Delving into the Latest Updates on Factor XIII(CSL Behring LLC) with Synapse

Overview

Basic Info

Drug Type

Non-recombinant coagulation factor

Synonyms

Blood coagulation factor XIII, Coagulation factor XIII, Corifact

+ [10]

Target

Fibrinogen

Mechanism

Factor XIII replacements

Therapeutic Areas

Cardiovascular DiseasesCongenital DisordersHemic and Lymphatic Diseases+ [1]

Active Indication

Postoperative HemorrhageFactor XIII DeficiencyVasculitis

Inactive Indication

Scleroderma, Systemic

Originator Organization

CSL Behring LLC

Active Organization

CSL Behring GmbH

CSL Behring LLC

Inactive Organization-

Drug Highest PhaseApproved

First Approval Date

JP (01 Sep 1994),

Factor XIII DeficiencyVasculitis

RegulationOrphan Drug (US)

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Gene Sequence

Sequence Code 26728

The goal of this trial is to determine if postpartum blood loss can be reduced by replenishing coagulation factor XIII (FXIII) at an early stage of postpartum hemorrhage (PPH).
Summary of current body of evidence:
* Morbidity and mortality due to PPH is rising.
* Current guidelines focus on replenishment of fibrinogen as an initial step in the treatment of PPH-related coagulopathy, despite non-conclusive evidence in all prospective trials.
* Trials from other specialties demonstrate a significant impact of FXIII on perioperative bleeding complications; a previous study at the University Hospital Zurich showed that pre-partum factor XIII activity had a strong association to postpartum blood loss.
Therefore, this nationwide, multi-center, randomized, controlled trial in multiple perinatal centers across Switzerland will be conducted. The goal is to determine if postpartum blood loss and PPH-related complications can be reduced by replenishing FXIII.
All participating women receive, according to the national guideline, 1g tranexamic acid (TXA) i.v. in case of PPH (measured blood loss [MBL] ≥ 500 mL) during the pre-study phase. Randomization takes place if bleeding continues and exceeds 700mL. The intervention group then receives FXIII (Fibrogammin®) according to approved dosage in addition to obstetric standard of care treatment for causes of PPH; the control group receives only standard of care treatment.

Start Date09 Jul 2024

Sponsor / Collaborator

University of Zurich

[+1]

DRKS00016699 / CompletedNot ApplicableIIT

Thrombelastographic investigation of the therapy of a dilutional coagulopathy with fibrinogen and factor XIII - WarmUp

Start Date04 Feb 2019

Sponsor / Collaborator-

JPRN-UMIN000019480 / Completed

Randomized controlled study of early replacement with factor XIII for fistula after pancreatic surgery (FIPS) - RCT of early replacement with factor XIII for pancreatic fistula

Start Date25 Oct 2015

Sponsor / Collaborator-

100 Clinical Results associated with Factor XIII(CSL Behring LLC)

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100 Translational Medicine associated with Factor XIII(CSL Behring LLC)

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100 Patents (Medical) associated with Factor XIII(CSL Behring LLC)

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226

Literatures (Medical) associated with Factor XIII(CSL Behring LLC)

01 Oct 2024·International Journal of Hematology

Detection of factor XIII inhibitors in 33 patients with autoimmune factor XIII deficiency in Japan

Article

Author: Osaki, Tsukasa ; Souri, Masayoshi ; Ichinose, Akitada

Autoimmune factor XIII (FXIII) deficiency (AiF13D) is a rare hemorrhagic disease. The anti-FXIII autoantibodies that cause this disease are classified into three types: type Aa inhibits the heterotetramer assembly and activation of FXIII, type Ab inhibits the enzymatic activity of activated FXIII, and type B enhances the elimination of FXIII from the blood. The former two are FXIII inhibitors and may be lethal if overlooked by conventional functional assays. To reliably detect both types of FXIII inhibitors, a new assay was developed by incorporating 5-(biotinamido)pentylamine (BAPA) into α₂-plasmin inhibitor (PI-BAPA assay). This assay was tested on plasma samples from 128 participants, including 60 healthy controls, 35 patients with non-immune acquired FXIII deficiency, and 33 patients with AiF13D (29 with type Aa inhibitors and 4 with type Ab inhibitors). The PI-BAPA assay successfully detected type Aa and Ab inhibitors in 5-step dilution cross-mixing tests between patient and normal plasma. This assay also showed comparable or superior inhibition rates in the 1:1 mixing test compared to conventional ammonia release and amine incorporation assays. Receiver operating characteristic curve analysis confirmed the excellent specificity and sensitivity of this assay for determining inhibition rates, and the assay has already been used for AiF13D diagnosis.

