2021
DOI: 10.1182/blood.2020010116
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How I treat dysfibrinogenemia

Abstract: Congenital dysfibrinogenemia (CD) is caused by structural changes in fibrinogen that modify its function. Diagnosis is based on discrepancy between decreased fibrinogen activity and normal fibrinogen antigen levels and is confirmed by genetic testing. CD results from monoallelic mutations in fibrinogen genes leading to clinically heterogenous disorders. Most patients with CD are asymptomatic at time of diagnosis but the clinical course may be complicated by a tendency to bleeding and/or thrombosis. Patients wi… Show more

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Cited by 28 publications
(29 citation statements)
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“…These include an accurate preconception counselling, a quarterly assessment of fibrinogen activity, a systematic monitoring of foetal growth, a fibrinogen supplementation targeting a fibrinogen trough level >1 g/L (1.5 g/L at labour for the neuraxial analgesia), avoiding of invasive foetal procedures and forceps delivery, early fibrinogen supplementation and antifibrinolytic drug in case of post-partum haemorrhage, accurate thromboprophylaxis if needed. 28 Our study has some limitations. The first is that we did not compare the patient to a healthy pregnant control.…”
Section: Discussionmentioning
confidence: 89%
See 1 more Smart Citation
“…These include an accurate preconception counselling, a quarterly assessment of fibrinogen activity, a systematic monitoring of foetal growth, a fibrinogen supplementation targeting a fibrinogen trough level >1 g/L (1.5 g/L at labour for the neuraxial analgesia), avoiding of invasive foetal procedures and forceps delivery, early fibrinogen supplementation and antifibrinolytic drug in case of post-partum haemorrhage, accurate thromboprophylaxis if needed. 28 Our study has some limitations. The first is that we did not compare the patient to a healthy pregnant control.…”
Section: Discussionmentioning
confidence: 89%
“…Expert recommendations for management of pregnancies in dysfibrinogenemia could also apply to hypofibrinogenemia. These include an accurate preconception counselling, a quarterly assessment of fibrinogen activity, a systematic monitoring of foetal growth, a fibrinogen supplementation targeting a fibrinogen trough level >1 g/L (1.5 g/L at labour for the neuraxial analgesia), avoiding of invasive foetal procedures and forceps delivery, early fibrinogen supplementation and antifibrinolytic drug in case of post‐partum haemorrhage, accurate thromboprophylaxis if needed 28 …”
Section: Discussionmentioning
confidence: 99%
“…8 Besides the bleeding risk, dysfibrinogenemia can also increase the thrombotic risk, illustrating the versatile function of fibrinogen in coagulation. 42 The first functionally characterized thrombotic-associated fibrinogen variant is Fibrinogen Dusart, also named Paris V or Chapel Hill III. 43 In 1983, Jeannette and Claudine Soria with Jacques Caen described four members of a family presenting with recurrent thrombosis.…”
Section: First Descriptions Of Afibrinogenemia and Dysfibrinogenemiamentioning
confidence: 99%
“…Altogether, clinical laboratories will have to manage three different situations: Perform diagnostic test with IVDR products used according with clinical indication certified by manufacturers (i.e., prothrombin time for monitoring vitamin K antagonists or coagulation factor measurements). Perform diagnostic test with certified IVDR products without clinical validation (i.e., coagulation factor inhibitor measurements). Perform diagnostic test using reagents without EU certification but classified as Research Use Only (RUO) (i.e., reptilase time useful in the diagnosis of dysfibrinogenemia, with reagents provided by manufacturers which have not undergone the IVDR certification process) 20,21 …”
Section: Impact Of Ivdr On Laboratory Medicinementioning
confidence: 99%