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Management of congenital dysfibrinogenemia in pregnancy: A challenging patient case

Afibrinogenemia and congenital dysfibrinogenemia (CD) are rare conditions with limited information available for appropriate management. Previous case reports have demonstrated the safe and efficacious use of fibrinogen replacement therapy (FRT) as a therapeutic approach to prevent hemorrhage and fe...

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Autores principales: Langer, Megan, Manire, Meredith, Clarkson, Matthew, Samhouri, Yazan, Shah, Deep, Bhagavatula, Rama
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8595964/
https://www.ncbi.nlm.nih.gov/pubmed/34816075
http://dx.doi.org/10.1002/rth2.12619
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author Langer, Megan
Manire, Meredith
Clarkson, Matthew
Samhouri, Yazan
Shah, Deep
Bhagavatula, Rama
author_facet Langer, Megan
Manire, Meredith
Clarkson, Matthew
Samhouri, Yazan
Shah, Deep
Bhagavatula, Rama
author_sort Langer, Megan
collection PubMed
description Afibrinogenemia and congenital dysfibrinogenemia (CD) are rare conditions with limited information available for appropriate management. Previous case reports have demonstrated the safe and efficacious use of fibrinogen replacement therapy (FRT) as a therapeutic approach to prevent hemorrhage and fetal loss in pregnant women with CD. In this case report, we present a 28‐year‐old pregnant woman who sought testing for CD given her family history. She denied any current or previous bleeding symptoms. Laboratory testing confirmed the diagnosis of CD. She was treated with FRT and prophylactic anticoagulation starting in her third trimester. She had preterm labor that prompted an urgent cesarean section with FRT support. This case adds to the sparse literature about fibrinogen disorders in pregnancy, and highlights the benefits, safety, and tolerability of FRT and prophylactic anticoagulation in pregnant women with CD. Finally, it emphasizes the importance of a multidisciplinary team approach for an uneventful delivery.
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spelling pubmed-85959642021-11-22 Management of congenital dysfibrinogenemia in pregnancy: A challenging patient case Langer, Megan Manire, Meredith Clarkson, Matthew Samhouri, Yazan Shah, Deep Bhagavatula, Rama Res Pract Thromb Haemost Case Report Afibrinogenemia and congenital dysfibrinogenemia (CD) are rare conditions with limited information available for appropriate management. Previous case reports have demonstrated the safe and efficacious use of fibrinogen replacement therapy (FRT) as a therapeutic approach to prevent hemorrhage and fetal loss in pregnant women with CD. In this case report, we present a 28‐year‐old pregnant woman who sought testing for CD given her family history. She denied any current or previous bleeding symptoms. Laboratory testing confirmed the diagnosis of CD. She was treated with FRT and prophylactic anticoagulation starting in her third trimester. She had preterm labor that prompted an urgent cesarean section with FRT support. This case adds to the sparse literature about fibrinogen disorders in pregnancy, and highlights the benefits, safety, and tolerability of FRT and prophylactic anticoagulation in pregnant women with CD. Finally, it emphasizes the importance of a multidisciplinary team approach for an uneventful delivery. John Wiley and Sons Inc. 2021-11-16 /pmc/articles/PMC8595964/ /pubmed/34816075 http://dx.doi.org/10.1002/rth2.12619 Text en © 2021 The Authors. Research and Practice in Thrombosis and Haemostasis published by Wiley Periodicals LLC on behalf of International Society on Thrombosis and Haemostasis (ISTH). https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ (https://creativecommons.org/licenses/by-nc-nd/4.0/) License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.
spellingShingle Case Report
Langer, Megan
Manire, Meredith
Clarkson, Matthew
Samhouri, Yazan
Shah, Deep
Bhagavatula, Rama
Management of congenital dysfibrinogenemia in pregnancy: A challenging patient case
title Management of congenital dysfibrinogenemia in pregnancy: A challenging patient case
title_full Management of congenital dysfibrinogenemia in pregnancy: A challenging patient case
title_fullStr Management of congenital dysfibrinogenemia in pregnancy: A challenging patient case
title_full_unstemmed Management of congenital dysfibrinogenemia in pregnancy: A challenging patient case
title_short Management of congenital dysfibrinogenemia in pregnancy: A challenging patient case
title_sort management of congenital dysfibrinogenemia in pregnancy: a challenging patient case
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8595964/
https://www.ncbi.nlm.nih.gov/pubmed/34816075
http://dx.doi.org/10.1002/rth2.12619
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