Gabow P A, Johnson A M, Kaehny W D, Manco-Johnson M L, Duley I T, Everson G T
Department of Medicine, University of Colorado Health Sciences Center, Denver 80262.
Hepatology. 1990 Jun;11(6):1033-7. doi: 10.1002/hep.1840110619.
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)
肝囊肿是常染色体显性遗传性多囊肾病的主要表现。本研究对239例常染色体显性遗传性多囊肾病患者和189名未患病的家庭成员进行了检查，以确定影响肝囊肿的存在及严重程度的因素。患有肝囊肿的常染色体显性遗传性多囊肾病患者比无肝囊肿的患者年龄更大（44.6±1.1岁对32.9±1.1岁；p<0.0001）。肝囊肿数量随年龄增加（r=0.43；p<0.0001）。女性比男性更易患巨大肝囊肿疾病（囊肿超过15个）（p<0.04）。女性最大囊肿尺寸也更大（4.2±0.4厘米对2.7±0.3厘米；p<0.004）。患有肝囊肿的女性更有可能怀孕（p<0.001）且怀孕次数更多（2.9±0.3次对1.6±0.2次；p<0.0009）。患有肝囊肿的常染色体显性遗传性多囊肾病患者的肾脏体积更大（p<0.0001）、囊肿数量更多（p<0.004）、囊肿实质百分比更高（p<0.001）、主要囊肿尺寸更大（p<0.001），而肌酐清除率更低（64.5±3.1毫升/分钟/1.73平方米对94.5±3.4毫升/分钟/1.73平方米；p<0.001）。通过逻辑回归分析，肝囊肿的发生频率与年龄增加、肾囊肿疾病严重程度增加及肌酐清除率降低有关。肝囊肿的数量和大小与怀孕、女性性别、年龄增加及肾脏病变严重程度相关。（摘要截短于250词）
