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     Canine Juvenile Renal Dysplasia

Renal Cortical Hypoplasia/Juvenile Renal Failure 

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Renal Cortical Hypoplasia is a condition which occurs in the embryo when there is a developmental disruption to the ureter, the tube connecting the bladder to the kidney, resulting in the kidneys developing incompletely and being smaller than average. It usually results in infection and stone formation.

When both kidneys are affected the condition is called Bilateral renal hypoplasia Usually this means you have chronic kidney failure as the normal functioning of the kidney is greatly impaired. The main role of the kidney is to filter the blood and regulate the amount of water absorbed, and the disruption to this function caused by the disorder is very serious. Affected dogs will experience intense thirst, excessive urination, and vomiting,leading to the dehydration of the animal, and creating a cycle where the dog is intensely thirsty again. Convulsions, anemia and weakness may also be present.  First signs may appear at 10 - 13 weeks of agebut generally it is young dogs,  3-24 months old which are affected.  Accompanying this the animal may have signs of ulceration in the mouth and foul-smelling breath associated with reduced appetite and weight loss. In the final stages of the disease the dog may pass small amounts of urine.  In addition to all of these symptoms, affected dogs are usually extremely depressed.

There is no cure; the treatment consists of managing the signs and trying to prevent additional damage to the kidneys.

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Juvenile Renal Dysplasia

http://www.upei.ca/cidd
related terms:
familial/hereditary/juvenile renal disease, familial/hereditary/juvenile nephropathy, renal dysplasia, hereditary nephritis, renal amyloidosis

What is familial kidney disease?
Several breeds of dogs are known to be affected by a wide range of kidney diseases that are proven or suspected to be inherited. In many of these disorders, the kidneys are apparently normal at birth but begin to deteriorate early in life (less than 1 year of age). While the underlying problem in the kidneys varies between breeds, the end result is the same - kidney failure for the dog, generally by 5 years of age, although the severity and rate of progression varies between breeds and between individual dogs.

For many of these disorders, inheritance is not proven. The term "hereditary" is used when the inheritance is known, and "familial" when the mode of inheritance is not known but the disorder occurs more commonly in related dogs than would be expected by chance. 

Where the actual defect in the kidney has been identified, a more specific name (given below) may be used  Often several names are in use for a particular disorder (see related terms above).
Breed
Disorder
 Inheritance (if known)
 (for veterinarian)
Bernese mountain dog
 familial glomerulonephritis
 thought to be autosomal recessive
 membranoproliferative glomerulonephritis; immune-mediated
bull terrier
 hereditary nephritis
 autosomal dominant 
 defect is in the structure of the glomerular basement membrane
Cairn terrier
 polycystic kidney disease
 inheritance tentative (rare)
 kidney tissue gradually replaced by fluid-filled cysts
Doberman pinscher
 familial glomerulonephropathy
 unknown
 irregularities in glomerular basement membrane
English cocker spaniel
 hereditary nephritis
 autosomal recessive
 abnormality in glomerular basement membrane 
Lhasa apso, shih tzu   *
 renal dysplasia
thought to be autosomal recessive
 abnormal differentiation of kidney tissue
Norwegian elkhound
 familial nephropathy
 unknown
 tubulo-interstitial nephropathy
Pembroke Welsh corgi
 renal telangiectasia
 unknown
 multiple dilated renal blood vessels
Samoyed
 hereditary nephritis
 X-linked dominant
abnormality in glomerular basement membrane 
Chinese shar pei
 renal amyloidosis
 unknown, possibly autosomal recessive
 amyloid deposition most disruptive in kidneys; other organs (spleen, liver, pancreas) can be affected but with minimal clinical signs; may also have intermittent fever and joint swelling
soft-coated wheaten terrier
 renal dysplasia
 thought to be autosomal recessive
 abnormal differentiation of kidney tissue
soft-coated wheaten terrier
 protein-losing enteropathy and nephropathy   

* Renal dysplasia may also be inherited in the Alaskan malamute, chow chow, golden retriever,  miniature schnauzer, and standard poodle.

   
For many breeds and many disorders, the studies to determine the mode of inheritance or the frequency in the breed have not been carried out, or are inconclusive. We have listed breeds for which there is a consensus among those investigating in this field and among veterinary practitioners, that the condition is significant in this breed.

What does familial kidney disease mean to your dog & you?
The signs of kidney failure are the same in young animals as in older dogs.  One of the earliest signs you may notice is that your dog drinks more and urinates more. (Sometimes in young pups, owners may not recognize excessive urination as such, but think it is due to the dog being slow to housetrain.) As more and more kidney function is lost, other signs develop including poor appetite, loss of weight, lethargy, vomiting, and pale gums due to anemia. Although the progression may be slowed by treatment, ultimately kidney failure is fatal.

For most of these disorders, dogs show signs of kidney dysfunction before 1 year of age, and their kidneys fail before 5 years of age. In pups affected at a very young age (less than 5 or 6 months), growth may be noticeably stunted.

