Abstract

Factors affecting the progression of autosomal dominant adult type polycystic kidney disease were analysed in 27 cases. The patients ages ranged from 10 to 74 (mean 44) years old and the serum creatinine values were within the normal limits except two cases, in which the values were 2.4 mg/dl and 2.1. They were followed for from 2 years to 12 years (mean 5.6 years). During the followup period, 6 cases showed elevation of the serum creatinine values and hemodialysis was necessary in 4 cases. There was a tendency of higher morbidity rate of hypertension, proteinuria, hematuria and pyuria in the cases with decreased renal function. These factors may have participated in the progression of polycystic kidney disease. Cystic fluid analysis was performed by percutaneous puncture of more than hundred cysts in 27 cases. The results showed that the cystic fluid components of most cysts of the well functioning kidneys were similar to those of serum values: so-called proximal cysts. On the other hand, in the cases with decreased renal function, there were many cysts with lower sodium concentration and higher creatinine values: so-called distal cysts. The results suggest that the existence of so-called distal cysts may indicate poorer prognosis. DMSA renoscintigraphy was useful for followup polycystic kidney patients because of the uptake of the radionuclide was decreased before rising the serum creatinine value. In 6 cases, the cysts were instilled with 95% ethanol. Followup ultrasonography and DMSA renoscintigraphy revealed a marked reduction of the cystic size and an improvement of DMSA uptake.(ABSTRACT TRUNCATED AT 250 WORDS)

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