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Factor XIII deficiency

MedGen UID:: 1385982
•Concept ID:: C4316906
•: Disease or Syndrome

Synonyms:	Factor XIII Deficiency; factor XIII deficiency; Factor XIII deficiency disease; Fibrin stabilizing factor deficiency; FXIIID
SNOMED CT:	Factor XIII deficiency (18604004); Factor XIII deficiency disease (18604004); Fibrin stabilizing factor deficiency (18604004)

Monarch Initiative:	MONDO:0002241

Definition

A coagulation disorder characterized by the partial or complete absence of factor XIII activity in the blood. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVFactor XIII deficiency

Phenotypic abnormality
- Abnormality of blood and blood-forming tissues
  - Abnormality of coagulation
    - Abnormality of the coagulation cascade
      - Coagulation factor deficiency syndrome
        Factor XIII deficiency
        Acquired factor XIII deficiency
        Factor XIII Inactivation
        Hereditary factor XIII deficiency disease

Professional guidelines

PubMed

Factor XIII Deficiency: A Review of Clinical Presentation and Management.

Pelcovits A, Schiffman F, Niroula R
Hematol Oncol Clin North Am 2021 Dec;35(6):1171-1180. Epub 2021 Oct 2 doi: 10.1016/j.hoc.2021.07.009. PMID: 34607717

Diagnosis, clinical manifestations and management of rare bleeding disorders in Iran.

Dorgalaleh A, Alavi SE, Tabibian S, Soori S, Moradi E, Bamedi T, Asadi M, Jalalvand M, Shamsizadeh M
Hematology 2017 May;22(4):224-230. Epub 2016 Nov 28 doi: 10.1080/10245332.2016.1263007. PMID: 27894217

Factor XIII deficiency management: a review of the literature.

Odame JE, Chan AK, Wu JK, Breakey VR
Blood Coagul Fibrinolysis 2014 Apr;25(3):199-205. doi: 10.1097/MBC.0000000000000029. PMID: 24401950

See all (27)

Recent clinical studies

Etiology

Factor XIII Deficiency: A Review of Clinical Presentation and Management.

Pelcovits A, Schiffman F, Niroula R
Hematol Oncol Clin North Am 2021 Dec;35(6):1171-1180. Epub 2021 Oct 2 doi: 10.1016/j.hoc.2021.07.009. PMID: 34607717

Valproic Acid-Induced Coagulopathy.

Kumar R, Vidaurre J, Gedela S
Pediatr Neurol 2019 Sep;98:25-30. Epub 2019 May 2 doi: 10.1016/j.pediatrneurol.2019.04.019. PMID: 31201069

Auto- and alloantibodies against factor XIII: laboratory diagnosis and clinical consequences.

Muszbek L, Pénzes K, Katona É
J Thromb Haemost 2018 May;16(5):822-832. Epub 2018 Mar 23 doi: 10.1111/jth.13982. PMID: 29460500

Biology of Factor XIII and clinical manifestations of Factor XIII deficiency.

Levy JH, Greenberg C
Transfusion 2013 May;53(5):1120-31. Epub 2012 Aug 28 doi: 10.1111/j.1537-2995.2012.03865.x. PMID: 22928875

Factor XIII deficiency.

Hsieh L, Nugent D
Haemophilia 2008 Nov;14(6):1190-200. doi: 10.1111/j.1365-2516.2008.01857.x. PMID: 19141159

See all (151)

Diagnosis

Factor XIII and surgical bleeding.

Guilabert P, Asmis L, Cortina V, Barret JP, Colomina MJ
Minerva Anestesiol 2022 Mar;88(3):156-165. Epub 2022 Jan 24 doi: 10.23736/S0375-9393.22.15772-X. PMID: 35072429

Acquired factor XIII deficiency: A review.

Yan MTS, Rydz N, Goodyear D, Sholzberg M
Transfus Apher Sci 2018 Dec;57(6):724-730. Epub 2018 Oct 30 doi: 10.1016/j.transci.2018.10.013. PMID: 30446212

Factor XIII deficiency diagnosis: Challenges and tools.

Karimi M, Peyvandi F, Naderi M, Shapiro A
Int J Lab Hematol 2018 Feb;40(1):3-11. Epub 2017 Oct 13 doi: 10.1111/ijlh.12756. PMID: 29027765

Diagnosis of factor XIII deficiency.

Dorgalaleh A, Tabibian S, Hosseini MS, Farshi Y, Roshanzamir F, Naderi M, Kazemi A, Zaker F, Aghideh AN, Shamsizadeh M
Hematology 2016 Aug;21(7):430-9. Epub 2016 Mar 30 doi: 10.1080/10245332.2015.1101975. PMID: 27077776

Factor XIII Deficiency.

Karimi M, Bereczky Z, Cohan N, Muszbek L
Semin Thromb Hemost 2009 Jun;35(4):426-38. Epub 2009 Jul 13 doi: 10.1055/s-0029-1225765. PMID: 19598071

See all (264)

Therapy

Factor XIII and surgical bleeding.

Guilabert P, Asmis L, Cortina V, Barret JP, Colomina MJ
Minerva Anestesiol 2022 Mar;88(3):156-165. Epub 2022 Jan 24 doi: 10.23736/S0375-9393.22.15772-X. PMID: 35072429

Valproic Acid-Induced Coagulopathy.

