Abdominal sonographic study of autosomal dominant polycystic kidney disease

Purpose. The purpose of this study was to determine whether kidney size in patients who have autosomal dominant polycystic kidney disease (ADPKD) is r elated to renal function, hypertension, or extrarenal manifestations of the disease and to sonographically evaluate the abdominal manifestations of AD PKD. Methods. Between 1994 and 1998, 400 individuals from 85 families with a his tory of ADPKD were examined. There were 213 persons with ADPKD and 187 unaf fected 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 ph ysical examination, measured the arterial blood pressure and serum creatini ne levels, and performed abdominal sonography on each subject. The sonograp hic features that were studied were renal length and the presence and numbe r of cysts on the kidneys, liver, and pancreas. Results. There was a relationship between kidney size and age (p < 0.05), k idney size and renal function (p < 0.001), and kidney size and hypertension (p < 0.001). The overall prevalence of hepatic cysts in patients with ADPK D was 67%, and the prevalence increased with age. The presence of hepatic c ysts was related to the severity of renal disease. Females had more severe polycystic liver disease, and massive polycystic liver disease (ie, hepatom egaly with innumerable cysts) was seen only in females. The prevalence of p ancreatic cysts in the 187 persons in whom the pancreas was well evaluated sonographically was 5%. Conclusions. Kidney size in patients with ADPKD is related to renal functio n, hypertension, and extrarenal involvement and can be used to predict the outcome of the disease. Hepatic cysts are very common in patients with ADPK D and are related to age and renal function; pancreatic cysts are infrequen t in these patients. (C) 2000 John Wiley & Sons, Inc.