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Polycystic Kidney Disease
(PKD)
 

 


 

by Arnold Plotnick MS, DVM, ACVIM, ABVP

Overview

Polycystic Kidney Disease is a slowly progressive, irreversible, inherited kidney disease in Persian and Persian-related cats.   Although reported in the veterinary literature for approximately 30 years, the disease and its genetic mode of inheritance has only been clearly understood in the last 10 years. 

Ultimately, PKD can result in renal (kidney) failure, with clinical signs similar to those of cats with naturally occurring renal failure.  The disorder is present at birth.  Multiple small cysts slowly grow in size, causing the kidney to enlarge dramatically.  The cysts replace the normal kidney tissue, while kidney function continuously declines.  The cysts can vary in size, from less than 1 millimeter to greater than 1 centimeter.  The older an affected cat gets, the larger and more numerous are the cysts.  PKD often progresses to cause clinical signs of kidney failure late in the cat’s life, with the average age being around 7 years, although the slowly progressive nature of the disorder results in some cats not developing signs clinical renal disease as their bodies learn to adjust to the slowly failing kidneys.

Clinical signs

Clinical signs of PKD are non-specific and are similar to those seen in cats with chronic renal failure of any cause.   These include lethargy, decreased appetite or anorexia, excessive drinking, excessive urination, weight loss, and sporadic vomiting. 

Diagnosis

Physical examination findings, and routine laboratory data are both insufficient for confirming or refuting a diagnosis of PKD early in the course of the disease because the kidneys remain normal in size, and maintain their normal contour.  As the disease progresses, and the size and number of cysts increase, the kidneys enlarge, and this may be detected on physical examination.  In severe, advanced cases, cysts may protrude beyond the normal kidney surface, causing an irregular contour that can be felt on physical examination.  If chronic renal failure develops, blood tests will detect this, but blood tests alone can not identify the cause of the renal failure as being due to PKD.

X-rays may or may not be helpful, depending on the age of the cat and the extent of the disease.  As cats with PKD get older, the kidneys enlarge, and this can be detected on the x-rays.  Early in the course of the disease, the contour of the kidneys is usually smooth.  Later in the disease, the outer surface of the kidney becomes more irregular.  Injection of an intravenous dye can highlight the urinary system on the x-rays.  This test is not very helpful early in the disease, however, as the disease progresses, the dye may outline numerous cysts present throughout the entire kidney.

Ultrasound is a sensitive, non-invasive technique for diagnosing cysts in the kidney. Small cysts are often detected as early as 6 to 8 weeks of age, and occasionally as young as 4 weeks of age.  After 10 months of age, the disease can be diagnosed with approximately 95% accuracy, using the proper equipment and experience. 

A genetic test is available that is likely to become the test of choice for many breeders because samples can be easily taken by veterinarians and shipped to special laboratories that perform the test.  Kittens do not have to be 10 months old before a reliable result can be obtained.  Cats can be tested at any age using a cheek swab.  (Pre-weaning kittens, if being tested, should have a blood sample drawn rather than a cheek swab, as nursing kittens may have traces of the queen’s DNA in their mouth, causing a false test result. It may be preferable to wait until the kitten is weaned so that testing can be done using a cheek swab, which is less stressful than taking a blood sample). Ultrasound screening will always be available to clients and breeders who prefer this method of diagnosis.

Treatment

There is no specific therapy for PKD at the moment.  Treatment for cats with PKD in which the disorder has progressed to chronic renal failure is similar to that for chronic renal failure of any cause: 

- restricted protein and phosphorus diet – these diets help reduce the levels of kidney toxins that contribute to the clinical signs of kidney failure, and may in fact slow the progression of renal failure.

- subcutaneous fluids – some cats benefit from having their owners administer fluids under the skin.  This helps encourage urination , maintain hydration, and keep the levels of toxins in the bloodstream lowered.

- phosphorus binders – cats with kidney failure have an impaired ability to excrete phosphorus.  Elevated phosphorus levels contribute to the progression of renal failure in cats.  Phosphorus binders will bind up much of the phosphorus in  the diet, making it unavailable to the cat and preventing elevated levels from developing.

- calcitriol (vitamin D) – cats with kidney failure cannot synthesize vitamin D properly.  This may have deleterious effects on several body systems.  Calcitriol is a synthetic form of Vitamin D.  Administration of calcitriol must be monitored closely by your veterinarian

- erythropoietin – the kidneys make a hormone that instructs the bone marrow to maintain the proper number of red blood cells in the circulation.  The hormone is called erythropoietin.  As the kidneys fail, they produce less erythropoietin, and cats become anemic.  Cats with severe anemia may benefit from having erythropoietin prescribed.

- antacids – elevated levels of kidney toxins can cause stomach ulcers, nausea, and vomiting.  Antacids may help reduce these signs.

- Careful monitoring of blood pressure – hypertension can increase the rate of progression of kidney disease.  Anti-hypertensive medications can be given to bring the blood pressure back to normal and reduce damage to the kidneys

Preventive Care

Prevention requires responsible breeding to eliminate PKD from Persians and Persian-related breeds. Genetic studies in cats have shown that PKD in cats in autosomal dominant.  This means that if an affected cat is bred to a normal cat, 50% of the offspring will be affected.  Cats diagnosed with PKD should not be bred.  If a cat is found positive for cysts, its parents, siblings, and offspring should be evaluated.  Those that are affected should be neutered or spayed so that they are effectively removed from the breeding population.  Breeders of Persian and Persian-related breeds should have all of their cats and kittens evaluated using ultrasound or genetic testing for the presence of PKD. 

 

Updated 2/9/06