Last update 01 Nov 2024

Factor XIII Deficiency

Last update 01 Nov 2024

Basic Info

Synonyms

Congenital factor XIII deficiency, Deficiencies, Factor 13, Deficiencies, Factor Thirteen

+ [49]

Introduction

A deficiency of blood coagulation FACTOR XIII or fibrin stabilizing factor (FSF) that prevents blood clot formation and results in a clinical hemorrhagic diathesis.

Drugs associated with Factor XIII Deficiency

Catridecacog

Target

fibrin

Mechanism

fibrin modulators [+1]

Active Org.

Novo Nordisk A/S [+5]

Originator Org.

Novo Nordisk A/S

Active Indication

Factor XIII Deficiency [+2]

Inactive Indication

Colitis, Ulcerative [+3]

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

EU [+3]

First Approval Date03 Sep 2012

Factor XIII(CSL Behring LLC)

Target

Fibrinogen

Mechanism

Factor XIII replacements

Active Org.

CSL Behring GmbH [+1]

Originator Org.

CSL Behring LLC

Active Indication

Postoperative Hemorrhage [+2]

Inactive Indication

Scleroderma, Systemic

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

First Approval Date01 Sep 1994

Freeze-dried Concentrated Human Blood Coagulation Factor IX(Japan Blood Products Organization)

Target-

Mechanism-

Active Org.

The Japan Blood Products Organization

Originator Org.

The Japan Blood Products Organization

Active Indication

Factor XIII Deficiency

Inactive Indication-

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

First Approval Date03 Mar 1993

Clinical Trials associated with Factor XIII Deficiency

IRCT20240130060862N1 / RecruitingNot ApplicableIIT

The effect of myofascial release and joint mobilization based on diagnostic sub group, on headache index, rang of motion, thickness of upper cervical muscles and neck disability index in cervicogenic headache subjects

Start Date30 May 2024

Sponsor / Collaborator

Iran University of Medical Sciences & Health Services

IRCT20220912055950N1 / RecruitingNot ApplicableIIT

The effect of educational program on the knowledge and care performance of parents of children with congenital heart disorders

Start Date02 Oct 2022

Sponsor / Collaborator-

DRKS00028547 / CompletedNot ApplicableIIT

Coagulation and wound healing of open vs. minimally invasive esophageal and pancreatic surgery - focus on factor XIII - OPEN-MIC-XIII Trial

Start Date01 Sep 2022

Sponsor / Collaborator-

100 Clinical Results associated with Factor XIII Deficiency

100 Translational Medicine associated with Factor XIII Deficiency

0 Patents (Medical) associated with Factor XIII Deficiency

687

Literatures (Medical) associated with Factor XIII Deficiency

01 Nov 2024·Child Abuse & Neglect

Corrigendum to “Traumatic intracranial hemorrhage in pediatrics: Implications of factor XIII deficiency and consumptive coagulopathy in abusive head trauma evaluation” [Child Abuse & Neglect 149 (2024) 106651]

Author: Cohen, Clay T ; Lam, Fong Wilson ; Small, Amanda ; Bachim, Angela N ; López, Arianexys Aquino

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 Aug 2024·Research and Practice in Thrombosis and Haemostasis

Letter in response to Bounaix et al. “Management of anticoagulation and factor XIII replacement in a patient with severe factor XIII deficiency and recurrent venous thromboembolic disease: case report and review of literature”

Article

Author: Croles, F Nanne ; Schutgens, Roger E.G. ; Schutgens, Roger E G ; van Vulpen, Lize F.D. ; Croles, F. Nanne ; van Vulpen, Lize F D

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Factor XIII Deficiency

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