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Abstract

Hepatic cysts are a major manifestation of autosomal dominant polycystic kidney disease. This study examined 239 autosomal dominant polycystic kidney disease patients and 189 unaffected family members to define the factors that influence the presence and severity of hepatic cysts. Autosomal dominant polycystic kidney disease patients with hepatic cysts were older than autosomal dominant polycystic kidney disease patients without such cysts (44.6 ± 1.1 yr vs. 32.9 ± 1.1 yr; p < 0.0001). The number of hepatic cysts increased with age (r = 0.43; p < 0.0001). Women were more likely to have massive hepatic cystic disease (> 15 cysts) than men (p < 0.04). Women also had larger maximal cyst men (p < 0.04). Women also had larger maximal cyst size (4.2 ± 0.4 cm vs. 2.7 ± 0.3 cm; p < 0.004). Women with hepatic cysts were more likely to have been pregnant (p < 0.001) and to have had more pregnancies (2.9 ± 0.3 pregnancies vs. 1.6 ± 0.2 pregnancies; p < 0.0009). Kidney volume (p < 0.0001), number of cysts (p < 0.004), percentage of cystic parenchyma (p < 0.001) and predominant cyst size (p < 0.001) were greater and creatinine clearance was lower (64.5 ± 3.1 ml/min/1.73 m2 vs. 94.5 ± 3.4 ml/min/1.73 m2; p < 0.001) in autosomal dominant polycystic kidney disease patients with hepatic cysts. By logistic regression, the frequency of hepatic cysts was related to increased age, increased severity of renal cystic disease and decreased creatinine clearance. Number and size of hepatic cysts correlated with the occurrence of pregnancy, female gender, increased age and severity of the renal lesion. In conclusion, the hepatic expression of the autosomal dominant polycystic kidney disease gene appears to be modulated by age, female gender, pregnancy, severity of the renal lesion and kidney function.(HEPATOLOGY 1990;11:1033-1037.).