Safety and pharmacokinetics of recombinant factor XIII-A2 administration in patients with congenital factor XIII deficiency

Lovejoy, Amy E.; Reynolds, Tom C.; Visich, Jennifer; Butine, Michael D.; Young, Guy; Belvedere, Alessandra; Blain, Rachelle; Pederson, Susan; Ishak, Laura; Nugent, Diane J.

doi:10.1182/blood-2005-02-0788

Cited by 103 publications

(106 citation statements)

References 31 publications

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“…In the field of platelet disorders, the thromboelastograph has demonstrated laboratory evidence of response to treatment with agents such as rFVIIa [27]. Other coagulation factor deficiencies such as FXIII deficiency are difficult to diagnose and monitor due to lack of universally available laboratory tests, and thromboelastography has been shown to be extremely useful in this situation [28]. Modified thromboelastography (TEG Platelet Mapping Ò ) has also been extensively used to monitor antiplatelet agents and prevent bleeding complications [29].…”

Section: Clinical Applicationsmentioning

confidence: 99%

Tests of global haemostasis and their applications in bleeding disorders

et al. 2010

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Summary. There is a potential for significant paradigm shift in the assessment of haemostasis from the conventional plasma recalcification times, such as prothrombin time (PT) and activated partial thromboplastin time (APTT), which correspond to artificially created compartments of haemostasis to tests that assess the entire process in a more physiological and holistic manner. These include the thrombin generation test, thromboelastogram and the clot wave form analysis. While these tests have been described many years ago, there is renewed interest in their use with modified technology for assessing normal haemostasis and its disorders. Although early data suggest that they can provide much greater information regarding the overall haemostasis process and its disorders, many challenges remain. Some of them are possible only on instruments that are proprietary technology, expensive and are not widely available. Furthermore, these tests need to be standardized with regard to their reagents, methodology and interpretation, and finally, much more data need to be collected regarding clinical correlations with the parameters measured.

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Section: Clinical Applicationsmentioning

confidence: 99%

Tests of global haemostasis and their applications in bleeding disorders

et al. 2010

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“…It plays a pivotal role in promoting clot stability. Factor XIII improves the mechanical strength of the fibrin clot and protect it from proteolytic degradation by forming covalent bonds between fibrin monomers and by cross-linking alpha-2 antiplasmin, fibrinogen, fibronectin, collagen, and other proteins (Lovejoy et al, 2006). Approximately 20-30% of factor XIII in the original plasma remains in CRYO (Klein & Anstee, 2005).…”

Section: Factor XIIImentioning

confidence: 99%

Cryoprecipitate transfusion: assessing appropriateness and dosing in trauma

Nascimento¹,

Rizoli²,

Rubenfeld³

et al. 2011

Transfusion Medicine

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Cryoprecipitate is commonly used outside guidelines. In trauma, the appropriate cryoprecipitate dose and its impact on plasma fibrinogen levels are unclear.This retrospective study aims to evaluate: (1) the appropriateness of cryoprecipitate transfusion in trauma; and (2) the plasma fibrinogen response to cryoprecipitate transfusion during massive transfusion in trauma.Fibrinogen levels of < 1.0 g/L within 2 and 6 hours of cryoprecipitate transfusion were used for assessing appropriateness. Out of 394 events, 238 (60%) and 259 (66%) were considered appropriate using 2 and 6 hour criteria, respectively. A dose of 8.7 (±1.7) units caused a mean increase in fibrinogen levels of 0.55 (±0.24) g/L, or 0.06g/L per unit.In our hospital, where transfusion guidelines and policies for rapid blood product and laboratory turnaround times exist, it is possible to achieve high rates of appropriateness for cryoprecipitate transfusion in trauma. The current recommended dose causes a modest increase in fibrinogen levels.iii

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“…in both healthy volunteers (36) and patients with congenital FXIII deficiency (8), and thus has more recently been used in a phase III prophylaxis trial for congenital FXIII deficiency (37).…”

Section: Instability Of Naturally Recurring R260c Mutant Of Fxiii-amentioning

confidence: 99%

“…Because FXIII is composed of FXIII-A and FXIII-B, and FXIII-B protects FXIII-A in vitro (5, 6) and in vivo (7,8), a genetic defect of either subunit leads to FXIII deficiency. Accordingly, FXIII deficiency is classified into two categories at the DNA level: FXIII-A deficiency and FXIII-B deficiency (9).…”

mentioning

confidence: 99%