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Arthropathy and surgery in congenital factor VII deficiency

  • Timothy Kuzel
    Affiliations
    Departments of Medicine and Orthopedic Surgery, Northwestern Memorial Hospital, Chicago, Illinois, USA

    Atherosclerosis Program, Rehabilitation Institute and Northwestern University Medical School, Chicago, Illinois, USA

    Department of Medicine, University of Chicago, Chicago, Illinois, USA
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  • David Green
    Correspondence
    Requests for reprints should be addressed to Dr. David Green, Atherosclerosis Program, Northwestern University, 345 East Superior Street—1407 RIC, Chicago, Illinois 60611.
    Affiliations
    Departments of Medicine and Orthopedic Surgery, Northwestern Memorial Hospital, Chicago, Illinois, USA

    Atherosclerosis Program, Rehabilitation Institute and Northwestern University Medical School, Chicago, Illinois, USA

    Department of Medicine, University of Chicago, Chicago, Illinois, USA
    Search for articles by this author
  • S.David Stulberg
    Affiliations
    Departments of Medicine and Orthopedic Surgery, Northwestern Memorial Hospital, Chicago, Illinois, USA

    Atherosclerosis Program, Rehabilitation Institute and Northwestern University Medical School, Chicago, Illinois, USA

    Department of Medicine, University of Chicago, Chicago, Illinois, USA
    Search for articles by this author
  • Joseph Baron
    Affiliations
    Departments of Medicine and Orthopedic Surgery, Northwestern Memorial Hospital, Chicago, Illinois, USA

    Atherosclerosis Program, Rehabilitation Institute and Northwestern University Medical School, Chicago, Illinois, USA

    Department of Medicine, University of Chicago, Chicago, Illinois, USA
    Search for articles by this author
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      Abstract

      A 35-year-old woman with severe (less than 1 percent) factor VII deficiency had recurrent hemarthroses involving the left knee, leading to deformity, pain, and virtually complete loss of function. It was elected to perform a total knee replacement. In preparation for surgery, the patient received heat-treated prothrombin complex concentrate containing 870 units of factor VII per vial. A dose of 50 U/kg raised the factor VII level to 115 percent. At surgery, dense adhesions were found within the joint, the articular cartilage was overgrown with pannus extending out to the lateral patella, and there was extensive deformity of the femoral condyle and tibial plateau. The joint was excised and replaced by a cemented Microloc prosthesis. Postoperatively, factor VII levels were maintained above 10 percent by six-hourly infusions of concentrate. Beginning on Day 4, single daily infusions of 25 U/kg were given prior to physical therapy. No bleeding occurred, and the patient was ambulating at the time of discharge 20 days postoperatively. This experience indicates that despite its short half-life (less than four hours), factor VII levels sufficient to prevent bleeding can be maintained in factor VII-deficient patients undergoing major operative procedures.
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