Hereditary nephritis in bull terriers is an exception. Affected animals often show no signs until 2 years of age or more, and may not develop kidney failure until 8 years. In Doberman pinschers and Norwegian elkhounds, signs of kidney failure may also appear later in life (6 years of age).

In the samoyed, because inheritance is sex-linked, males are more severely affected. Male pups with hereditary glomerulonephropathy usually show signs by 2 to 3 months of age, and kidney function deteriorates rapidly, resulting in death by an early age (approximately 1 year). Although affected females still show some kidney dysfunction, it is less severe and does not progress to kidney failure. Even if only mildly affected, they are carriers of the disorder and should not be bred.

In Chinese shar-pei dogs affected with amyloidosis, amyloid (a type of protein) is deposited in the kidneys and other organs. Most of the time, the clinical signs you see relate to disruption of kidney function which eventually (by about 3 to 5 years of age) leads to kidney failure. Affected dogs may have intermittent fever and swelling and pain in their joints, especially the hocks.

Pembroke Welsh corgis with renal telangiectasia have episodes of blood in the urine as adults.

How is familial kidney disease diagnosed?
The signs described above can have several causes, including other inherited disorders such as a heart or liver defect. Your veterinarian will do blood and urine tests that will point to your dog's kidneys as the cause of the problem.

For the veterinarian: Typical signs of CRF include azotemia, hyperphosphatemia, isosthenuria, and nonregenerative anemia. Calcium concentrations are variable. Persistent proteinuria is highly suggestive of hereditary nephritis, even before any clinical signs are evident. Proteinuria is not a consistent sign in renal dysplasia.

In suspect bull terriers, repeated protein/creatinine ratios >0.3 provide supportive evidence for hereditary nephritis in dogs that are older than 2 years of age, but who show no overt signs of renal failure. This provides important information in decisions about breeding.

Male samoyeds with hereditary glomerulonephropathy develop proteinuria, glucosuria and isosthenuria by 2 to 3 months of age, and azotemia and overt renal failure by 6 to 9 months. Affected females (carriers) develop proteinuria at a young age but may never progress to renal failure.

In cocker spaniels with familial nephropathy, proteinuria is usually present by 4 to 6 months of age and is persistent and marked. This is followed by decreased growth, and the development of isosthenuria and azotemia.

There are 2 different familial renal syndromes in the soft-coated wheaten terrier - renal dysplasia (onset usually before 2 years of age) and protein-losing enteropathy and nephropathy (onset 2 to 11 years of age).

In Chinese shar pei dogs with renal amyloidosis, proteinuria is usually marked, although it may be mild or absent in dogs with renal medullary, but not glomerular, amyloidosis.

How is familial kidney disease treated?
There is no cure, but there are ways to slow the progression of kidney disease, which your veterinarian will discuss with you. These include making sure your dog always has access to fresh drinking water, feeding a special (high quality, low-protein) diet, and the use of some medications which help to support kidney function.

Your veterinarian will help you to recognize the signs that your dog's kidneys have deteriorated to the point where euthanasia becomes the best option.

For the veterinarian: In some cases, angiotensin converting enzyme inhibitors (such as enalapril) may delay the onset of azotemia, lower the blood pressure, improve renal blood flow, delay decline in glomerular filtration rate, and slow the rate of increases in proteinuria.

Breeding advice
Affected animals should not be bred, and neither should their parents. Any related animal being considered for breeding should be screened for protein in the urine, which is an early sign of kidney disease.

All bull terriers being considered for breeding should be screened by measuring protein/creatinine ratios ( a simple test requiring a blood and a urine sample). Ratios greater than 0.3 on 2 occasions more than a month apart, indicate potential hereditary nephritis. Kidney function in these dogs should be monitored, and they should not be used for breeding.

FOR MORE INFORMATION ABOUT THIS DISORDER, PLEASE SEE YOUR VETERINARIAN.

Resources
Ackerman, L. 1999. The Genetic Connection:A Guide to Health Problems in Purebred Dogs. AAHA Press, Lakewood, Colorado.
Lees, GE. 1998. Congenital renal disease in dogs and cats. ACVIM-Proceedings of the 16th Annual Veterinary Medical Forum, p 28-30.
DiBartola, SP. 1995. Familial renal disease in dogs and cats. In E.J. Ettinger and E.C. Feldman (eds) Textbook of Veterinary Internal Medicine, p. 1796-1801.  W.B. Saunders Co., Toronto.
Gleadhill, A. 1997. Juvenile nephropathies in dogs and cats. In Practice. Vol 19(5):270-277.
DiBartola, S. 1998. Renal amyloidosis. ACVIM-Proceedings of the 16th Annual Veterinary Medical Forum. p. 23-24.
Grower, GF, DiBartola, SP. 1995. Glomerular disease. In E.J. Ettinger and E.C. Feldman (eds.) Textbook of Veterinary Internal Medicine, p. 1760-1775. W.B. Saunders Co., Toronto.
Copyright © 1998 Canine Inherited Disorders Database. All rights reserved.
Revised: December 14, 2001.
This database is a joint initiative of the Sir James Dunn Animal Welfare Centre at the Atlantic Veterinary College, University of Prince Edward Island, and the Canadian Veterinary Medical Association
reprinted with kind permission from Alice Crook,
DVM Coordinator, Sir James Dunn Animal Welfare Centre
Atlantic Veterinary College University of Prince Edward Island

