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

非在研适应症

原研机构

在研机构

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

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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148

项与凝血因子XIII 相关的文献（医药）

2026-02-01·Clinical Case Reports

A Case of Factor XIII Deficiency Identified by Recurrent Postoperative Bleeding After Tonsillectomy

Article

作者: Ai Yamamoto ; Koichiro Wasano ; Akihiro Sakai ; Takanobu Teramura ; Masashi Hamada ; Hikaru Yamamoto ; Kenji Okami

ABSTRACT:

Postoperative bleeding is a well‐known complication of tonsillectomy. Although inadequate hemostasis and vascular injury are common causes, occult coagulation disorders may also contribute. Factor XIII deficiency is an extremely rare condition that is particularly difficult to detect preoperatively because PT and APTT typically remain normal. A 27‐year‐old man underwent bilateral tonsillectomy for a recurrent peritonsillar abscess. Despite normal coagulation screening, the patient developed repeated postoperative hemorrhages requiring surgical management. Further evaluation revealed reduced Factor XIII activity (36%). After Factor XIII concentrate administration, bleeding ceased, and the postoperative course stabilized. Factor XIII deficiency should be considered in patients with recurrent postoperative bleeding despite normal routine coagulation tests. Early recognition and timely replacement therapy may prevent severe complications.

2025-10-21·Cureus Journal of Medical Science

Efficacy of Thoracic Duct Embolization in Managing Severe Refractory Chyle Leak Post-cervical Reconstruction in a Patient With Liver Cirrhosis: A Case Report

Article

作者: Nagaoka, Masato ; Kojima, Hiromi ; Ashida, Hirokazu ; Fukuda, Nobuki ; Ishida, Katsuhiro

This case report describes a patient with a history of liver cirrhosis who underwent neck reconstruction surgery and subsequently experienced substantial chyle leakage, reaching volumes of up to 6,620 mL per day. Conservative interventions such as fasting, a low-fat diet, total parenteral nutrition, drainage management, somatostatin analogs, and factor XIII supplementation proved ineffective, and the patient exhibited a refractory treatment course. The pronounced chyle leakage in this instance was hypothesized to be linked to increased lymph production resulting from portal hypertension associated with liver cirrhosis. Given the resistance to conservative treatment, thoracic duct embolization was performed by the interventional radiology department, effectively controlling the chyle leakage. The patient experienced a favorable clinical trajectory, devoid of complications. These findings indicate that thoracic duct embolization is a safe and efficacious minimally invasive treatment for refractory and massive chyle leakage following neck dissection and may represent a promising alternative before surgical intervention.

2025-01-01·[Rinsho ketsueki] The Japanese journal of clinical hematology

[Disseminated intravascular coagulation associated with vascular abnormalities].

Review

作者: Asakura, Hidesaku ; Yamada, Shinya

This paper discusses the diagnostic findings and treatment options for DIC associated with vascular abnormalities based on the "Clinical practice guidelines for management of disseminated intravascular coagulation (DIC) in Japan 2024" released in early 2025. The guidelines define vascular abnormalities as aortic aneurysm, aortic dissection, vasculitis syndromes, and vascular malformations. DIC with enhanced fibrinolysis is a type of DIC often observed in association with vascular abnormalities. Key diagnostic tests for DIC include coagulation activation markers such as thrombin-antithrombin complexes and fibrinolysis activation markers such as plasmin-α₂ plasmin inhibitor complex. Treatment consists of addressing the underlying disease, anticoagulant therapy, and combination therapy with anticoagulants and antifibrinolytics, or potentially observation, depending on bleeding symptoms and test results. Use of Factor XIII concentrates may also be considered. Treatment selection should consider the patient's condition and the treatment duration, with ongoing adjustments based on continuous testing. A well-established testing system and collaboration with experts are crucial in the treatment of DIC associated with vascular abnormalities.

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


NCT00883090 (Pubmed \| Haemophilia)	临床2期	凝血因子XIII缺乏症	15	FXIII concentrate (human) 40 IU kg(-1)	蓋餘膚鹽淵廠願範蓋網(選襯壓糧鹽繭鹽鹽齋蓋) = Two patients had possibly treatment-related adverse events 鬱製齋遞鬱製夢廠構壓 (廠餘衊淵醖齋廠衊壓窪 )	-	2015-01-01
NCT00883090 (CTgov)	临床2期	凝血因子XIII缺乏症	15	FXIII Concentrate (Human)	製糧觸壓獵觸鏇簾夢醖(鹽獵顧網鑰艱願網鬱簾) = 醖築鬱構鹽繭鹹廠鹹蓋淵淵遞製築壓淵膚廠艱 (選淵淵醖願選網淵積淵, 0.20) 更多	-	2012-01-16
Phase III study (ASH2006)	临床3期	-	61	Fibrogammin P	蓋餘網憲醖餘獵膚壓鹽(淵範鑰齋壓鑰壓艱膚淵) = 顧衊鹹鹹廠憲窪鏇積餘衊築膚膚製憲壓憲製醖 (顧獵艱鹹獵襯淵夢網鹽 )	-	2006-11-16