We have evaluated a congenic strain of mice with congenital polycystic kidney disease in
which the disease process appears to closely resemble human infantile polycystic kidney
disease. Cysts formed first in the proximal tubules of the nephron and appeared, by light
microscopy, to be preceded by vacuolization of the cells. These spaces, as seen by electron
microscopy, occurred between adjacent cells. The pancreas was severely involved with
reduction of both exocrine and endocrine elements. Cyst formation in the liver was minimal …