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Last update 23 Jan 2025

Factor XIII Deficiency

Last update 23 Jan 2025

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

692

Literatures (Medical) associated with Factor XIII Deficiency

01 Dec 2024·Clinical Case Reports

Factor XIII Deficiency Presenting With Duodenal Hematoma in a 3‐Year‐Old Female: A Comprehensive Radiological Assessment

Article

Author: Ullah, Muhammad Khubaib ; Khan, Faheemullah ; Nadeem, Naila ; Aman, Muhammad ; Irfan, Muhammad ; Zafar, Uffan ; Nadeem, Arsalan ; Khandwala, Kumail ; Khan, Noman ; Ahmad, Izaz

ABSTRACTIn the pediatric age group, FXIII deficiency can present as abnormal or delayed bleeding from the umbilical stump, soft tissue and subcutaneous bleeding, intracerebral hemorrhages, intraoral bleeding, and poor wound healing. This case report describes an atypical presentation of FXIII deficiency in a 3‐year‐old female who presented to the emergency department with complaints of abdominal pain and vomiting. She was managed conservatively under the care of the pediatric surgery team.

20 Nov 2024·Cureus

Bilateral Compartment Syndrome Affecting the Upper Limbs in a Patient With Chronic Myeloid Leukemia

Article

Author: Edmunds, Christopher T ; Varadhan, Abhinav ; Bagavandoss, Aswinishree ; Oyibo, Samson O

A 70-year-old male patient who had recently been recently diagnosed with chromic myeloid leukemia (CML) presented to the emergency department with bilateral upper limb pain and swelling. Examination of his upper limbs revealed features suggestive of compartment syndrome. Blood investigations demonstrated anaemia, rhabdomyolysis and renal function impairment. Because of the rapid fall in hemoglobin levels, he required clotting factors and blood product resuscitation. Compartment pressure measurements were elevated, confirming the diagnosis of bilateral acute compartment syndrome secondary to bleeding. Initial investigations demonstrated low factor XIII levels. Once stabilized, he underwent bilateral fasciotomies but required intensive care management post-surgery, including ongoing blood product resuscitation and renal replacement therapy. Levels of factor XIII continued to fall. Although there have been reported cases of acute leukaemia involving factor XIII deficiency, reports of chronic leukaemia involving factor XIII deficiency are rare. This case provides key learning of a known complication of acute leukaemia and highlights a rare surgical complication of factor XIII deficiency.

03 Nov 2024·Cureus

A Rare Case of Congenital Factor XIII Deficiency Secondary to Hyperconsumption: A Case Report

Article

Author: S, Magesh Kumar ; Koduri, Amukthamalyada ; Kakumani, Jagadeswar

Acquired Factor XIII (FXIII) deficiency is a rare condition often associated with underlying medical conditions or medications. We present a case of a 23-year-old male, who presented with prolonged bleeding from a traumatic ulcer site on his left leg. Initial laboratory investigations revealed a severe deficiency in FXIII activity (30%) and antigen levels (25%), with no evidence of congenital disorders or other underlying pathologies. Further evaluation indicated a pattern of hyperconsumption due to ongoing bleeding episodes in the form of excessive use of clotting factors due to repeated bleeding episodes and impaired clot stability, leading to a coagulopathy where factors are consumed faster than they can be produced. The patient was treated with six units of cryoprecipitate to address the bleeding and was started on high-end antibiotics, including meropenem and vancomycin, to manage a suspected septic process. This comprehensive approach led to significant clinical improvement, with the resolution of symptoms and stabilization of the ulcer site. This case highlights the importance of considering acquired FXIII deficiency in patients with unexplained bleeding diathesis, especially in the context of alcohol abuse or conditions associated with hyperconsumption. Early recognition and immediate treatment are crucial for improving prognostic outcomes for these patients.

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Factor XIII Deficiency

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