Results 21 to 30 of about 850 (150)

Diagnostic utility of bleeding assessment tools in congenital fibrinogen deficiencies

open access: yesHaemophilia, Volume 29, Issue 3, Page 827-835, May 2023., 2023
Abstract Background The assessment of clinical history is crucial before referring a patient for further laboratory testing. Bleeding assessment tools (BAT) are developed to standardize clinical evaluation. A small number of patients with congenital fibrinogen deficiencies (CFDs) have been evaluated with these tools without definitive results.
Samin Mohsenian   +8 more
wiley   +1 more source

Combined life-threatening thromboses and hemorrhages in a patient with afibrinogenemia and antithrombin deficiency

open access: yesThrombosis Journal, 2018
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

Global epidemiology of factor XI deficiency: A targeted review of the literature and foundation reports

open access: yesHaemophilia, Volume 29, Issue 2, Page 423-434, March 2023., 2023
Abstract Introduction Hereditary factor XI (FXI) deficiency is a rare coagulation disorder that may result in excessive bleeding requiring intervention to restore haemostasis. Aim The aim of this review was to report the current knowledge of the worldwide incidence and prevalence of FXI deficiency.
Xinruo Zhang   +5 more
wiley   +1 more source

Pseudohomozygous dysfibrinogenemia

open access: yesResearch and Practice in Thrombosis and Haemostasis, 2021
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 ...
Rachel C. Peck   +5 more
doaj   +1 more source

A Causal Classification System for Intracerebral Hemorrhage Subtypes

open access: yesAnnals of Neurology, Volume 93, Issue 1, Page 16-28, January 2023., 2023
Objective Determining the underlying causes of intracerebral hemorrhage (ICH) is of major importance, because risk factors, prognosis, and management differ by ICH subtype. We developed a new causal CLASsification system for ICH Subtypes, termed CLAS‐ICH, based on recent advances in neuroimaging.
Nicolas Raposo   +13 more
wiley   +1 more source

Addressing some challenges of congenital fibrinogen disorders in 2023 and beyond

open access: yesBleeding, Thrombosis and Vascular Biology, 2023
Congenital fibrinogen disorders (CFD) include several types and subtypes of fibrinogen deficiency, resulting from monoallelic or biallelic mutations in one of the three fibrinogen genes. While it is relatively easy to make an accurate diagnosis based on
Cristina Santoro, Alessandro Casini
doaj   +3 more sources

Recurrent myocardial infarction in a case of congenital Afibrinogenemia

open access: yesHeart Views, 2014
Afibrinogenemia is a rare autosomal recessive bleeding disorder with an estimated prevalence of 1:1,000,000. Usual presentation of this disorder is spontaneous bleeding, bleeding after minor trauma and excessive bleeding during interventional procedures.
Soumya Patra   +4 more
doaj   +1 more source

Isolated scalp hematoma: An unusual presentation of congenital afibrinogenemia

open access: yesIndian Pediatrics Case Reports, 2023
Background: Congenital fibrinogen disorder is a rare autosomal recessive blood coagulation disorder, where majority of patients present with bleeding, whereas a few may paradoxically develop thrombosis.
Arnab Ghorui   +2 more
doaj   +1 more source

Congenital Afibrinogenemia presenting as antenatal intracranial bleed: a case report

open access: yesItalian Journal of Pediatrics, 2010
Congenital afibrinogenemia is a very rare inherited coagulation disorder, characterized by virtual absence of plasma fibrinogen (factor I). There are only about 250 cases reported in the world literature 1.
Parapurath Rajiv   +2 more
doaj   +1 more source

Pregnancy outcome in afibrinogenemia: Are we giving enough fibrinogen concentrate? A case series

open access: yesResearch and Practice in Thrombosis and Haemostasis, 2020
Congenital afibrinogenemia is a rare autosomal recessive disorder associated with an increased risk of hemorrhage, thrombosis, and obstetric complications. This case series of 4 pregnancies in 2 related patients seeks to address the key clinical question
Joline L. Saes   +3 more
doaj   +1 more source

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