Disorders of fibrinogen and fibrinolysis

JE May, AS Wolberg, MY Lim - Hematology/Oncology …, 2021 - hemonc.theclinics.com
BACKGROUND Fibrinogen is a large (340 kDa) hexameric glycoprotein expressed by
hepatocytes. 1 Fibrinogen is encoded by 3 different genes (FGA, FGB, and FGG) and …

Management of rare inherited bleeding disorders: proposals of the French Reference Centre on Haemophilia and Rare Coagulation Disorders

M Trossaert, V Chamouard… - European Journal of …, 2023 - Wiley Online Library
Introduction The rare coagulation disorders may present significant difficulties in diagnosis
and management. In addition, considerable inter‐individual variation in bleeding phenotype …

Human fibrinogen: molecular and genetic aspects of congenital disorders

GL Tiscia, M Margaglione - International Journal of Molecular Sciences, 2018 - mdpi.com
Congenital fibrinogen disorders can be quantitative (afibrinogenemia, hypofibrinogenemia)
or functional (dysfibrinognemia). To date, several genetic variants have been identified in …

[HTML][HTML] Heterogeneity of congenital afibrinogenemia, from epidemiology to clinical consequences and management

A Casini, M Neerman-Arbez, P De Moerloose - Blood Reviews, 2021 - Elsevier
Fibrinogen is a complex protein playing a major role in coagulation. Congenital
afibrinogenemia, characterized by the complete absence of fibrinogen, is associated with …

Congenital fibrinogen disorders: strengthening genotype–phenotype correlations through novel genetic diagnostic tools

R Ramanan, JD McFadyen… - British Journal of …, 2023 - Wiley Online Library
Congenital fibrinogen disorders or CFDs are heterogenous, both in clinical manifestation
and array of culprit molecular lesions. Correlations between phenotype and genotype …

Big picture initiatives in bleeding disorders

A Rauch, LA Valentino, K Mills, ML Witkop… - …, 2022 - Wiley Online Library
Introduction The inherited bleeding disorders (IBD) community has witnessed significant
therapeutic advances recently, yet important gaps persist, particularly for those with rare …

Fibrinogen concentrates in hereditary fibrinogen disorders: Past, present and future

A Casini, P de Moerloose - Haemophilia, 2020 - Wiley Online Library
Hereditary fibrinogen disorders (HFD) are rare coagulation disorders. Even if the spectrum
of symptoms is broad depending on the sub‐type, bleeding is the most common …

Perioperative management of a severe congenital hypofibrinogenemia with thrombotic phenotype

T Simurda, A Casini, J Stasko… - Thrombosis …, 2020 - thrombosisresearch.com
Dear Editors, we had read with a great interest the paper written by Lim et al., published in a
recent issue of the journal with title: Global coagulation assays—is there a role in thrombosis …

International council for standardization in haematology recommendations for hemostasis critical values, tests, and reporting

RC Gosselin, D Adcock, A Dorgalaleh… - … in Thrombosis and …, 2020 - thieme-connect.com
This guidance document was prepared on behalf of the International Council for
Standardization in Haematology (ICSH), the aim of which is to provide hemostasis-related …

Comparison of Fibrinogen concentrations determined by the Clauss method with prothrombin-derived measurements on an automated Coagulometer

B Guven, M Can, A Tekin - The Journal of Applied Laboratory …, 2022 - academic.oup.com
Background This research aims to compare fibrinogen results, obtained from the Clauss and
PT-derived method on the Cobas t511 analyzer, in patients with specific categories of …