Juvenile Laryngeal Paralysis & Polyneuropathy
The Officers, Board of Directors and members of the Health Committee are pleased to announce that the first major Health Initiative undertaken by the BRTCA will be a research project conducted by Dr. Dennis O’Brien, DVM PhD and Gary Johnson, DVM PhD at the University of Missouri in Juvenile Laryngeal Paralysis & Polyneuropathy (JLPP) in Black Russian Terriers.
While JLPP is by far not the most common problem affecting the breed, we feel that it is the first area to concentrate on because of the devastating effects of JLPP, with a 100% mortality rate in affected puppies. There is no treatment to offer and all will die at between 4-6 months of age. The disease is difficult to diagnose so the true incidence is unknown, as the deaths are generally attributed to respiratory failure, such as pneumonia, which is secondary to the disease. We are just now in the infancy stage of seeing this disease in the United States, however we know that carriers are present from Europe and we feel that it is imperative to find the genetic marker before an explosion of cases develop now that these descendants are of breeding age.
Description:
The effects of the disease are first seen in the larynx and throat. Affected pups appear to develop normally, but shortly after weaning age they develop difficulty breathing caused by paralysis of the muscle in the larynx (voice box). The muscles fail to pull the vocal folds out of the way when the dog inhales causing a wheezing sound as air flow into the lungs is restricted. The paralysis rapidly progress to the limbs and if the pups have not succumbed to pneumonia first, they ultimately are totally paralyzed and humanely euthanized.
JLPP appears to be an autosomal recessive hereditary disease. That is, each parent must be a carrier of the gene. Recessive genes, whether they deal with conformation traits or genetic defects, can stay hidden for generations.
Incidence:
We have definite lab diagnosis of it occurring in 3 litters in US. Also, JLPP is suspected in 2 other US litters and one in Canada. Dr. Obrien has information on 2 affected litters in Europe.
The big problem is that the litters we have documented here are beginning to show some common breeding which include some of the most famous and popular modern lines right now.
Remembering that recessive genes can stay hidden for generations, there are many litters with these ancestors here and around the world just coming to breeding age.
Without a genetic test, the only way to know if a dog is a carrier is after the disease shows up in a litter. Being a simple recessive inheritance, the statistical odds for an average litter where both parents are carriers are: 25% affected (1-3 pups will die), 50% carrier, and 25% clear. However, more could be affected and die and more could be carriers. More carriers in the breeding pool will increase the probability of producing affected litters in just a few years.
Dr. O’Brien believes that this recessive gene has probably been in the breed from its early days, but is just starting to concentrate enough to show up more and be recognized as a genetic disease.
Until a marker and DNA test are developed, there is no way, short of diagnosing an affected puppy, that the parents are each carriers; and surviving puppies of carriers may themselves be unknown carriers that eventually become part of a breeding pool.
The awareness of JLPP in Black Russians has been as a direct result of a few dedicated individuals who recognized a potential genetic problem and took the initiative to make a difference. This is an opportunity for the BRT Community worldwide to step up and work together for the future of the breed.
Funding
Funding is our major issue. This study will cost $12,000. It will yield benefits beyond this JLLP project because all of the genetic data will be mapped and can be used in other studies.
BRTCA has already contributed $5,000 to the University of Missouri towards the study. Additional funding will need to be raised through direct donations and other fundraising events.
It is our hope that the BRT Community will donate to this much needed research project. Donations may be made by check or through the “donation PayPal” link at the top of the page. This study needs to happen as quickly as possible, so that these carriers can be identified. It is too important to the future of the breed to delay it any longer.
We will also gladly accept items that we can sell from the Website to raise funds dedicated to this important research project as well as items donated to the National Auction and Raffle and earmarked for the JLPP Project.
Please do whatever you can to help and circulate to any other BRT owners who you may know.
While JLPP is by far not the most common problem affecting the breed, we feel that it is the first area to concentrate on because of the devastating effects of JLPP, with a 100% mortality rate in affected puppies. There is no treatment to offer and all will die at between 4-6 months of age. The disease is difficult to diagnose so the true incidence is unknown, as the deaths are generally attributed to respiratory failure, such as pneumonia, which is secondary to the disease. We are just now in the infancy stage of seeing this disease in the United States, however we know that carriers are present from Europe and we feel that it is imperative to find the genetic marker before an explosion of cases develop now that these descendants are of breeding age.
Description:
The effects of the disease are first seen in the larynx and throat. Affected pups appear to develop normally, but shortly after weaning age they develop difficulty breathing caused by paralysis of the muscle in the larynx (voice box). The muscles fail to pull the vocal folds out of the way when the dog inhales causing a wheezing sound as air flow into the lungs is restricted. The paralysis rapidly progress to the limbs and if the pups have not succumbed to pneumonia first, they ultimately are totally paralyzed and humanely euthanized.
JLPP appears to be an autosomal recessive hereditary disease. That is, each parent must be a carrier of the gene. Recessive genes, whether they deal with conformation traits or genetic defects, can stay hidden for generations.
Incidence:
We have definite lab diagnosis of it occurring in 3 litters in US. Also, JLPP is suspected in 2 other US litters and one in Canada. Dr. Obrien has information on 2 affected litters in Europe.
The big problem is that the litters we have documented here are beginning to show some common breeding which include some of the most famous and popular modern lines right now.
Remembering that recessive genes can stay hidden for generations, there are many litters with these ancestors here and around the world just coming to breeding age.
Without a genetic test, the only way to know if a dog is a carrier is after the disease shows up in a litter. Being a simple recessive inheritance, the statistical odds for an average litter where both parents are carriers are: 25% affected (1-3 pups will die), 50% carrier, and 25% clear. However, more could be affected and die and more could be carriers. More carriers in the breeding pool will increase the probability of producing affected litters in just a few years.
Dr. O’Brien believes that this recessive gene has probably been in the breed from its early days, but is just starting to concentrate enough to show up more and be recognized as a genetic disease.
Until a marker and DNA test are developed, there is no way, short of diagnosing an affected puppy, that the parents are each carriers; and surviving puppies of carriers may themselves be unknown carriers that eventually become part of a breeding pool.
The awareness of JLPP in Black Russians has been as a direct result of a few dedicated individuals who recognized a potential genetic problem and took the initiative to make a difference. This is an opportunity for the BRT Community worldwide to step up and work together for the future of the breed.
Funding
Funding is our major issue. This study will cost $12,000. It will yield benefits beyond this JLLP project because all of the genetic data will be mapped and can be used in other studies.
BRTCA has already contributed $5,000 to the University of Missouri towards the study. Additional funding will need to be raised through direct donations and other fundraising events.
It is our hope that the BRT Community will donate to this much needed research project. Donations may be made by check or through the “donation PayPal” link at the top of the page. This study needs to happen as quickly as possible, so that these carriers can be identified. It is too important to the future of the breed to delay it any longer.
We will also gladly accept items that we can sell from the Website to raise funds dedicated to this important research project as well as items donated to the National Auction and Raffle and earmarked for the JLPP Project.
Please do whatever you can to help and circulate to any other BRT owners who you may know.
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