German Clinical Trials Register

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Brief summary in lay language

Recommendations for FXIII supplementation are inconsistent. Therefore, identification of patients who benefit from FXIII administration is critical. The aim of this data collection is to gain further knowledge about the therapy of patients with non-congenital FXIII deficiency. Primary Goal: Generate real world evidence of what level of FXIII activity leads to supplementation. Secondary Objectives: To investigate triggers for FXIII determination. Generate Real World evidence on patient characteristics, diagnoses, and outcomes of FXIII supplementation. Existing hospital records will be retrospectively evaluated through March 31, 2022. In the first part, facilities are encouraged to document FXIII laboratory testing for 20-30 consecutive patients at their hospital in the one-page "Documentation Form for Measured FXIII Activities." Data on treatment modalities and outcome of FXIII supplementation are to be collected from patient medical records and transcribed into standardized paper documentation forms (approximately 4-5 pages) by local staff.

Brief summary in scientific language

Studies have shown that the administration of Factor XIII (FXIII) results in clinical benefit in a number of previously unstudied a number of previously unstudied indications outside of congenital FXIII deficiency. These include perioperative bleeding, systemic sclerosis, invasive bacterial infections such as necrotizing fasciitis, and wound healing disorders. These studies generally did not have sufficient statistical power to draw definitive conclusions. Acquired FXIII deficiency can be manifested by spontaneous or delayed life-threatening hemorrhages manifestation [1]. Causes of acquired deficiency include immune-mediated inhibition as well as Increased consumption or hyposynthesis without involvement of the immune system. The identification of FXIII deficiency and its underlying cause is imperative, as treatment options vary according to the etiology. Treatment options vary according to etiology. One of the most common situations with increased consumption of coagulation factors is the is the postoperative state, as clotting factors are needed to optimize clot formation and wound healing, which is wound healing, leading to a decrease in FXIII levels. Lison et al. have shown that FXIII activity is reduced after various types of major surgery (abdominal, vascular, oral/ maxillofacial tumor surgery, gynecologic tumor surgery, trauma surgery) is significantly reduced on day 1 to a a median of 76%. While other coagulation factors, such as factors II, VII, VIII and X, return to baseline levels by postoperative day 3, FXIII remains stable with a median activity level of 66% by postoperative day 6, remains persistently low [2]. Therefore, identification of patients who will benefit from FXIII administration is of critical importance. Factor XIII deficiency often manifests itself in unstoppable, diffuse, or postoperative bleeding. The problem is not recognized and can lead to a massive need for transfusions. In addition, the role of FXIII in wound healing, severe burns and pressure ulcers has been documented. Individual studies have shown that, particularly in patients with Heat injuries may have developed FXIII deficiency (<60% factor XIII) [3]. Substitution of substitution of factor XIII in these patients leads to a reduced need for transfusion and faster wound wound healing [4]. Plasma-derived factor XIII concentrates may have the following effects [5]: - For prophylaxis of congenital FXIII deficiency, - for perioperative treatment of surgical bleeding in congenital FXIII deficiency, - in hemorrhagic diathesis caused or contributed to by acquired FXIII deficiency, - for the supportive treatment of wound healing disorders, especially in the case of ulcus cruris, after extensive operations and injuries. At an advisory board on Jan. 29, 2019, German experts recommended factor XIII measurement and substitution especially in the following clinical situations: Oozing and/or postoperative bleeding and factor XIII deficiency with infection/sepsis or hepatic/renal insufficiency or extensive soft tissue defect. In addition, also in relevant FXIII deficiency in patients with Burns and/ or the need for mesh graft transplantation. Furthermore, national and international guidelines do not provide a consistent recommendation on the supplementation of FXIII: The ESA guidelines for the management of severe perioperative bleeding recommend the administration of factor XIII in cases of bleeding and low factor XIII activity (e.g., <30%) [6]. The European guideline on the management of major bleeding and coagulation disorders after trauma suggests monitoring of FXIII and supplementation in bleeding patients with a functional FXIII functional deficiency [7].