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

作用方式-

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

术后出血凝血因子XIII缺乏症血管炎

非在研适应症

血友病系统性硬皮病

原研机构

CSL Behring LLC

在研机构

CSL Behring LLCCSL Behring GmbH

非在研机构-

权益机构-

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-

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结构/序列

Sequence Code 26728

关联

5

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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148

项与凝血因子XIII 相关的文献（医药）

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

ABSTRACT:

Autoimmune 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.

2023-08-01·The clinical respiratory journal

A retrospective comparison between digital to conventional drainage systems for secondary spontaneous pneumothorax related to diffuse interstitial lung disease.

Review

作者: Hirama, Ryutaro ; Kawasaki, Takeshi ; Nakamura, Hidenori ; Horiuchi, Dai ; Shikano, Kohei ; Ishii, Daisuke ; Maruyama, Kanae ; Suzuki, Takuji ; Sakuma, Noriko ; Abe, Mitsuhiro ; Kitahara, Shinsuke

INTRODUCTION:

Secondary spontaneous pneumothorax (SSP) occurs as one of the complications associated with interstitial pneumonia (IP). Chest drainage is performed when there is a large volume of air in the pleural space. Notably, SSP with IP (SSP-IP) is frequently not curable by chest drainage only. A digital drainage system (DDS) provides an objective evaluation of air leakage and maintains a pre-determined negative pressure, compared to an analog drainage system (ADS). Few studies have reported the effectiveness of DDS in the treatment of SSP-IP. This study aimed to assess the usefulness of DDS for SSP-IP.

METHODS:

This retrospective study included patients with SSP-IP who had undergone chest drainage. We reviewed the included patients' medical records, laboratory data, computed tomography findings, and pulmonary function data.

RESULTS:

DDS was used in 24 patients and ADS in 49 patients. The mean duration of chest drainage was 11.4 ± 1.9 days in the DDS group and 14.2 ± 1.3 days in the ADS group, which was not significantly different (p = 0.218). Surgery, pleurodesis, and/or factor XIII administration were performed in 40 patients. Additionally, five (20.8%) patients in the DDS group and nine (18.4%) in the ADS group had a recurrence of pneumothorax within 4 weeks (p = 1.000). One patient (14%) in the DDS group and six (12.2%) in the ADS group (p = 0.414) were cured of pneumothorax but later died.

CONCLUSION:

DDS did not demonstrate a significant difference in the shortening of chest drainage duration. Further study is needed to validate the results of this study.

2023-07-01·Blood transfusion = Trasfusione del sangue

Achieving haemostasis in thrombocytopenia in remote settings: an in vitro comparison of frozen and lyophilized products.

Article

作者: Crispin, Philip J ; Gardiner, Elizabeth E ; Coupland, Lucy A

BACKGROUND:

Platelet concentrates have a limited shelf life due to room temperature storage and therefore, are not kept in regional centres where turnover is low. Cryopreserved platelets have been proposed as an alternative to platelet transfusion in austere circumstances and fibrinogen concentrate has improved thromboelastometry parameters in thrombocytopenia. This study compared the ability of stored haemostatic products and platelets to correct thromboelastometry parameters in thrombocytopenia.

MATERIALS AND METHODS:

Blood from eight patients with severe thrombocytopenia was combined with platelet concentrates, cryoprecipitate, fibrinogen concentrate, factor VIII, factor XIII and cryopreserved platelets in ratios equivalent to transfusion. Tissue factor initiated thromboelastometry (EXTEM) was compared between the products.

RESULTS:

EXTEM amplitude at 20 minutes (A20) improved by 13.1 mm with platelets (p<0.01). The 5mm increase in A20 seen with cryoprecipitate (p=0.06) was not statistically different from platelets (p=0.19). No improvement in A20 was observed with cryopreserved platelets or factor concentrates. EXTEM clotting times (CT) improved with cryopreserved platelets (19.4 s, p=0.001) and cryoprecipitate (24.1 s, p<0.05), but not fibrinogen, and both were superior to platelets (9.9 s, p<0.05). Clotting concentrates did not improve EXTEM parameters although further studies suggested the improvement in A20 was largely driven by higher fibrinogen concentrations in cryoprecipitate.

DISCUSSION:

These results suggest that cryopreserved platelets enhance clot initiation but do not contribute to clot strength in thrombocytopenia. When platelets are not available for transfusion, cryoprecipitate may be of value, however this requires further clinical studies.

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
血友病	临床阶段不明	美国	CSL Behring LLC	2000-01-01

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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)	鑰鹹齋鑰衊願觸夢襯壓(襯膚願廠襯餘鑰衊醖選) = 鹽廠觸醖網顧製鬱鏇醖簾齋憲夢範選夢鏇鑰蓋 (鹹壓衊窪觸製窪築糧願 )	-	2014-12-06
ASH2014	N/A	急性淋巴细胞白血病	-	Coagulation Factor XIII (FXIIIA-negative ALL)	鑰鹹齋鑰衊願觸夢襯壓(襯膚願廠襯餘鑰衊醖選) = 夢齋衊衊獵齋餘廠鹹餘簾齋憲夢範選夢鏇鑰蓋 (鹹壓衊窪觸製窪築糧願 )	-	2014-12-06
NCT00883090 (CTgov)	临床2期	凝血因子XIII缺乏症	15	FXIII Concentrate (Human)	夢膚衊觸鹽願製蓋獵顧(膚艱鬱簾膚鑰鬱選製獵) = 蓋醖鹽觸鹽艱簾製衊願憲憲鹽艱蓋繭鑰願簾艱 (獵襯網鹹膚範願簾糧餘, 0.20) 更多	-	2012-01-16