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Von Willebrand Disease III (Kooikerhondje Type)

Von Willebrand Disease III (vWDIII) in Kooikerhondjes is an inherited bleeding disorder characterized by extremely low levels of von Willebrand factor, critical for blood clotting.

Affected Genes: VWF

Inheritance: Autosomal Recessive

Variant(canFam6):
chr27:7722078: C>T

Breed: Kooikerhondje

General Information: Von Willebrand Disease III (vWDIII) in Kooikerhondjes is a serious inherited bleeding disorder that leads to significantly low or even undetectable levels of the von Willebrand factor (vWF), an essential protein for normal blood clotting. Dogs with vWDIII are prone to easy bruising, frequent nosebleeds, spontaneous bleeding from the mouth, and prolonged bleeding following injuries or surgeries. The severity of the bleeding can be life-threatening in some cases, making immediate access to veterinary care and blood transfusions crucial. Carriers of the gene mutation may also exhibit decreased vWF levels, though typically not as severely, and may not be identified until they experience a traumatic event or surgery that triggers excessive bleeding. Despite these challenges, many affected dogs can lead normal lives if carefully managed, but the risk of severe bleeding remains a significant concern.

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 essential for identifying carriers of the von Willebrand Disease III (vWDIII) mutation in Kooikerhondjes. This disorder is inherited in an autosomal recessive manner, meaning a dog must inherit two copies of the mutated gene, one from each parent, to be affected by the disease. Carrier dogs, which have only one copy of the mutation, may show reduced levels of vWF and have mild symptoms, but they play a critical role in the genetic propagation of the disease. When two carriers are bred, each puppy has a 25% chance of being affected by vWDIII and a 50% chance of being a carrier. Genetic testing is crucial for responsible breeding practices to avoid pairing two carriers and thus prevent the birth of severely affected puppies. By ensuring that only non-carriers are used in breeding, the prevalence of this debilitating disorder can be significantly reduced, promoting healthier future generations.

References:
Rieger M, Schwarz HP, Turecek PL, Dorner F, van Mourik JA, Mannhalter C. Identification of mutations in the canine von Willebrand factor gene associated with type III von Willebrand disease. Thromb Haemost. 1998 80(2):332-7.

van Oost BA, Versteeg SA, Slappendel RJ. DNA testing for type III von Willebrand disease in Dutch kookier dogs. J Vet Intern Med. 2004 18(3):282-288.