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Von Willebrand Disease III (Shetland Sheepdog Type)

Von Willebrand Disease III (vWDIII) in Shetland Sheepdogs is an inherited bleeding disorder marked by extremely low levels of von Willebrand factor, leading to significant bleeding risks.

Affected Genes: VWF

Inheritance: Autosomal Recessive

Variant(canFam6):
chr27:7745370: 1 bp deletion A

Breed: Shetland Sheepdog

General Information: Von Willebrand Disease III (vWDIII) in Shetland Sheepdogs is a serious genetic bleeding disorder that causes extremely low or nearly absent levels of the von Willebrand factor (vWF), a critical protein for blood clotting. Affected dogs are highly susceptible to easy bruising, frequent nosebleeds, spontaneous bleeding from the mouth, particularly when juvenile teeth are lost, and extended bleeding following surgeries or injuries. The severity of bleeding can be life-threatening, necessitating vigilant care and preparedness for emergency blood transfusions, especially during surgical procedures. Carriers of the vWDIII mutation, having one copy of the gene, may also exhibit decreased vWF levels, but not all carriers are equally affected, and they may remain asymptomatic until a bleeding event is triggered by surgery or trauma. Despite the potential for increased bleeding times, many carrier dogs live normal lives without significant issues, though they remain at risk for serious bleeding during accidental injuries or surgical interventions.

How to Read Your Dog's Test Results for this Genetic Variant:

Two Variants Detected: Dog Likely Affected

One Variant Detected: Dog Unlikely Affected

No Variants Detected: No Effect

Gene / Testing Information: Genetic testing for the VWF gene is crucial for identifying carriers of the von Willebrand Disease III (vWDIII) mutation specific to Shetland Sheepdogs. This disorder is inherited in an autosomal recessive manner, which means that a dog must inherit two copies of the mutated gene, one from each parent, to be affected. Carrier dogs, possessing only one copy of the mutation, generally do not display severe symptoms but can pass the gene to their offspring. If two carriers are bred, each puppy has a 25% chance of developing vWDIII and a 50% chance of being a carrier. Accurate genetic testing is vital for responsible breeding decisions to prevent mating two carriers, thereby reducing the risk of producing severely affected puppies. By selectively breeding only non-carrier dogs, breeders can help eliminate this debilitating condition from Shetland Sheepdog lines, ensuring healthier generations in the future.

References:
Raymond SL, Jones DW, Brooks MB, Dodds WJ. Clinical and laboratory features of a severe form of von Willebrand disease in Shetland sheepdogs. J Am Vet Med Assoc. 1990 197(10):1342-1346.