CSPCA Charitable Trust
Reprinted from September/October 1996 BARKER
I have been asked to summarize remarks presented at the recent CSPCA National Specialty concerning a disease sometimes called "Swollen Hock Syndrome" or "Shar-Pei Fever". Typically, affected Shar-Pei experience repeated episodes of fever and/or lameness. Unfortunately, during these inflammatory episodes, amyloid accumulates in the kidneys and eventually destroys these organs, leading to premature death. Shar-Pei Fever is believed to be an inherited disease because efforts to identify an infectious agent have been unsuccessful.
My involvement in Shar-Pei Fever research began about three years ago when I became aware of a theory by Dr. Linda Tintle and her colleagues that Shar-Pei Fever is the canine version of a human disease known as Mediterranean Fever. This idea seems reasonable because symptoms of the human disease closely resemble Shar-Pei Fever. Mediterranean Fever was known to be an inherited genetic defect and the responsible mutation was known to reside on the short arm of human chromosome 16. As part of an NIH-supported project, I had developed some DNA markers for genes from corresponding regions of dog chromosomal DNA. I, therefore, contacted Dr. Tintle to see if she could supply me with blood samples from families of Shar-Pei so that I could determine if these markers would be useful in identifying dogs that would eventually develop the disease or pass the disease onto their offspring. As the result of subsequent chromosomal maping experiments, the position of the Mediterranean Fever mutation is now known much more precisely than it was three years ago. As it turned out, our markers were from the correct half of chromosome 16, but they were too far away from the Mediterranean Fever defect to be useful, even if Dr. Tintle's theory is correct.
A little over a year ago, Dr. Tintle informed me of an effort to raise $50,000 to support research on Shar-Pei Fever. Because this money could be used to develop additional canine DNA markers closer to the Mediterranean Fever mutation, I told her I was interested and, in fact, would be able to contribute an addition al $25,000 in discretionary funds toward the project. More recently, we have submitted a success- ful Shar-Pei Fever grant proposal to the Canine Health Foundation for an additional $25,000, bringing the total funds for the project to $100,000. These funds are now in the hands of administrators, accountants and lawyers; however, I am confident they will become available to me soon so that I can begin this project in earnest.
The discretionary funds mentioned above stem from a project we are doing for the North American Limousin Foundation which promotes and registers a breed of beef cattle. In the past, a substantial number of these cattle produced calves with protoporphyria, an inherited severe photosensitivity (tendency toward severe sunburn). With support from the NALF, we developed a DNA marker that enables us to identify symptomless carriers of the disease. By excluding carriers from their breeding stock, cattlemen have been able to eliminate protoporphyria from their herds. In less than three years, we have tested over 5,000 head of cattle and greatly decreased the prevalence of protoporphyria in Limousin cattle in this country.
If Dr. Tintle's theory about the relationship of Shar-Pei Fever and Mediterranean Fever is correct, we have a good chance of developing a DNA marker that identifies carriers of Shar-Pei Fever. If so, a simple, reliable and inexpensive blood test could be used to select mutation-free Shar-Pei for breeding puppies that should live long, healthy lives.