

Physiological correction of hereditary mild hypofibrinogenemia during pregnancy.

التفاصيل البيبلوغرافية
العنوان: Physiological correction of hereditary mild hypofibrinogenemia during pregnancy.
المؤلفون: Marchi R; Department of Genetic Medicine and Development, Faculty of Medicine, University of Geneva, Geneva, Switzerland., Durual S; Biomaterials Laboratory, University Clinics of Dental Medicine, University of Geneva, Geneva, Switzerland., Pecheux O; Division of Obstetric, University Hospitals of Geneva, Geneva, Switzerland., Neerman-Arbez M; Department of Genetic Medicine and Development, Faculty of Medicine, University of Geneva, Geneva, Switzerland., Casini A; Division of Angiology and Hemostasis, University Hospitals of Geneva and Faculty of Medicine, Geneva, Switzerland.
المصدر: Haemophilia : the official journal of the World Federation of Hemophilia [Haemophilia] 2023 May; Vol. 29 (3), pp. 836-843. Date of Electronic Publication: 2023 Feb 09.
نوع المنشور: Journal Article
اللغة: English
بيانات الدورية: Publisher: Blackwell Science Country of Publication: England NLM ID: 9442916 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1365-2516 (Electronic) Linking ISSN: 13518216 NLM ISO Abbreviation: Haemophilia Subsets: MEDLINE
أسماء مطبوعة: Original Publication: Osney Mead, Oxford, UK : Blackwell Science, [1994-
مواضيع طبية MeSH: Afibrinogenemia*/genetics , Thrombosis* , Hemostatics*/pharmacology, Pregnancy ; Humans ; Female ; Blood Coagulation ; Thrombin ; Fibrinolysis ; Fibrin ; Fibrinogen/pharmacology
مستخلص: Introduction: Hereditary hypofibrinogenemia is a rare fibrinogen disorder characterised by decreased levels of fibrinogen. Pregnant women with hypofibrinogenemia are at risk of adverse obstetrical outcomes, depending on the fibrinogen level.
Aim: We investigated how the physiological changes of hemostasis throughout the pregnancy impact the hemostatic balance in a woman with hereditary mild hypofibrinogenemia.
Methods: Fibrin clot properties were analyzed by turbidimetry and scanning electron microscopy, clot weight and red blood cells retention were measured by whole clot contraction, and in vitro thrombin generation was assessed by calibrated automated thrombogram and ex vivo by TAT.
Results: Throughout the pregnancy, the fibrinogen levels increased reaching normal values in the third trimester (activity 3.1 g/L, antigen 3.2 g/L). In parallel, the fibrin polymerisation increased, the fibrinolysis decreased, the fibrin clot network became denser with thicker fibrin fibers, and the fibrin clot weight and red blood cells retention increased, reaching control's value at the third trimester. Similarly, in vitro and ex vitro thrombin generation increased, reaching maximum values at the delivery.
Conclusion: In this case of hereditary mild hypofibrinogenemia we observed a physiological increase of fibrinogen and thrombin generation. Future studies should focus on moderate and severe hypofibrinogenemia, to assess fibrinogen variation and the overall impact of increased TG on the hemostasis balance.
(© 2023 The Authors. Haemophilia published by John Wiley & Sons Ltd.)
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فهرسة مساهمة: Keywords: fibrinogen; fibrinogen disorders; hypofibrinogenemia; pregnancy; thrombin generation
المشرفين على المادة: EC 3.4.21.5 (Thrombin)
9001-31-4 (Fibrin)
0 (Hemostatics)
9001-32-5 (Fibrinogen)
تواريخ الأحداث: Date Created: 20230209 Date Completed: 20230518 Latest Revision: 20230518
رمز التحديث: 20240628
DOI: 10.1111/hae.14754
PMID: 36757142
قاعدة البيانات: MEDLINE
الوصف
تدمد:1365-2516
DOI:10.1111/hae.14754