Pediatric Factor VII Deficiency Follow-up

  • Author: Helge Hartung, MD; Chief Editor: Max J Coppes, MD, PhD, MBA   more...
 
Updated: Mar 25, 2011
 

Further Outpatient Care

Ideally, individuals with severe factor VII (FVII) deficiency should be monitored by a comprehensive hemophilia care team that has experience in the diagnosis and management of inherited bleeding disorders.

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Deterrence/Prevention

Individuals who may require plasma-derived coagulation factor concentrates should be immunized with hepatitis A and hepatitis B vaccines.

Patients should avoid use of aspirin and other drugs, including alcohol, that affect platelet function.

Consider prophylactic therapy in patients with recurrent bleeding episodes or CNS hemorrhage.

Patients should maintain good dental hygiene to prevent dental or periodontal disease.

Individuals should participate in appropriate physical activity to maintain muscle strength, and they should wear appropriate protective gear for activities.

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Complications

Complications may include the following:

  • Recurrent bleeding, particularly hemarthroses, which can result in progressive damage to joints and chronic arthropathy. Management of recurrent hemarthroses applies the principles used in hemophilia care.
  • A risk of thrombosis can be associated with the combination of surgery and factor VII replacement therapy using prothrombin complex concentrate or high doses of factor VII concentrates.
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Patient Education

Patients and families should be given instruction and educational materials to enable them to understand FVII deficiency, to recognize the symptoms and signs of bleeding, and to identify emergency situations.

Patients should know how to contact their treatment center for immediate treatment, and they should know where to receive emergency care.

Patients should wear a MedicAlert bracelet or carry other identification showing their bleeding disorder and recommended therapy.

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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. Mandhyan R, Tiwari A, Cherian G. Congenital factor VII deficiency. Br J Anaesth. Feb 2010;104(2):267-8. [Medline].

  2. Mariani G, Herrmann FH, Dolce A, et al. Clinical phenotypes and factor VII genotype in congenital factor VII deficiency. Thromb Haemost. Mar 2005;93(3):481-7. [Medline].

  3. Peyvandi F, Mannucci PM. Rare coagulation disorders. Thromb Haemost. Oct 1999;82(4):1207-14. [Medline].

  4. Mariani G, Bernardi F. Factor VII Deficiency. Semin Thromb Hemost. Jun 2009;35(4):400-6. [Medline].

  5. Levi M, Levy JH, Andersen HF, Truloff D. Safety of recombinant activated factor VII in randomized clinical trials. N Engl J Med. Nov 4 2010;363(19):1791-800. [Medline].

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

  7. Di Paola J, Nugent D, Young G. Current therapy for rare factor deficiencies. Haemophilia. Jan 2001;7 Suppl 1:16-22. [Medline].

  8. Giansily-Blaizot M, Schved JF. Potential predictors of bleeding risk in inherited factorVII deficiency. Clinical, biological and molecular criteria. Thromb Haemost. Nov 2005;94(5):901-6. [Medline].

  9. Mariani G, Dolce A, Marchetti G, Bernardi F. Clinical picture and management of congenital factor VII deficiency. Haemophilia. Oct 2004;10 Suppl 4:180-3. [Medline].

  10. Mariani G, Lapecorella M, Dolce A. Steps towards an effective treatment strategy in congenital factor VII deficiency. Semin Hematol. Jan 2006;43(1 Suppl 1):S42-7. [Medline].

  11. Perry DJ. Factor VII Deficiency. Br J Haematol. Sep 2002;118(3):689-700. [Medline].

  12. Tuddenham EG, Pemberton S, Cooper DN. Inherited factor VII deficiency: genetics and molecular pathology. Thromb Haemost. Jul 1995;74(1):313-21. [Medline].

 
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Intrinsic and extrinsic pathways of coagulation. Factor VII/tissue factor complex activates factor IX and factor X. Factor IXa along with factor VIIIa results in formation of more factor Xa. Factor Xa along with factor Va converts prothrombin to thrombin.
 
 
 
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