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Pediatric Factor XIII Deficiency Differential Diagnoses

  • Author: Helge Dirk Hartung, MD; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
 
Updated: Jun 06, 2016
 
 

Diagnostic Considerations

Acquired factor XIII (FXIII) deficiency can be caused by liver disease, inflammatory bowel disease, and disseminated intravascular coagulation, although controversy surrounds whether the low plasma levels in these conditions actually contribute to clinical bleeding. The development of autoantibodies to factor XIII has been reported. Consider other congenital coagulation factor deficiencies, most notably afibrinogenemia.

 
 
Contributor Information and Disclosures
Author

Helge Dirk Hartung, MD Attending Physician, Division of Hematology, The Children's Hospital of Philadelphia

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

James L Harper, MD Associate Professor, Department of Pediatrics, Division of Hematology/Oncology and Bone Marrow Transplantation, Associate Chairman for Education, Department of Pediatrics, University of Nebraska Medical Center; Associate Clinical Professor, Department of Pediatrics, Creighton University School of Medicine; Director, Continuing Medical Education, Children's Memorial Hospital; Pediatric Director, Nebraska Regional Hemophilia Treatment Center

James L Harper, MD is a member of the following medical societies: American Society of Pediatric Hematology/Oncology, American Federation for Clinical Research, Council on Medical Student Education in Pediatrics, Hemophilia and Thrombosis Research Society, American Academy of Pediatrics, American Association for Cancer Research, American Society of Hematology

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA Executive Vice President, Chief Medical and Academic Officer, Renown Heath

Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American College of Healthcare Executives, American Society of Pediatric Hematology/Oncology, Society for Pediatric Research

Disclosure: Nothing to disclose.

Acknowledgements

Gary R Jones, MD Associate Medical Director, Clinical Development, Berlex Laboratories

Gary R Jones, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

References
  1. Dickneite G, Herwald H, Korte W, Allanore Y, Denton CP, Matucci Cerinic M. Coagulation factor XIII: a multifunctional transglutaminase with clinical potential in a range of conditions. Thromb Haemost. 2015 Apr. 113 (4):686-97. [Medline]. [Full Text].

  2. Karimi M, Bereczky Z, Cohan N, Muszbek L. Factor XIII Deficiency. Semin Thromb Hemost. 2009 Jun. 35(4):426-38. [Medline].

  3. Naderi M, Alizadeh S, Kazemi A, et al. Central nervous system bleeding in pediatric patients with factor XIII deficiency: a study on 23 new cases. Hematology. 2015 Mar. 20 (2):112-8. [Medline].

  4. Anwar R, Minford A, Gallivan L, Trinh CH, Markham AF. Delayed umbilical bleeding--a presenting feature for factor XIII deficiency: clinical features, genetics, and management. Pediatrics. 2002 Feb. 109(2):E32. [Medline].

  5. Inbal A, Oldenburg J, Carcao M, Rosholm A, Tehranchi R, Nugent D. Recombinant factor XIII: a safe and novel treatment for congenital factor XIII deficiency. Blood. 2012 May 31. 119(22):5111-7. [Medline].

  6. Fadoo Z, Merchant Q, Rehman KA. New developments in the management of congenital Factor XIII deficiency. J Blood Med. 2013. 4:65-73. [Medline]. [Full Text].

  7. Naderi M, Eshghi P, Cohan N, Miri-Moghaddam E, Yaghmaee M, Karimi M. Successful delivery in patients with FXIII deficiency receiving prophylaxis: report of 17 cases in Iran. Haemophilia. 2012 Sep. 18(5):773-6. [Medline].

  8. Todd T, J Perry D. A review of long-term prophylaxis in the rare inherited coagulation factor deficiencies. Haemophilia. 2009 Nov 11. [Medline].

  9. Anwar R, Miloszewski KJ. Factor XIII deficiency. Br J Haematol. 1999 Dec. 107(3):468-84. [Medline].

  10. Ariens RA, Lai TS, Weisel JW, Greenberg CS, Grant PJ. Role of factor XIII in fibrin clot formation and effects of genetic polymorphisms. Blood. 2002 Aug 1. 100(3):743-54. [Medline].

  11. Ichinose A. Physiopathology and regulation of factor XIII. Thromb Haemost. 2001 Jul. 86(1):57-65. [Medline].

  12. Ichinose A, Asahina T, Kobayashi T. Congenital blood coagulation factor XIII deficiency and perinatal management. Curr Drug Targ. 2005. 6:541-549.

  13. Israels LG, Israels ED. Fibrinogen, factor XIII, and fibrinolysis. Mechanisms in Hematology. 3rd ed. Concord ON: Core Health Services, Inc; 2002. 355-367.

  14. Jennings I, Kitchen S, Woods TA, Preston FE,. Problems relating to the laboratory diagnosis of factor XIII deficiency: a UK NEQAS study. J Thromb Haemost. 2003 Dec. 1(12):2603-8. [Medline].

  15. Lovejoy AE, Reynolds TC, Visich JE, Butine MD, Young G, Belvedere MA, et al. Safety and pharmacokinetics of recombinant factor XIII-A2 administration in patients with congenital factor XIII deficiency. Blood. 2006 Jul 1. 108(1):57-62. [Medline].

  16. Nugent DJ. Prophylaxis in rare coagulation disorders -- factor XIII deficiency. Thromb Res. 2006. 118 Suppl 1:S23-8. [Medline].

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Activation of factor XIII (FXIII) by thrombin and calcium is a 2-step process. Thrombin cleaves an arginine-lysine bond in the A subunit and calcium causes dissociation of the B subunit, exposing the active site on the A subunit (XIIIa).
 
 
 
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