Abstract
Extract A lethal congenital polycystic kidney disease (PCKD) has been known in lambs in New Zealand for more than 50 years and assumed to be genetic in origin. We have studied this in lambs from an experimental mating in a flock experiencing an outbreak the previous year. Two Perendale rams identified by DNA profiling (SignaGen) as sires of PCKD lambs were each mated to the other's daughters. From 71 such matings, 10 ewes gave birth to 15 PCKD lambs of both sexes. The results are in accord with those expected for a recessive autosomal trait. All lambs were dead or died shortly after birth. Their abdomens were distended by enlarged kidneys 40–140 mm in length. Their cut surface showed a multitude of cystic spaces 1–5 mm in diameter in both the cortex and medulla. Livers of affected lambs showed complex tubular and cystic patterns of bile ducts containing bile, and supported by an excess of fibrous tissue. Cystic changes also occurred in the pancreas and salivary glands.
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