Disease Ontology ID: DDTO:0000452

Another Name: Factor IX deficiency

Disease Description: A blood clotting disorder due to lack of coagulation factor IX.

Inherit Mode: Hemophilia is an X-linked,recessive disorder.It is one of the few sex-linked traits in dogs. Because males have only 1 X chromosome, a male dog is either affected or clear of the defect. Females, with 2 X chromosomes, may be affected (abnormal gene on both chromosomes), clear, or a carrier with no clinical signs (one gene affected). In effect, the disease is carried by females but affects mostly males. The disease occurs in many different breeds and in mixed breed dogs as well. The German shepherd is the breed most commonly affected.

Disease Symptom: Dogs with mild forms of hemophilia may experience few or no signs, and may never require treatment until/unless surgery or trauma is followed by excessive bleeding. Where hemophilia is more severe, you may see signs of a problem at a fairly early age. Your pup may have prolonged bleeding associated with the loss of baby teeth, or unexplained areas of bleeding under the skin. Bleeding into muscles or joints will often cause lameness. Once the condition is diagnosed, your veterinarian will discuss ways to manage this lifelong problem. These include being alert for signs of bleeding episodes in your dog, and tips on housing and maintenance so as to minimize risks of bleeding. Periodic blood transfusions will generally be required. Unfortunately, dogs with severe hemophilia often die or are euthanized because of recurrent or uncontrollable bleeding problems.

Disease Cause: -

Disease Diagnose: The clinical signs associated with hemophilia vary widely, based on the severity of the bleeding disorder and where in the body the bleeding occurs. Because this is a sex-linked disorder, dogs with hemophilia are almost always male. Affected dogs are commonly brought to the veterinarian for problems such as bloody diarrhea that is difficult to control, areas of bleeding under the skin, or lameness (due to bleeding into muscles or joints). Bleeding under the skin or into the muscle may occur after routine vaccination, or there may be prolonged or severe bleeding at surgery (such as when your dog is neutered.) Other less common problems include respiratory difficulties due to bleeding into the chest or around airways, or weakness, paralysis, or even sudden death due to bleeding into the brain or spinal cord. Once a bleeding disorder is suspected, specialized laboratory tests are carried out to diagnose the specific disorder. If your pup is diagnosed with hemophilia, it is important that you inform the breeder so that he or she can have your dog's parents tested. (The mother is likely a carrier and the father free of the defect.)

Treat Method: There is no cure for this disorder. Mildly affected dogs may never require treatment, or only after surgery or trauma. With more severe hemophilia, your dog will require periodic transfusions when bleeding occurs, to replace the deficient coagulation factor activity. Strict cage rest is important along with transfusion, to decrease further hemorrhage.

Breeder Advice: Because hemophilia is a sex-linked recessive trait and the carrier state can be detected by testing, this disorder can be controlled. German shepherd females and females from lines of other breeds where hemophilia has been diagnosed, should be tested for the carrier state. Males used for breeding should be screened for the disorder.

Disease Description Source: Link