Pediatric Factor XIII Deficiency Medication

Author: Helge Hartung, MD; Chief Editor: Max J Coppes, MD, PhD, MBA more...

Updated: Mar 9, 2011

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Medication Summary
Clotting factors
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Medication Summary

Scheduled factor XIII (FXIII) replacement every 4-6 weeks maintains factor XIII levels above the critical threshold for spontaneous bleeding and allows patients to participate in regular activities.

Class Summary

Hemostasis is the physiological response to bleeding. Injury to the blood vessel wall and factors released by platelets initiate the coagulation cascade. Formation of an insoluble fibrin clot, which reinforces the initial platelet plug, is mediated by blood clotting factors. Clotting factors function as cofactors in the blood-coagulation cascade.

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Factor XIII concentrate (Corifact)

Orphan drug. FXIII is a proenzyme that is activated, in the presence of calcium ion, by thrombin cleavage of the A-subunit to become activated FXIII (FXIIIa). Promotes cross-linking of fibrin during coagulation and is essential to the physiological protection of the clot against fibrinolysis.

Indicated for routine prophylactic treatment of congenital factor XIII (FXIII) deficiency.

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Contributor Information and Disclosures

Author

Helge Hartung, MD Attending Physician, Division of Hematology, Center for Cancer and Blood Disorders

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

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; Assistant Clinical Professor, Department of Pediatrics, Creighton University; 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 Academy of Pediatrics, American Association for Cancer Research, American Federation for Clinical Research, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Council on Medical Student Education in Pediatrics, and Hemophilia and Thrombosis Research Society

Disclosure: Nothing to disclose.

Helen SL Chan, MBBS, FRCP(C), FAAP Senior Scientist, Research Institute; Professor, Division of Hematology/Oncology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Canada

Helen SL Chan, MBBS, FRCP(C), FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Society of Hematology, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA Senior Vice President, Center for Cancer and Blood Disorders, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University School of Medicine; Clinical Professor of Pediatrics, George Washington University School of Medicine and Health Sciences

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

Disclosure: Nothing to disclose.

References

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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. Feb 2002;109(2):E32. [Medline].
Todd T, J Perry D. A review of long-term prophylaxis in the rare inherited coagulation factor deficiencies. Haemophilia. Nov 11 2009;[Medline].
Anwar R, Miloszewski KJ. Factor XIII deficiency. Br J Haematol. Dec 1999;107(3):468-84. [Medline].
Ariens RA, Lai TS, Weisel JW, Greenberg CS, Grant PJ. Role of factor XIII in fibrin clot formation and effects of genetic polymorphisms. Blood. Aug 1 2002;100(3):743-54. [Medline].
Ichinose A. Physiopathology and regulation of factor XIII. Thromb Haemost. Jul 2001;86(1):57-65. [Medline].
Ichinose A, Asahina T, Kobayashi T. Congenital blood coagulation factor XIII deficiency and perinatal management. Curr Drug Targ. 2005;6:541-549.
Israels LG, Israels ED. Fibrinogen, factor XIII, and fibrinolysis. In: Mechanisms in Hematology. 3^rd ed. Concord ON: Core Health Services, Inc; 2002:355-367.
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. Dec 2003;1(12):2603-8. [Medline].
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. Jul 1 2006;108(1):57-62. [Medline].
Nugent DJ. Prophylaxis in rare coagulation disorders -- factor XIII deficiency. Thromb Res. 2006;118 Suppl 1:S23-8. [Medline].

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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