Copper-Storage Hepatopathy in Dogs

 

Copper storage hepatopathy is a condition caused by an abnormal accumulation of copper in the animal's liver, which leads to hepatitis and progressive damage and scarring of the liver (cirrhosis) over the long term. This condition is believed to be secondary to a primary disease, the result of genetic-based abnormal copper metabolism.

 

Bedlington Terriers, Doberman Pinschers, and Labrador Retrievers are dog breeds known to be susceptible to this type of liver disease. In the case of Doberman Pinschers, copper storage hepatopathy is more prevalent in females than males.

 

The condition or disease described in this medical article can affect both dogs and cats. If you would like to learn more about how this disease affects cats, please visit this page in the PetMD health library.

 

Symptoms and Types

 

Primary copper liver diseases (medically referred to as hepatopathies) generally fall into one of three categories:

 

1. Subclinical disease: a condition where the disease is present in the organ or body, but not detectable by abnormal signs or changes in the dog
2. Acute (sudden) disease which most frequently affects young dogs; associated with a condition that causes the death of liver tissue (hepatic necrosis)
3. Chronic progressive disease in which symptoms are observed in middle-aged and older dogs with chronic hepatitis, with damage and scarring of the liver (cirrhosis)

 

Conversely, secondary copper hepatopathies show symptoms of progressive signs of liver disease due to chronic hepatitis or progressive cirrhosis. Liver disease in which the flow of bile is slowed or stopped is known as cholestatic liver disease; the abnormal flow of bile results in secondary copper retention.

 

Both types may display symptoms in their acute or chronic forms; they are as follows:

 

Acute:

• Lethargy
• Anorexia
• Depression
• Vomiting
• Yellowish discoloration of skin and moist tissues (icterus or jaundice)
• Moist tissues of the body (mucous membranes) are pale due to low red blood cell count; simply referred to as anemia
• Dark urine due to the presence of bilirubin (bilirubinuria)
• Hemoglobin in the urine (hemoglobinuria)

Chronic signs:

• Lethargy
• Depression
• Anorexia
• Weight loss
• Vomiting
• Diarrhea
• Excessive thirst and urination (polydipsia and polyuria)
• Abdominal distention due to fluid build up in the abdomen (ascites)
• Yellowish discoloration of skin and moist tissues (icterus or jaundice),
• Spontaneous bleeding, black or tarry stools (melena)
• Nervous system dysfunction due to the liver being unable to break down ammonia in the body (hepatic encephalopathy)

 

Causes

 

It is important to note that dogs can be affected by copper storage hepatopathy at any age. Genetics, however, is the main contributing factor in contracting this liver disease. Here is some information that is known about the contributing genetic factors:

 

• An autosomal recessive trait in Bedlington terriers due to the lack of a specific gene (COMMD1) coding for a liver protein involved in the excretion of copper in the bile is confirmed
• At one time, possibly as many as two-thirds of Bedlington terriers either were carriers of the gene or were affected by the disease; with recent genetic screening, the incidence is now much lower
• The prevalence in certain lines of West Highland white terriers appears to be high, but the incidence in all West Highland White Terriers is low
• The mode of inheritance in West Highland White Terriers, Skye terriers, and other affected breeds is unknown
• A reported four to six percent of Doberman Pinschers may have chronic hepatitis, a sign of copper storage hepatopathy

 

Diagnosis

 

A complete blood profile will be conducted, including a chemical blood profile, a complete blood count, and a urinalysis. You will need to give a thorough history of your dog's health, including a history of its symptoms, and possible incidents that might have precipitated this condition. The history you provide may give your veterinarian clues as to whether the condition is of primary or secondary origin.

 

A tissue sample will then be taken from the dog's liver for laboratory analysis (biopsy), and ultrasound images will be taken of the abdominal area to examine the condition of the liver.

 

 

Treatment

 

Inpatient evaluation and treatment are needed for dogs with signs of liver failure. Treatment will be determined by the type of disease, whether it is acute or chronic hepatitis, or it is liver scarring/cirrhosis.

 

Dogs with liver failure will require inpatient care with fluids and electrolytes supplements.

 

Making modifications to the dog's diet and providing it with foods low in copper has proven to be effective in most cases. However, most commercially available diets contain excessive amounts of copper, so follow your veterinarian's instructions as to the specifically tailored for your dog. You should also avoid giving your dog mineral supplements containing copper. If required, your veterinarian may provide you with water-soluble vitamins.

 

In rarer cases, a surgical liver biopsy may be needed to screen dogs for copper-storage liver disease, and to monitor their response to treatment. Be aware that dogs with liver failure are surgical and anesthetic risks.

 

Living and Management

 

Following therapy (six months to one year), your dog should be re-biopsied to monitor the effectiveness of the therapy. Additionally, blood tests will be done every four to six months to monitor its liver enzyme levels. The veterinarian may also ask you to monitor your dog's body weight.

 

Prevention

 

If you are considering  purchasing a Bedlington Terrier, Labrador Retriever, or Doberman Pinscher, you should have the dog tested for the gene which causes this type liver disease. In the case of Bedlington Terriers, if it's liver copper concentration is less than 400 μ g/g DW at one year of age, it is unaffected. There are also liver registries avaialable for these breeds that indicate "clean" lineages, which diminish the porbability of receiving a diseased dog.