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 d
isease. 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 simil
ar 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.