Polycystic kidney and liver disease in two related West Highland White Terrier litters

Polycystic kidney and liver disease was present in four of six female and three of five male offspring born in two matings between the same pair of West Highland White Terriers. Clinical signs were apparent and serum biochemistry analysis consistent with liver failure was evident by 5 weeks of age. Affected pups were euthanatized because of their disease. Renal cysts were confirmed to be of collecting duct origin by Dolichos bifluros agglutinin lectin histochemistry, and hepatic cysts were of biliary origin. The clinically unaffected parents were related through multiple common ancestors, and there were no reports of similar disease in related dogs. An autosomal recessive mode of inheritance is therefore suggested. This is the first report of polycystic kidney and liver disease in the West Highland White Terrier. The features of the disease in these pups are similar to those of autosomal recessive polycystic kidney disease (ARPKD) in humans. The West Highland White Terrier may therefore be a potential animal model for ARPKD.