Polycystic kidney and liver disease and XXXX changes in the @entity43048 @entity19 .

@entity8 @entity19 @entity384 @entity43048 @entity2819 @entity99
@entity19 homozygous for the mutation @entity43048 develop a lethal @entity8 ( @entity8 ) and have abnormal elevations of @entity2819 in the postnatal period. We examined the development of these elevated glucocorticoid levels in early life. The histological progression of a cystic lesion of the biliary ducts in older heterozygotes was also studied. Normal @entity19 had low (less than 85 nmol/liter) or undetectable (less than 36 nmol/liter) levels from the sixth to twelfth day of life. @entity2819 levels were significantly higher in all homozygote ( @entity43048 / @entity43048 ) and some of the normal sibs, who were presumed to be heterozygote ( @entity43048 /+) @entity19 compared to age-matched controls from day seven to 12. @entity2819 levels were similar from the fifteenth day. A significant proportion of adult obligate heterozygotes from breeding pairs were found to have focal areas of @entity384 of the biliary ducts, in the absence of any @entity8 . The incidence of this lesion increased with age (10% at 150 days, 23% at 300 days, 29% at 450 days, 65% at greater than 450 days of age). The livers of homozygote @entity19 dying in infancy, and control adult @entity19 of the C57BL/6J background strain were free of cysts. The finding of @entity99 is reminiscent of adult type @entity8 . The @entity43048 model thus supports the concept that the different forms of @entity8 may represent varying expression of a similar gene complex.
