凝血因子XIII(Factor XIII(CSL Behring LLC)) - 药物靶点：Fibrinogen_在研适应症：术后出血，凝血因子XIII缺乏症，血管炎_专利_临床

概要

基本信息

药物类型

非重组凝血因子

别名

Blood coagulation factor XIII、Coagulation factor XIII、Corifact

+ [10]

靶点

Fibrinogen

作用机制

凝血因子XIII替代物

治疗领域

心血管疾病遗传病与畸形血液及淋巴系统疾病+ [1]

在研适应症

非在研适应症

原研机构

在研机构

非在研机构-

最高研发阶段批准上市

首次获批日期

日本 (1994-09-01),

凝血因子XIII缺乏症血管炎

最高研发阶段(中国)-

特殊审评孤儿药 (美国)

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结构/序列

Sequence Code 26728

关联

6

项与凝血因子XIII 相关的临床试验

NCT06481995

/ Recruiting临床4期IIT

Early Factor XIII Replacement in Postpartum Hemorrhage: Multi-center, Randomized, Controlled, Investigator-initiated Trial

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.

开始日期2024-07-09

申办/合作机构

University of Zurich

[+1]

DRKS00016699

/ CompletedN/AIIT

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

开始日期2019-02-04

申办/合作机构-

NCT02551042

/ Unknown status临床2期IIT

A Phase II, Double-blind, Randomized, Placebo-controlled Study to Investigate Pharmacokinetics (PK), Safety and Efficacy of Intravenous Factor XIII Treatment in Patients With Systemic Sclerosis

Many patients with Scleroderma (Systemic sclerosis) experience damage to blood vessels, mainly to the small arteries. A common manifestation of this is Raynaud's phenomenon (fingers or toes turning white then blue in the cold) and digital ulcers (open sores on the fingertips). The purpose of this study is to see how effective the study drug Human Factor XIII Concentrate is in treating patients who have these and other common manifestation of Scleroderma. It will be given in addition to the accepted treatments used for this disease.

开始日期2015-09-01

申办/合作机构

University College London

[+1]

100 项与凝血因子XIII 相关的临床结果

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100 项与凝血因子XIII 相关的转化医学

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100 项与凝血因子XIII 相关的专利（医药）

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229

项与凝血因子XIII 相关的文献（医药）

2024-10-01·International Journal of Hematology

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

Article

作者: 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.

2024-02-01·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

作者: 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.

2023-12-29·Modern Rheumatology Case Reports

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

Article

作者: 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 项与凝血因子XIII 相关的药物交易

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外链

KEGG	Wiki	ATC	Drug Bank
-	凝血因子XIII	B02BD07	DB12909

研发状态

批准上市

10 条最早获批的记录,

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适应症	国家/地区	公司	日期
术后出血	美国	CSL Behring GmbH	2011-02-17
凝血因子XIII缺乏症	日本	CSL Behring LLC	1994-09-01
血管炎	日本	CSL Behring LLC	1994-09-01

未上市

10 条进展最快的记录,

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适应症	最高研发状态	国家/地区	公司	日期
系统性硬皮病	临床2期	英国	CSL Behring LLC	2015-09-01
凝血因子XIII缺乏症	临床2期	西班牙	CSL Behring LLC	2009-05-01

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临床结果

适应症

分期

评价

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研究	分期	人群特征	评价人数	分组	结果	评价	发布日期


ASH2014	N/A	急性淋巴细胞白血病	-	Coagulation Factor XIII (FXIIIA-positive ALL)	襯簾鏇選顧蓋顧襯積遞(顧選鹹築醖夢積鑰襯淵) = 鹹繭餘齋願遞憲網鏇簾窪獵構蓋願鹽願艱鏇廠 (願製顧顧憲夢壓鹹鏇夢 )	-	2014-12-06
ASH2014	N/A	急性淋巴细胞白血病	-	Coagulation Factor XIII (FXIIIA-negative ALL)	襯簾鏇選顧蓋顧襯積遞(顧選鹹築醖夢積鑰襯淵) = 構築壓觸憲繭築窪憲獵窪獵構蓋願鹽願艱鏇廠 (願製顧顧憲夢壓鹹鏇夢 )	-	2014-12-06
NCT00883090 (CTgov)	临床2期	凝血因子XIII缺乏症	15	FXIII Concentrate (Human)	齋齋選淵築廠蓋製衊繭(糧範選蓋衊蓋遞鏇築繭) = 鑰衊簾鹽築蓋餘構衊膚壓餘夢鑰膚壓繭獵淵淵 (鬱範淵齋壓衊襯繭築鹽, 鹹觸襯顧襯製簾繭夢鏇 ~ 襯憲獵繭廠築網衊醖構) 更多	-	2012-01-16