Copper Storage Hepatopathy, Wilson’s Disease (Copper Storage Disease of the Liver) in Dogs

  • Occurrence: very rare
  • Location of illness: Abdomen/pelvis
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Copper storage hepatopathy, also known as Wilson’s disease in dogs, is a genetic disorder characterized by the abnormal accumulation of copper in the liver. This leads to liver damage and, if left untreated, can lead to serious health problems.

The most important facts at a glance

Copper storage hepatopathy, also known as Wilson’s disease in dogs, is a genetically determined disease that occurs primarily in certain breeds such as Bedlington Terriers, West Highland White Terriers, and Doberman Pinschers. These dogs have difficulty excreting copper from the liver, which leads to an accumulation. The excess amounts of copper cause oxidative stress and cell damage in the liver, which can lead to Inflammatory conditions and fibrosis. Symptoms of the disease include loss of appetite, Vomiting, Diarrhea, lethargy, and jaundice. In severe cases, neurological symptoms and coagulopathies can occur.

The diagnosis is made through clinical examinations, laboratory analyses, and imaging procedures. A liver biopsy is often necessary to directly determine the copper content. Treatment includes a low-copper diet and medications that promote copper excretion. Antioxidants can help protect the liver. The prognosis depends on the stage of the disease at diagnosis; early detection improves the chances of a good outcome.

Prevention focuses on genetic testing in breeding to identify and exclude affected dogs. For dogs with a genetic predisposition, a low-copper diet is crucial. Regular veterinary examinations are important to intervene early if signs of the disease appear. A comprehensive approach that considers both genetic and environmental factors is crucial to minimize the risk of the disease.

Causes

The disease is genetic and often occurs in certain dog breeds, including Bedlington Terriers, West Highland White Terriers, and Doberman Pinschers. These breeds have genetic predispositions that impair their ability to eliminate copper from the liver.

Normally, copper absorbed through food is stored in the liver and used or excreted from the body as needed. In dogs with copper storage hepatopathy, this mechanism is disrupted, causing copper to accumulate in the liver. This accumulation leads to oxidative stress and cell damage, which can eventually lead to liver inflammation and fibrosis.

The genetic basis of the disease varies depending on the breed. In Bedlington Terriers, a specific genetic trait has been identified that is responsible for the accumulation of copper. In other breeds, the genetic basis is not yet fully understood, but there is evidence that multiple genes may be involved.

Environmental factors can also play a role in the severity of the disease. A copper-rich diet can worsen the disease, especially in genetically predisposed dogs. Therefore, managing copper intake is an important aspect of disease control.

Symptoms

Rare symptoms:

The symptoms of copper storage hepatopathy can vary, depending on the stage of the disease and the severity of copper accumulation. The most common signs include loss of appetite, weight loss, lethargy, and Vomiting. These symptoms are non-specific and can easily be confused with other liver diseases.

In the advanced stages, dogs may develop jaundice, which is characterized by a yellowing of the skin and mucous membranes. This is a sign that the liver is severely compromised and can no longer effectively process bilirubin, a waste product of hemoglobin breakdown.

Neurological symptoms can also occur if liver function is severely impaired. These can include Behavioral changes, coordination problems, and, in severe cases, seizures. These symptoms are often indicative of advanced liver disease and require immediate veterinary attention.

Diagnosis

The diagnosis of copper storage hepatopathy requires a combination of clinical examinations, laboratory tests, and imaging procedures. A thorough history and physical examination are the first step in making a presumptive diagnosis. The veterinarian will ask about the dog’s specific symptoms and breed.

Blood tests are crucial to assess liver function. Elevated liver enzymes, bilirubin, and specific copper levels can provide clues to the disease. However, definitive evidence of copper accumulation can only be obtained through a liver biopsy. In this invasive method, a small sample of liver tissue is taken and examined for copper content.

Imaging techniques such as ultrasound or CT scans can be helpful in assessing the condition of the liver and ruling out other possible causes of the symptoms. These methods are non-invasive and provide valuable information about the structure of the liver.

Therapy

The treatment of copper storage hepatopathy aims to reduce copper accumulation in the liver and control symptoms. A special low-copper diet is often the first step. This diet helps to prevent further accumulation of copper and slow the progression of the disease.

Medications that promote the excretion of copper are also an essential part of the therapy. Penicillamine and trientine are chelating agents that help remove copper from the body. However, these medications can have side effects and must be dosed under veterinary supervision.

Antioxidants such as vitamin E and S-adenosylmethionine (SAMe) can also be considered to reduce oxidative stress in the liver. These supplements can help protect liver cells from further damage.

In severe cases where liver function is severely impaired, supportive therapy may be necessary. This may include the administration of fluids and electrolytes, as well as the treatment of complications such as infections or neurological disorders.

Prognosis and follow-up care

The prognosis for dogs with copper storage hepatopathy depends on various factors, including the stage of the disease at the time of diagnosis, the breed, and the response to treatment. With early diagnosis and adequate therapy, the prognosis can be favorable, and many dogs can achieve a good quality of life.

However, dogs diagnosed in the advanced stages of the disease have a poorer prognosis. The liver damage may be irreversible, and treatment often focuses on alleviating symptoms and improving quality of life.

Long-term follow-up and regular veterinary check-ups are crucial to monitor the progression of the disease and adjust treatment accordingly. With proper care, many dogs can lead a relatively normal life despite the diagnosis.

Prevention

Because copper storage hepatopathy is genetic, the focus of prevention is on responsible breeding practices. Breeders should conduct genetic tests to identify affected dogs and exclude them from breeding. This can help reduce the spread of the disease in the population.

For dogs that already have a genetic predisposition, controlling copper intake is crucial. A low-copper diet can help prevent the accumulation of copper in the liver. Veterinarians can recommend special diets tailored to the dog’s needs.

Regular veterinary examinations are important to detect early signs of the disease. Blood tests and, if necessary, liver biopsies can help monitor liver function and intervene early if abnormalities are detected.

Overall, prevention requires a holistic approach that considers both genetic and environmental factors. By understanding the disease and working with a veterinarian, dog owners can help minimize the risk of developing copper storage hepatopathy.

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