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Медицина

 Factor VII deficiency

 

  • Illustrations
  • Alternative names
  • Definition
  • Causes, incidence, and risk factors
  • Symptoms
  • Signs and tests
  • Treatment
  • Support Groups
  • Expectations (prognosis)
  • Complications
  • Calling your health care provider
  • Prevention
Illustrations Blood clot formation Blood clots

Alternative names    Return to top

Extrinsic factor deficiency

Definition    Return to top

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

Causes, incidence, and risk factors    Return to top

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 reactionsВ 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    Return to top

  • Bleeding of mucous membranes
  • Nosebleeds
  • Excessive bruising
  • Excessive menstrual bleeding
  • Bleeding into muscles
  • Bleeding into joints

Signs and tests    Return to top

  • Prolonged prothrombin time
  • Normal partial thromboplastin time
  • Decreased plasma factor VII activity

Treatment    Return to top

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    Return to top

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

Expectations (prognosis)    Return to top

The probable outcome is good with proper treatment.

Complications    Return to top

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

Calling your health care provider    Return to top

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

Prevention    Return to top

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








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