Pediatric Factor VII Deficiency Follow-up
- Author: Helge Dirk Hartung, MD; Chief Editor: Max J Coppes, MD, PhD, MBA more...
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.
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.
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.
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.
Mandhyan R, Tiwari A, Cherian G. Congenital factor VII deficiency. Br J Anaesth. 2010 Feb. 104(2):267-8. [Medline].
Acharya SS. Rare bleeding disorders in children: identification and primary care management. Pediatrics. 2013 Nov. 132 (5):882-92. [Medline].
Tuddenham EG, Pemberton S, Cooper DN. Inherited factor VII deficiency: genetics and molecular pathology. Thromb Haemost. 1995 Jul. 74(1):313-21. [Medline].
Mariani G, Herrmann FH, Dolce A, et al. Clinical phenotypes and factor VII genotype in congenital factor VII deficiency. Thromb Haemost. 2005 Mar. 93(3):481-7. [Medline].
Peyvandi F, Mannucci PM. Rare coagulation disorders. Thromb Haemost. 1999 Oct. 82(4):1207-14. [Medline].
Mariani G, Bernardi F. Factor VII Deficiency. Semin Thromb Hemost. 2009 Jun. 35(4):400-6. [Medline].
Levi M, Levy JH, Andersen HF, Truloff D. Safety of recombinant activated factor VII in randomized clinical trials. N Engl J Med. 2010 Nov 4. 363(19):1791-800. [Medline].
Todd T, J Perry D. A review of long-term prophylaxis in the rare inherited coagulation factor deficiencies. Haemophilia. 2009 Nov 11. [Medline].
Kim SH, Park YS, Kwon KH, Lee JH, Kim KC, Yoo MC. Surgery in patients with congenital factor VII deficiency: A single center experience. Korean J Hematol. 2012 Dec. 47(4):281-5. [Medline]. [Full Text].
Di Paola J, Nugent D, Young G. Current therapy for rare factor deficiencies. Haemophilia. 2001 Jan. 7 Suppl 1:16-22. [Medline].
Giansily-Blaizot M, Schved JF. Potential predictors of bleeding risk in inherited factorVII deficiency. Clinical, biological and molecular criteria. Thromb Haemost. 2005 Nov. 94(5):901-6. [Medline].
Mariani G, Dolce A, Marchetti G, Bernardi F. Clinical picture and management of congenital factor VII deficiency. Haemophilia. 2004 Oct. 10 Suppl 4:180-3. [Medline].
Mariani G, Lapecorella M, Dolce A. Steps towards an effective treatment strategy in congenital factor VII deficiency. Semin Hematol. 2006 Jan. 43(1 Suppl 1):S42-7. [Medline].
Perry DJ. Factor VII Deficiency. Br J Haematol. 2002 Sep. 118(3):689-700. [Medline].