Chronic Hepatitis of Bedlington Terriers

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Chronic hepatitis of Bedlington Terriers is an autosomal recessive genetic disease characterized by hepatic copper toxicosis[1].

The disease results from progressive copper accumulation due to abnormal copper metabolism. The accumulated copper results in progressive liver disease, causing oxidative injury to hepatocyte mitochondria and leads to chronic hepatitis and cirrhosis.

Affected dogs may have hepatic copper levels as high as 12,000 µg/g (normal = <400 µg/g).

Clinical symptoms depend on severity and in severe cases, anorexia, vomiting, icterus, hepatomegaly, hemosiderosis are common[2]. Secondary immune-mediated hemolytic anemia may be a sequel.

In less severe cases, weight loss, ascites and neurological disease may predominate, particularly mental depression, seizures and ataxia.

Mildly affected dogs are often asymptomatic.

Diagnosis requires histological examination of liver biopsies, which often shows varying degrees of hepatic necrosis and large numbers of the copper-positive, lipofuscin-containing lysosomes in centrilobular and periportal hepatocytes[3]. The severity of histopathological changes increase with age.

A differential diagnosis would include infectious canine hepatitis, portosystemic shunt, hepatic lymphoma and bacterial meningoencephalitis.

Treatment of this condition primarily involves the use of chelation drugs such as d-penicillamine (10 - 15 mg/kg orally twice daily before meals), trientine hydrochloride (10 - 15 mg/kg orally twice daily) and vitamin E (400-600 IU once daily).

Foods high in copper, including red meat, liver, shellfish, and fish, should be avoided. Chicken, dairy products, and rice have a low copper content.

Prognosis is guarded if the dog is showing clinical signs.

Screening of potential carriers should be done after 1 year for quantitative copper analysis[4].

References

  1. Ubbink GJ et al (1998) Cluster analysis of the genetic heterogeneity and disease distributions in purebred dog populations. Vet Rec 142(9):209-213
  2. Fuentealba IC & Aburto EM (2003) Animal models of copper-associated liver disease. Comp Hepatol 2(1):5
  3. Hultgren BD et al (1986) Inherited, chronic, progressive hepatic degeneration in Bedlington terriers with increased liver copper concentrations: clinical and pathologic observations and comparison with other copper-associated liver diseases. Am J Vet Res 47(2):365-377
  4. Merck Vet Manual