Ten-year clinical and ultrasonographic follow-up of siblings from families with autosomal dominant polycystic kidney disease.

We conducted a ten-year clinical and ultrasound follow-up study of 120 siblings with ADPKD (68 men and 52 women, aged 19-40). 40 subjects had polycystic kidney disease. During the study period, the number and size of the cysts increased. Symptoms and signs also changed: at baseline 51% of the subjects were asymptomatic dropping subsequently to 2%. Initially, 32 subjects had 1-5 cysts in one or both kidneys and they were classified as suspected of having ADPKD. Significant changes were found in this group at the end of the follow-up. In 12 of them (37.50%) subsequent ultrasonograms revealed an increase in the number and size of the cysts--i.e. evolution towards ADPKD. None of the subjects in this group had a decrease in the number of cysts. In the control group, three had multiple cysts but most subjects were ultrasonographically negative for polycystic kidney disease. In conclusion, the authors recommend a clinical and ultrasonographic long-term follow-up of subjects at risk for ADPKD which should allow early diagnosis as well as prevention of the complications which result in chronic renal failure.