Nicolau C, Torra R, Bianchi L, Vilana R, Gilabert R, Darnell A, Brú C
Imaging Diagnosis Center, Ultrasound Unit, Hospital Clínic, University of Barcelona, Spain.
J Clin Ultrasound. 2000 Jul-Aug;28(6):277-82. doi: 10.1002/1097-0096(200007/08)28:6<277::aid-jcu2>3.0.co;2-l.
The purpose of this study was to determine whether kidney size in patients who have autosomal dominant polycystic kidney disease (ADPKD) is related to renal function, hypertension, or extrarenal manifestations of the disease and to sonographically evaluate the abdominal manifestations of ADPKD.
Between 1994 and 1998, 400 individuals from 85 families with a history of ADPKD were examined. There were 213 persons with ADPKD and 187 unaffected family members; there were 182 males and 218 females, 1-82 years old (mean, 39.3 years). We obtained a complete medical history, performed a physical examination, measured the arterial blood pressure and serum creatinine levels, and performed abdominal sonography on each subject. The sonographic features that were studied were renal length and the presence and number of cysts on the kidneys, liver, and pancreas.
There was a relationship between kidney size and age (p < 0.05), kidney size and renal function (p < 0.001), and kidney size and hypertension (p < 0.001). The overall prevalence of hepatic cysts in patients with ADPKD was 67%, and the prevalence increased with age. The presence of hepatic cysts was related to the severity of renal disease. Females had more severe polycystic liver disease, and massive polycystic liver disease (ie, hepatomegaly with innumerable cysts) was seen only in females. The prevalence of pancreatic cysts in the 187 persons in whom the pancreas was well evaluated sonographically was 5%.
Kidney size in patients with ADPKD is related to renal function, hypertension, and extrarenal involvement and can be used to predict the outcome of the disease. Hepatic cysts are very common in patients with ADPKD and are related to age and renal function; pancreatic cysts are infrequent in these patients.
本研究旨在确定常染色体显性多囊肾病（ADPKD）患者的肾脏大小是否与肾功能、高血压或该疾病的肾外表现相关，并通过超声检查评估ADPKD的腹部表现。
1994年至1998年间，对85个有ADPKD病史家庭的400人进行了检查。其中有213例ADPKD患者和187名未受影响的家庭成员；有182名男性和218名女性，年龄在1至82岁之间（平均39.3岁）。我们获取了完整的病史，进行了体格检查，测量了动脉血压和血清肌酐水平，并对每位受试者进行了腹部超声检查。所研究的超声特征包括肾脏长度以及肾脏、肝脏和胰腺上囊肿的存在情况和数量。
肾脏大小与年龄（p < 0.05）、肾脏大小与肾功能（p < 0.001）以及肾脏大小与高血压（p < 0.001）之间存在关联。ADPKD患者肝囊肿的总体患病率为67%，且患病率随年龄增加而升高。肝囊肿的存在与肾脏疾病的严重程度相关。女性的多囊肝病更为严重，仅在女性中可见巨大多囊肝病（即肝脏肿大伴无数囊肿）。在超声检查对胰腺评估良好的187人中，胰腺囊肿的患病率为5%。
ADPKD患者的肾脏大小与肾功能、高血压及肾外受累情况相关，可用于预测疾病的预后。肝囊肿在ADPKD患者中非常常见，且与年龄和肾功能相关；胰腺囊肿在这些患者中较少见。