Kumar R, Vidaurre J, Gedela S
Pediatr Neurol 2019 Sep;98:25-30. Epub 2019 May 2 doi: 10.1016/j.pediatrneurol.2019.04.019. PMID: 31201069

Factor XIII deficiency: an update.

Schroeder V, Kohler HP
Semin Thromb Hemost 2013 Sep;39(6):632-41. Epub 2013 Aug 8 doi: 10.1055/s-0033-1353392. PMID: 23929307

Factor XIII Deficiency.

Karimi M, Bereczky Z, Cohan N, Muszbek L
Semin Thromb Hemost 2009 Jun;35(4):426-38. Epub 2009 Jul 13 doi: 10.1055/s-0029-1225765. PMID: 19598071

Factor XIII deficiency.

Hsieh L, Nugent D
Haemophilia 2008 Nov;14(6):1190-200. doi: 10.1111/j.1365-2516.2008.01857.x. PMID: 19141159

See all (164)

Prognosis

Auto- and alloantibodies against factor XIII: laboratory diagnosis and clinical consequences.

Muszbek L, Pénzes K, Katona É
J Thromb Haemost 2018 May;16(5):822-832. Epub 2018 Mar 23 doi: 10.1111/jth.13982. PMID: 29460500

Factor XIII deficiency in south of Tunisia.

Maaloul I, Medhaffer M, Louhichi N, Krichen I, Alibi S, Kmiha S, Aloulou H, Fakhfakh F, Elloumi M, Kallel C, Hachicha M
Blood Coagul Fibrinolysis 2017 Sep;28(6):485-489. doi: 10.1097/MBC.0000000000000649. PMID: 28704210

Autoimmune acquired factor XIII deficiency due to anti-factor XIII/13 antibodies: A summary of 93 patients.

Ichinose A; Japanese Collaborative Research Group on AH13
Blood Rev 2017 Jan;31(1):37-45. Epub 2016 Aug 11 doi: 10.1016/j.blre.2016.08.002. PMID: 27542511

Factor XIII deficiency.

Hsieh L, Nugent D
Haemophilia 2008 Nov;14(6):1190-200. doi: 10.1111/j.1365-2516.2008.01857.x. PMID: 19141159

Factor XIII: inherited and acquired deficiency.

Board PG, Losowsky MS, Miloszewski KJ
Blood Rev 1993 Dec;7(4):229-42. doi: 10.1016/0268-960x(93)90010-2. PMID: 8130686

See all (81)

Clinical prediction guides

Auto- and alloantibodies against factor XIII: laboratory diagnosis and clinical consequences.

Muszbek L, Pénzes K, Katona É
J Thromb Haemost 2018 May;16(5):822-832. Epub 2018 Mar 23 doi: 10.1111/jth.13982. PMID: 29460500

Blood coagulation factor XIII and factor XIII deficiency.

Dorgalaleh A, Rashidpanah J
Blood Rev 2016 Nov;30(6):461-475. Epub 2016 Jun 16 doi: 10.1016/j.blre.2016.06.002. PMID: 27344554

Diagnosis of factor XIII deficiency.

Biology of Factor XIII and clinical manifestations of Factor XIII deficiency.

Levy JH, Greenberg C
Transfusion 2013 May;53(5):1120-31. Epub 2012 Aug 28 doi: 10.1111/j.1537-2995.2012.03865.x. PMID: 22928875

Hemorrhagic acquired factor XIII (13) deficiency and acquired hemorrhaphilia 13 revisited.

Ichinose A
Semin Thromb Hemost 2011 Jun;37(4):382-8. Epub 2011 Jul 30 doi: 10.1055/s-0031-1276587. PMID: 21805444

See all (112)

Recent systematic reviews

Acquired Factor XIII Inhibitor in Hospitalized and Perioperative Patients: A Systematic Review of Case Reports and Case Series.

Tone KJ, James TE, Fergusson DA, Tinmouth A, Tay J, Avey MT, Kilty S, Lalu MM
Transfus Med Rev 2016 Jul;30(3):123-31. Epub 2016 Apr 22 doi: 10.1016/j.tmrv.2016.04.001. PMID: 27167905

Molecular pathology of rare bleeding disorders (RBDs) in India: a systematic review.

Kulkarni BP, Nair SB, Vijapurkar M, Mota L, Shanbhag S, Ali S, Shetty SD, Ghosh K
PLoS One 2014;9(9):e108683. Epub 2014 Oct 2 doi: 10.1371/journal.pone.0108683. PMID: 25275492 Free PMC Article

Congenital factor XIII deficiency in women: a systematic review of literature.

Sharief LA, Kadir RA
Haemophilia 2013 Nov;19(6):e349-57. Epub 2013 Aug 28 doi: 10.1111/hae.12259. PMID: 23992439

Acquired FXIII inhibitors: a systematic review.

Franchini M, Frattini F, Crestani S, Bonfanti C
J Thromb Thrombolysis 2013 Jul;36(1):109-14. doi: 10.1007/s11239-012-0818-3. PMID: 23065324

Bleeding risk and reproductive capacity among patients with factor XIII deficiency: a case presentation and review of the literature.

Burrows RF, Ray JG, Burrows EA
Obstet Gynecol Surv 2000 Feb;55(2):103-8. doi: 10.1097/00006254-200002000-00024. PMID: 10674253

See all (5)

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Factor XIII deficiency

Definition

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Recent systematic reviews

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Practice guidelines

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