Risk factors for the development of hepatic cysts in autosomal dominant polycystic kidney disease.
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 less than 0.0001). The number of hepatic cysts increased with age (r = 0.43; p less than 0.0001). Women were more likely to have massive hepatic cystic disease (greater than 15 cysts) than men (p less than 0.04). Women also had larger maximal cyst size (4.2 +/- 0.4 cm vs. 2.7 +/- 0.3 cm; p less than 0.004). Women with hepatic cysts were more likely to have been pregnant (p less than 0.001) and to have had more pregnancies (2.9 +/- 0.3 pregnancies vs. 1.6 +/- 0.2 pregnancies; p less than 0.0009). Kidney volume (p less than 0.0001), number of cysts (p less than 0.004), percentage of cystic parenchyma (p less than 0.001) and predominant cyst size (p less than 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 less than 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.(ABSTRACT TRUNCATED AT 250 WORDS)
Gabow
P A
PA
Department of Medicine, University of Colorado Health Sciences Center, Denver 80262.
Johnson
A M
AM
Kaehny
W D
WD
Manco-Johnson
M L
ML
Duley
I T
IT
Everson
G T
GT
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D003560:Cysts / Q000453:epidemiology / Q000235:genetics*; D005260:Female; D005799:Genes, Dominant; D006801:Humans; D008107:Liver Diseases / Q000453:epidemiology / Q000235:genetics*; D008297:Male; D008875:Middle Aged; D007690:Polycystic Kidney Diseases / Q000453:epidemiology / Q000235:genetics*; D011247:Pregnancy; D011248:Pregnancy Complications; D012044:Regression Analysis; D012307:Risk Factors; D012737:Sex Factors