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Fanconi Syndrome

Fanconi Syndrome: An Overview

Fanconi syndrome is a condition where the tubules of the kidneys do not function properly. In the normal kidneys the tubules there function to reabsorb vitamins, minerals, and sugars back into the body to be reused. In Fanconi affected dogs the reabsorption of these solutes does not occur and are consequently dumped into the urine and excreted. The solutes affected include sodium, glucose, phosphorus, amino acids, potassium, and bicarbonate. The lost of these solutes can lead to dehydration and electrolyte imbalances along with other problems as well. The disease is progressive though rate of progression is variable.

The most common breed affected by this disease is the Basenji. It is genetic in nature and there is currently a linkage test available to breeders. The test does not locate the specific gene for Fanconi's but is a tool breeders can use to improve their breeding program. Other breeds which have a high prevalence of the condition are Norwegian Elkhounds and Schnauzers.

Causes of Fanconi Syndrome

In some breeds, primarily the Basenji, Fanconi syndrome is genetic meaning it is passed down from parents to offspring. There are cases however where the disease is acquired due to idiopathic (unknown) means or from medication side affects. In many cases it is hard to differentiate between genetic and idiopathic causes.

Symptoms of Fanconi Syndrome

The most common signs associated with Fanconi syndrome are polydipsia and polyuria or excessive drinking and urinating respectively. This is due to the inability of the dogs to concentrate their urine. Weight loss and overall poor condition are also seen in Fanconi affected dogs. As the condition progresses electrolyte imbalances and metabolic acidosis may occur also.

Diagnosing Fanconi Syndrome

Fanconi syndrome is diagnosed by testing the levels of solutes in the urine and the blood. Dogs with Fanconi syndrome will have high amounts of the solutes normally reabsorbed back into the body including glucose, sodium phosphorus, amino acids, potassium, and bicarbonate. Any combination of these can be present. One of the more common signs of Fanconi's is high levels of glucose in the urine with normal levels of glucose in the blood. Along with urine tests, blood tests are also performed to determine the level of the solutes in the blood.

Treating Fanconi Syndrome

There is currently no treatment which can fix the tubular defect which causes Fanconi?s. The treatment suggested is to control the disease allowing the dog to live a long, happy life. Fresh water should always be available. This reduces the chance of the dog becoming dehydrated. Research done by Dr. Steve Gonto has lead to a protocol which specifies what supplements can be given to help regulate the dog's solute levels. The Gonto protocol, or Fanconi protocol, can be found at https://www.basenji.org/ClubDocs/fanconiprotocol2003.pdf or www.zandebasenjis.com/protocol.htm. These sites give an in depth look at the protocol and how it can be administered. The most important objective in treatment is to prevent metabolic acidosis which can be fatal. Each dog should be evaluated on an individual bases as no one case is the same.

Care for Dogs with Fanconi Syndrome

Once Fanconi Syndrome is under control the dog can be treated like any other dog and will be able to participate in normal family activities such as hiking or running. Supplements should be given routinely as directed by your veterinarian and annual tests should be done to confirm that the Fanconi's is still under control.

It is suggested that in dogs which are at a great risk to develop Fanconi's, mainly as Basenjis, be tested for glucose in their urine once a month starting at age 3. This can be done using tests strips which are available at most pharmacies. A negative test does not mean that your dog does not have Fanconi's. High risk dogs should continue to be tested every month as the syndrome can develop at any point. Just as a negative test does not mean the dog does not have the condition, a positive urine glucose test does not indicate that the dog has Fanconi?s, but is an indication that further testing should be done. Early detection of the disease decreases the risk of kidney damage. With proper management these dogs can live to ages similar to those without Fanconi syndrome.

The AKC Canine Health Foundation and Fanconi Syndrome

The AKC Canine Health Foundation has funded 4 projects from the University of Missouri on researching Fanconi syndrome in the Basenji. The funds, which total $59,000, have been used to develop a genetic linkage test to help Basenji breeders. Research continues with the hope that eventually the specific gene associated with the condition will be identified.

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