Abstract Introduction Congenital afibrinogenaemia and hypofibrinogenaemia are rare coagulation disorders where clotting is impaired due to a lack of fibrinogen. Consequent bleeding episodes (BEs) are treated using human fibrinogen concentrate (HFC). Aim This post‐hoc analysis compared HFC pharmacokinetics (PK) and dosing between patient age groups and ...
Claudia Djambas Khayat +6 more
wiley +1 more source
Congenital fibrinogen deficiency in Hemophilia Center Medical City/Baghdad
CONTEXT: Congenital fibrinogen deficiency is a rare inherited coagulation disorder with an estimated prevalence of 1:1,000,000 which is characterized by bleeding that varies from mild to severe and by an extremely low level or complete absence of plasma ...
Afrah A Salih +2 more
doaj +1 more source
Congenital afibrinogenemia in a 4-year-old girl complicated with acute lymphoblastic leukemia
Background. Congenital fibrinogen deficiency is one of the rare inherited coagulation disorders. Congenital fibrinogen deficiency complicated with a hematological malignancy can be life threatening. Case.
Alper Özcan +6 more
doaj +1 more source
Misdiagnosis of a patient with congenital dysfibrinogenemia: A case report and literature review
Use PT‐derived assay when fibrinogen level is reduced and thrombin time is prolonged. Abstract Background We reported a patient with congenital dysfibrinogenemia who was misdiagnosed and reviewed relevant literature, in order to discuss the methods to reduce misdiagnosis.
Xinyan Chen +3 more
wiley +1 more source
Cellulitis on face in a patient with congenital afibrinogenemia
Congenital afibrinogenemia is a rare coagulation disorder, with an estimated prevalence of 1 : 1,000,000, characterized by a complete absence to reduced level of circulating fibrinogen.
G D Chandan +4 more
doaj +1 more source
Clinical, biological, and genetic features in an afibrinogenemia patient series in Algeria
Abstract Introduction The incidence of afibrinogenemia had not been previously reported in Algeria. Afibrinogenemia patients are prone to both haemorrhagic and thrombotic complications. Predictive markers of thrombosis in afibrinogenemia patients are not existent.
Soraya Hadjali‐Saichi +8 more
wiley +1 more source
Fibrinogen concentrate for treatment of bleeding and surgical prophylaxis in congenital fibrinogen deficiency patients [PDF]
Background: Congenital fibrinogen deficiency is an ultra-rare disorder in which patients can experience severe and/or frequent bleeding episodes (BEs). Here, we present the largest prospective study to date on the treatment of this disorder.
A. Almomen +16 more
core +1 more source
Pseudohomozygous dysfibrinogenemia [PDF]
Hypodysfibrinogenemia (HD) is a heterogeneous disorder in which plasma fibrinogen antigen and function are both reduced but discordant. This report addresses the key clinical question of whether genetic analysis enables clinically useful ...
Doherty, Christopher +5 more
core +2 more sources
In vitro transcription of compound heterozygous hypofibrinogenemia Matsumoto IX; first identification of FGB IVS6 deletion of 4 nucleotides and FGG IVS3-2A > G causing abnormal RNA splicing [PDF]
ArticleClinica Chimica Acta.
Fujihara, N +7 more
core +3 more sources
Background Patients with congenital afibrinogenemia suffer from spontaneous recurrent severe bleeding. While fibrinogen concentrates are known to effectively treat bleeding episodes, thrombotic complications often occur upon replacement therapy ...
S. Le Quellec +6 more
doaj +1 more source