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Below is a copy of many of the recent e mails on this subject, circulated by David Payne, Videx.
According to this e mail and conversations with various people, this is actually quite a big problem in the UK and with GSD's dying as puppies very distressing for breeders and new puppy owners.


To all GSD breeders & owners in the UK
 
Over many years I have heard of the occasional litter being apparently affected by this disease, indeed I have been contacted by several of the breeders of such litters, and I felt somewhat lacking in knowledge and experience to offer them any meaningful advice, other than in the manner they dealt with devastated owners who had lost their much loved pet GSD puppy, in most cases between 4 and 6 months of age.

During the last year or so there has been a spate of litters affected, where all or the majority of each litter have died in the same manner. Kidney failure through underdeveloped kidneys.

Having had an enquiry to purchase one of my puppies from a couple who had very recently lost their adored 5 month old male GSD puppy, I invited them to come and visit to view my puppy, which they bought. However I found it a very emotional experience when they explained how they had lost their previous puppy aged 5 months with kidney failure, and how they were told by the breeder that four of the litter of seven had died of the same cause, and more deaths were possible amongst the remainder. This lovely couple were devastated and in tears over their tragic loss. (see the story of Buster below)

There and then I decided to start some very serious and thorough research into this terminal disease of renal dysplasia.

It soon became apparent that there had been many years of denial within our breed with regards to its existence, and also varying degrees of suppression of information surrounded some affected litters. However with my ability to research and obtain information, coupled with my complete inability to be intimidated by anyone, including well known breeders and leading personalities within our breed, I soldered on.

As I gained clearer information about the disease, most of which came from other affected breeds, I circulated this information by email throughout the GSD fraternity within the UK, and on occasions outside of the UK also. I was informing, raising awareness, and on a fishing trip. I was, as expected, NOT inundated with responses, but those responses I did get were very useful and gave me leads to various avenues of research to explore.

After some 100+ hours of internet research, and various phone calls, I made a major breakthrough. I discovered that a Genetic DNA test for canine JRD had been developed in Canada, apparently late in 2007. This test was apparently developed for the Lhasa Apso dog breed, but is now used to test many more canine breeds. The test is done by DOGenes canine DNA services of Canada.

DNA TEST FOR JRD
This DNA test (a swab of the gums) can identify whether a dog (male or female) is affected, a carrier, or free of the JRD disease. The normal charge for this DNA test is between 70 US$ and 155US$ per test. I am pleased to report that I have been offered some DNA tests for JRD free of charge by DOGenes Inc. of Canada, and they will send me the test swab kits also free of charge. There will obviously be postal charges.

I would like to send swabs for analysis with this DNA test from a range of GSD, for example:

1) Sire and/or dam of affected litters

2) surviving puppies from affected litters

3) affected GSD

I may also be able to include swab samples from litters which the breeder is seriously concerned may develop the disease. Especially litters from a suspect line.

I have identified two sires that appear in most, if not ALL cases of JRD that I have identified. (both Sires are in Busters pedigree) However until these DNA genetic tests are complete, it would be wrong for me to name them, however there is an urgency to collect these swab samples and complete the tests, so that breeders can make informed decisions regarding their future breeding plans, with due regard to this JRD disease.

I envisage the possibility of GSD who are tested being identified as either – affected, carrier, or clear.

I am also in contact with Animal Health Trust (AHT)of Newmarket, with a view to DNA tests for JRD being done by them, however that may require an agreement with DOGenes Inc. of Canada.

If any GSD breeder or GSD owner in the UK is interested in one or more of their GSD being included in the free DNA test for JRD, please contact me, in strict confidence, and I will then get an idea of the number of tests desired, and see if I can obtain them all free. I would like to indicate numbers to DOGenes Inc. within the next 7 days.

David Payne - VIDEX GSD
email: enquiries@videxgsd.com
phone +44 (0)1905 830900

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Juvenile Renal Disease
 Renal Dysplasia in the Shih tzu Dog
 Juvenile Renal Disease
 DNA Test Study in German Shepherd Dogs (GSD) UK.
is making excellent progress.
JRD Testing




chloebutton   talabutton

The above information is simply informational. It's intent is not to replace the advice of a veterinarian nor to assist you in making a diagnosis of your pet. Please consult with your own veterinarian for confirmation of any diagnosis. Your pets life may depend on it.