ReviewIranian journal of pediatric hematology and oncology2013
Current understanding in diagnosis and management of factor XIII deficiency.
Review in Iranian journal of pediatric hematology and oncology, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers.
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Who cites it
9 citing papers in PubMed.
- Acquired Coagulation Factor XIII Deficiency With Spontaneous Splenic Rupture: A Case Report.Clinical case reports · 2026Article
- Delayed and prolonged umbilical stump bleeding in a Caucasian newborn as a presenting feature of factor XIII deficiency.BMJ case reports · 2022Article
- Recombinant Destabilase fromCurrent issues in molecular biology · 2021Article
- How to Assess Founder Effect in Patients with Congenital Factor XIII Deficiency.International journal of hematology-oncology and stem cell research · 2020Review
- A novel Cys328-terminator mutant implicated in severe coagulation factor XIII deficiency: a case report.BMC medical genetics · 2020Article
- Article
- Effects of Factor XIII Deficiency on Thromboelastography. Thromboelastography with Calcium and Streptokinase Addition is more Sensitive than Solubility Tests.Mediterranean journal of hematology and infectious diseases · 2016Article
- Factor XIII deficiency and head trauma: management and therapy.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2015Article
- Clinical manifestations and management of life-threatening bleeding in the largest group of patients with severe factor XIII deficiency.International journal of hematology · 2014Article
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Authors and funding
6 authors.
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No grant is acknowledged in the PubMed record.
Abstract
Factor XIII or "fibrin-stabilizing factor," is a transglutaminase circulates in the blood circulation as a hetero tetramer with two catalytic A subunits and two carrier B subunits. This important coagulation factor has a crucial role in clotting cascade and produces strong covalent bonds between soluble formed fibrin monomers during coagulation. This stable cross linked fibrin strands are resistanttodegradationby thefibrinolyticsystem that enablesthe bodyto stoppotential bleeding episodes. In the absence or severe decrease of factor XIII, although the clot is formed, but is rapidly degraded by the fibrinolytic system, and delayed bleedingoccurs.Factor XIII deficiency is an extremely rare bleeding disorder with estimated incidence of 1/2-3000, 000 in the general population. Presumptive diagnosis of factor XIII deficiency was by clot solubility test in 5M urea or 1% monochloroacetic acid environments. In patients with abnormal screening clot solubility test, the disease can be confirmedbymore specifictestssuch as quantitative factor XIII activity assay andFXIIIAgassay.After diagnosis of disease all patients with severe factor XIII deficiency(<1 U/dl) shouldreceive prophylactic substitution therapywith fresh frozen plasma (FFP) and cryoprecipitate as traditional choices or purified concentrateof blood coagulation factor XIII (Fibrogammin P) inorder to control severe and life-threatening clinical complications of factor XIII deficiency.
Identifiers
24575291PMC3915454What OpenQuestion holds
Registered trials
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