Quantification and longitudinal trends of kidney, renal cyst, and renal parenchyma volumes in autosomal dominant polycystic kidney disease.

ABSTRACT.: The aims of this study were to assess the accuracy and reproducibility of volumetric determinations of total kidney, renal cyst, and renal parenchymal volumes, using fast electron-beam computerized tomography scanning, and to determine the rate of change of these volumes. Nine patients with autosomal dominant polycystic kidney disease (ADPKD) and serum creatinine < or = 1.3 mg/dl and/or an initial iothalamate clearance > or = to 60 ml/min per 1.73 m(2) were imaged weekly over a 3-wk period (total of 3 times). Approximately 8 yr later, they returned for follow-up studies. The kidney volume estimation technique involved a manual segmentation (perimeter drawing) of the kidneys and a semiautomatic threshold approach, using a histogram analysis of the peak densities of renal parenchyma and renal cysts. At entry, total kidney and renal cyst volumes correlated positively with age, while renal parenchymal volumes and GFR correlated negatively with age. The average coefficient of variation values for the three initial consecutive measurements of total kidney, renal cyst (actual and as a percent of total volume), and renal parenchymal volume were 3.4, 7.2, 5.3, and 5.6%, respectively. During the 8 yr of follow-up, total kidney and renal cyst volumes increased, while renal parenchymal volumes and GFR declined. The rate of increase in total kidney and renal cyst volumes varied markedly from patient to patient. There was a significant correlation between rate of increase in renal cyst volume and the rate of decline in GFR. The patients with an initial urine protein/osmolality ratio >0.13 mg/L per mosmol per kg had a significantly higher increase in renal volume and decline in GFR than those with a lower ratio. In summary, the results of this pilot study suggest that: (1) electron-beam computerized tomography is capable of measuring total kidney, renal cyst, and renal parenchymal volumes reproducibly; (2) total kidney and renal cyst volumes increase, while parenchymal volumes decrease with time; (3) the increase in cyst volume correlates best with the decline in renal function; and (4) renal volumes appear to be good surrogate markers for disease progression in ADPKD.