01 Feb 2024·Journal of Obstetrics and Gynaecology Research

Successful perinatal management of a woman with congenital factor XIII deficiency using recombinant factor XIII: A case report and literature review

Review

Author: Tokoro, Shinsuke ; Murakami, Takashi ; Takayama, Hiroshi ; Kasahara, Makiko ; Ito, Yuya ; Tsuji, Shunichiro

AbstractFactor XIII deficiency is an extremely rare autosomal recessive genetic disorder, occurring in 1 of 3–5 million people, and is associated with perinatal complications, such as habitual abortion and prolonged bleeding. Although plasma‐derived factor XIII (Fibrogamin®) carries a risk of infection and contains very low concentrated forms of factor XIII (FXIII) used for a pregnant woman with congenital coagulation factor XIII deficiency, recombinant factor XIII (rFXIII, Novo Thirteen®; Tretten®, Novo Nordisk, Bagsværd, Denmark), which has no risk of infection and is highly concentrated, has emerged as a novel formulation. Herein, we report the first case of a Japanese pregnant woman with congenital coagulation factor XIII deficiency successfully managed by rFXIII. She had a good perinatal course without pregnancy‐related complications and transfusion through the perinatal period.

29 Dec 2023·Modern Rheumatology Case Reports

A case of anti-NPX-2 antibody-positive dermatomyositis resulting in massive haemothorax with acquired factor XIII deficiency

Article

Author: Matsuda, Takuya ; Sakaguchi, Takaaki ; Otsuka, Yasunori ; Kan, Toshiaki ; Haga, Taiki

ABSTRACTAutoimmune diseases, including dermatomyositis, can be complicated by an acquired autoimmune coagulation factor XIII deficiency, which sometimes results in fatal bleeding. Here, we report the case of a young woman with anti-NPX-2 antibody-positive dermatomyositis who developed massive haemothorax with acquired factor XIII deficiency during treatment, including plasma exchange therapy. Emergency transcatheter arterial embolisation was performed and coagulation factor XIII concentrates (Fibrogammin P® 240 U/day for 5 days) were supplemented. Subsequently, the patient was discharged and managed with oral prednisolone and tacrolimus. Coagulation system test results were followed up regularly and remained within normal limits and the patient progressed without recurrence of bleeding symptoms. Coagulation factor XIII deficiency cannot be assessed without measuring coagulation factor XIII activity because common coagulation-fibrinolytic system test results are not abnormal. The measurement of factor XIII activity should be performed when autoimmune diseases are complicated by unexplained bleeding.

100 Deals associated with Factor XIII(CSL Behring LLC)

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External Link

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-	Factor XIII(CSL Behring LLC)	B02BD07	DB12909

R&D Status

Approved

10 top approved records.

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Indication	Country/Location	Organization	Date
Postoperative Hemorrhage	US	CSL Behring GmbH	17 Feb 2011
Factor XIII Deficiency	JP	CSL Behring LLC	01 Sep 1994
Vasculitis	JP	CSL Behring LLC	01 Sep 1994

Developing

10 top R&D records.

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Indication	Highest Phase	Country/Location	Organization	Date
Scleroderma, Systemic	Phase 2	GB	CSL Behring LLC	01 Sep 2015
Factor XIII Deficiency	Phase 2	ES	CSL Behring LLC	01 May 2009

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Clinical Result

Indication

Phase

Evaluation

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Study	Phase	Population	Analyzed Enrollment	Group	Results	Evaluation	Publication Date


ASH2014	Not Applicable	Acute Lymphoblastic Leukemia	-	Coagulation Factor XIII	(hwxcyijxcm) = ecmqrrleez xcswferzrx (bbefqhcpmg )	-	06 Dec 2014
				Coagulation Factor XIII	(hwxcyijxcm) = evgnxepwyu xcswferzrx (bbefqhcpmg )