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    Factor VII deficiency

    Definition

    Factor VII deficiency is an inherited disorder that causes abnormal blood clotting (coagulation), resulting from a deficiency of the plasma protein factor VII.

    Alternative Names

    Extrinsic factor deficiency

    Causes

    This disorder is caused by an inherited deficiency of factor VII, an important clotting protein. Normal blood coagulation is a complex process involving as many as 20 different plasma proteins, which are known as blood coagulation factors. A series of complex chemical reactions involving these coagulation factors takes place very rapidly, forming an insoluble protein called fibrin that stops bleeding.

    When certain coagulation factors are deficient or missing, the chain reaction does not take place normally. In this disorder, bleeding can vary from mild to severe within the same person over time. A history of bleeding may occur in infancy or childhood. Gastrointestinal and central nervous system bleeding can occur.

    The risk factor is a family history of bleeding. The incidence is 1 in 500,000.

    Symptoms

    Exams and Tests

    Treatment

    Bleeding episodes can be controlled with normal plasma, concentrates containing factor VII, or recombinant factor VII. During bleeding episodes, frequent treatment is needed because the life span of factor VII is short. Menstrual bleeding can be controlled by the use of oral contraceptives.

    Support Groups

    The stress of illness can often be helped by joining a support group where members share common experiences and problems. See hemophilia - resources.

    Outlook (Prognosis)

    The probable outcome is good with proper treatment.

    Possible Complications

    • Hemorrhage
    • Stroke or other neurological problems related to central nervous system bleeding
    • Joint deformities may develop in severe cases due to recurrent bleeds

    When to Contact a Medical Professional

    Go to the emergency room or call the local emergency number (such as 911) if you have severe, unexplained bleeding.

    Prevention

    This is an inherited disorder. There is no known prevention.


    Review Date: 4/15/2005
    Reviewed By: Rita Nanda, MD, Department of Medicine, Section of Hematology/Oncology, University of Chicago Medical Center, Chicago, IL. Review provided by VeriMed Healthcare Network.
    The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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