Pediatric Factor XIII Deficiency Follow-up

  • Author: Helge Hartung, MD; Chief Editor: Max J Coppes, MD, PhD, MBA   more...
 
Updated: Mar 9, 2011
 

Further Outpatient Care

Ideally, a comprehensive hemophilia care team with experience in the diagnosis and management of inherited bleeding disorders should monitor individuals with severe factor XIII (FXIII) deficiency.

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Deterrence/Prevention

For patients with homozygous factor XIII deficiency, prophylaxis is the best approach.[3]

  • Individuals who require plasma-derived factor XIII concentrate should be immunized with hepatitis A and hepatitis B vaccines.
  • Aspirin and other drugs that impair platelet function should be avoided.
  • Women at risk for spontaneous abortion should be evaluated for the need of enhanced prophylaxis starting in early pregnancy.
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Patient Education

Provide patients and families with individual instruction and educational materials so they can understand factor XIII deficiency, recognize the symptoms and signs of bleeding, and identify emergency situations.

  • Patients should know where to receive emergency care and how to contact their treatment center for immediate treatment.
  • Patients should wear a MedicAlert bracelet or carry other identification stating their bleeding disorder and recommended therapy.

For excellent patient education resources, visit eMedicine's Teeth and Mouth Center. Also, see eMedicine's patient education article Teething.

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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

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

  2. 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].

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

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

  5. 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].

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

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

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

  9. 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].

  10. 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].

  11. 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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