Littérature scientifique

Année
Mois

Résultats

2024
Décembre
Soins intensifs

How I treat quantitative fibrinogen disorders

Quantitative fibrinogen disorders, including afibrinogenemia and hypofibrinogenemia, are defined by the complete absence or reduction of fibrinogen, respectively. The diagnosis is based on the measurement of fibrinogen activity and antigen levels, which define the severity of this monogenic disorder.
Immunologie

Intravenous immunoglobulin as a therapy for autoimmune conditions

Intravenous immunoglobulin (IVIg) is a medical preparation used as replacement therapy for patients with immunodeficiencies. Over time, IVIg's anti-inflammatory and immunomodulatory effects have been recognized, which have led to the approval of this therapy in the treatment of various pathologies, such as Kawasaki disease, immune thrombocytopenia, and Guillain-Barré syndrome.
Hemostase

The History of Rare Bleeding Disorders

Deficiencies in coagulation factors I (FI), FII, FV, combined FV and FVIII (CF5F8) and vitamin K-dependent coagulation factors FVII, FX, FXI, and FXIII have been referred to as rare bleeding disorders (RBDs), rare coagulation factor deficiencies (RCFDs), or recessively inherited coagulation disorders.
Hemostase

Prophylaxis in von Willebrand disease with von Willebrand factor concentrate and nonfactor therapies

This manuscript summarizes the current status of prophylaxis and novel potential therapies to prevent bleeding in patients with von Willebrand disease (VWD).
Hemostase

Pain-Related Quality of Life Outcomes in People With Haemophilia A Receiving Emicizumab: A Post Hoc Analysis of the HAVEN 1, 3 and 4 and STASEY Studies

This post hoc analysis of pooled data from the HAVEN 1 (NCT02622321), 3 (NCT02847637), 4 (NCT03020160) and STASEY (NCT0319179) studies assessed the impact of emicizumab prophylaxis on pain-related QoL in PwHA.
Hemostase

EAHAD statement on the ISTH clinical practice guideline for treatment of congenital haemophilia A and B

The European Association for Haemophilia and Allied Disorders (EAHAD) commends the International Society’s on Thrombosis and Haemostasis (ISTH) initiative to publish evidence-based guidelines.
Hemostase

The history of women and hemophilia: a narrative review of evolving beliefs and testing practices

The history of hemophilia is well-documented yet reports focus heavily on the male-perspective and severe forms of the disease. Although hemophilia was initially believed to only affect men with women seen as silent carriers, it is now universally acknowledged that women and girls can also be affected.
Hemostase

Blood coagulation factor IX: structural insights impacting hemophilia B therapy

Coagulation factor IX plays a central role in hemostasis through interaction with factor VIIIa to form a factor X-activating complex at the site of injury. The absence of factor IX activity results in the bleeding disorder hemophilia B. This absence of activity can arise either from a lack of circulating factor IX protein or mutations that decrease the activity of factor IX.
Hemostase

International Society on Thrombosis and Haemostasis Clinical Practice Guideline for Treatment of Congenital Haemophilia-A Critical Appraisal

Evidence-based clinical practice guidelines drive optimal patient care and facilitate access to high-quality treatment. Creating guidelines for rare diseases such as haemophilia, where evidence does not often come from randomized controlled trials but from non-randomized and well-designed observational studies and real-world data, is challenging.
Hemostase

Looking Beyond Thrombocytopenia for Bleeding in a Case of Dengue Fever: From Dengue to Hemophilia C

Dengue fever causes thrombocytopenia, which can present with bleeding manifestations. Bleeding can range from less severe conditions like petechiae, epistaxis, and menorrhagia, to life-threatening situations like gastrointestinal bleed or intracranial bleed.
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