Management of congenital quantitative fibrinogen disorders: a Delphi consensus

Casini, Alessandro; Moerloose, Philippe de

doi:10.1111/hae.13061

Cited by 41 publications

(66 citation statements)

References 44 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…26 Supplementary fibrinogen infusions are required in the postsurgical period to target fibrinogen levels at least >1 g L −1 until haemostasis is achieved and above 0.5 g L −1 until wound healing is complete. 26 Supplementary fibrinogen infusions are required in the postsurgical period to target fibrinogen levels at least >1 g L −1 until haemostasis is achieved and above 0.5 g L −1 until wound healing is complete.…”

Section: Effi C Ac Ymentioning

confidence: 99%

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

Casini

Moerloose²

2019

Haemophilia

View full text Add to dashboard Cite

Hereditary fibrinogen disorders (HFD) are rare coagulation disorders resulting from mono-or bi-allelic mutations in fibrinogen genes. 1According to the levels of fibrinogen activity and antigen, HFD are classified into quantitative (afibrinogenemia and hypofibrinogenemia) or qualitative (dysfibrinogenemia and hypodysfibrinogenemia) disorders. 2 The spectrum of symptoms is broad and heterogeneous among patients with HFD, even if bleeding is the most common complication of all HFD sub-types. 3 The bleeding phenotype is mostly correlated to the fibrinogen level, 4 ranging from life-threatening haemorrhage to postsurgical bleeding. In afibrinogenemia, the majority of patients suffer from severe bleeding such as umbilical cord bleeds, muscle haematomas, haemarthroses and bleeding in the central nervous system. 5 Paradoxically, thrombotic events can occur in patients with afibrinogenemia, even in the absence of fibrinogen administration. 6 In hypofibrinogenemia, spontaneous bleeding is less frequent and complications are mainly associated with trauma or surgery. 7 In dysfibrinogenemia, the cumulative incidence of major bleeding has been estimated to be about 19% at 50 years of age. 8 However, some dysfibrinogen variants are strongly associated with an increased risk of thrombosis. 9 In all types of HFD, surgery and pregnancy are high-risk bleeding situations.As for most coagulation factor deficiencies, in HFD, the bleeding prevention and management is based on replacement of the lacking protein. Three sources of fibrinogen are currently available: fresh frozen plasma, cryoprecipitate and lyophilized fibrinogen concentrate. 10 The latter is the best option, although still not widely available. It confers several advantages compared to fresh frozen plasma and cryoprecipitate: (a) it represents a safer therapeutic option due AbstractHereditary 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 complication. Of the available sources of fibrinogen replacement, fibrinogen concentrate provides a safer and more effective option to treat and prevent bleeding.Recent clinical trials on established and new fibrinogen concentrates have increased our knowledge on the clinical pharmacology of these products, pointing out possible age and weight differences for dose adjustment. The efficacy of fibrinogen infusions has been demonstrated, especially for the management of acute bleeding with an excellent response based on investigator rating. The target fibrinogen levels in the setting of both minor and major surgeries have been better specified. The safety has been confirmed with a low number of adverse events but there still remains concern over possible thrombotic risks. Pharmacological, clinical aspects and future perspectives on the utilization of fibrinogen concentrates in the treatment and prevention of bleeding in patients with HFD are reviewed.

show abstract

Section: Effi C Ac Ymentioning

confidence: 99%

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

Casini

Moerloose²

2019

Haemophilia

View full text Add to dashboard Cite

show abstract

“…Delphi methodology has been already proposed with success to reach a consensus in a variety of complex medical issues [e.g., see Ref. (23–25)]. Delphi methodology has also been used in the tinnitus field (26).…”

mentioning

confidence: 99%

Call for an Evidence-Based Consensus on Outcome Reporting in Tinnitus Intervention Studies

Londero

Hall

2017

Front. Med.

View full text Add to dashboard Cite

“…They reported that fibrinogen levels significantly increased after fibrinogen substitution therapy [19]. According to Casini et al [20], the following formula should be used to evaluate the amount of fibrinogen to be administered = (target fibrinogen level [g/L] -basal fibrinogen level) 9 0.043 9 ?? weight (kg) in patients suffering from afibrinogenemia or congenital hypofibrinogenemia.…”

Section: Discussionmentioning

confidence: 99%

Reducing Fibrinogen and Factor XIII Using Double-Filtration Plasmapheresis for Antibody-Mediated Rejection: Predictive Models

et al. 2018

View full text Add to dashboard Cite

Background/Aims: Antibody-mediated rejection (AMR) is related to circulating donor-specific anti-human leukocyte antigen alloantibodies (DSAs). DSAs can be removed by apheresis, for example, double-filtration plasmapheresis (DFPP). However, DFPP removes some clotting factors (fibrinogen and factor XIII [FXIII]). Methods: This was a prospective trial including 6 DSA-mediated AMR kidney transplant recipients. Patients received 2 cycles of 3–4 consecutive DFPP sessions followed by 1 injection of rituximab (break of 4–5 days between the 2 cycles). We monitored fibrinogen and FXIII levels before and after each session of DFPP. Results: Overall, fibrinogen and FXIII levels were significantly decreased after each session, and were significantly reduced between the very first and very last sessions. In addition, we established a model that predicted fibrinogen and FXIII values after each session and after 2 cycles. Conclusion: We established a model in order to predict fibrinogen and FXIII depletion after DFPP sessions; it may help clinicians supplement fibrinogen and/or FXIII when appropriate.

show abstract

Management of congenital quantitative fibrinogen disorders: a Delphi consensus

Abstract: The results of this initiative should help clinicians in the difficult management of patients with congenital fibrinogen disorders.

Cited by 41 publications

References 44 publications

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

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

Call for an Evidence-Based Consensus on Outcome Reporting in Tinnitus Intervention Studies

Reducing Fibrinogen and Factor XIII Using Double-Filtration Plasmapheresis for Antibody-Mediated Rejection: Predictive Models

Contact Info

Product

Resources

About