Canine Scott Syndrome

Other Names: Platelet Procoagulant Deficiency, Scott Syndrome, CSS
Affected Genes: ANO6
Inheritance: Autosomal Recessive
Mutation: chr27:8912219 (canFam3): C/T

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Common Symptoms

Canine Scott syndrome is an inherited blood clotting disorder affecting Shiloh shepherds. Affected dogs are born with platelet defects resulting in inadequate blood clotting at sites of tissue damage. These dogs commonly develop excessive bruising and bleeding associated with surgery, veterinary dental procedures, or traumatic events. In addition, some affected dogs may also develop spontaneous, nontraumatic bleeding episodes including severe nosebleeds, bruising, bleeding in joints, and excessive bleeding during teething. Veterinarians performing surgery on known affected dogs should have ready access to blood banked for transfusions.

Breed-Specific Information for the Shiloh Shepherd

Shiloh shepherd is included as a breed susceptible to Canine Scott Syndrome because of its close relatedness to the German shepherd dog breed, which is known to develop this disease due to Mutation of the ANO6 gene. The frequency of the causal mutation in the general Shiloh shepherd population is unknown.

Testing Tips

Genetic testing of the ANO6 gene in Shiloh shepherds will reliably determine whether a dog is a genetic Carrier of canine Scott syndrome. Canine Scott syndrome is inherited in an Autosomal Recessive manner in dogs meaning that they must receive two copies of the mutated gene (one from each parent) to develop the disease. In general, carrier dogs do not have features of the disease but when bred with another carrier of the same Mutation, there is a risk of having affected pups. Each pup that is born to this pairing has a 25% chance of inheriting the disease and a 50% chance of inheriting one copy and being a carrier of the ANO6 gene mutation. Reliable genetic testing is important for determining breeding practices. In order to eliminate this mutation from breeding lines and to avoid the potential of producing affected pups, breeding of known carriers to each other is not recommended. Shiloh shepherds that are not carriers of the mutation have no increased risk of having affected pups.

There may be other causes of this condition in dogs and a normal result does not exclude a different mutation in this gene or any other gene that may result in a similar genetic disease or trait.

References

  • Brooks MB, Catalfamo JL, MacNguyen R, Tim D, Fancher S, McCardle JA. A TMEM16F Point Mutation Causes an Absence of Canine Platelet TMEM16F and Ineffective Activation and Death-Induced Phospholipid Scrambling. J Thromb Haemost. 2015 Dec;13(12):2240-52. doi: 10.1111/jth.13157. [PubMed: 26414452]
  • Jandrey KE, Norris JW, Tucker M, Brooks MB. Clinical Characterization of Canine Platelet Procoagulant Deficiency (Scott Syndrome). J Vet Intern Med. 2012 Nov-Dec;26(6):1402-7. doi: 10.1111/j.1939-1676.2012.01012.x. [PubMed: 23061683]