In a sonographic and clinical study the authors evaluated 71 children (29 boys and 42 girls), incl. 70 with a positive family history of autosomal dominant polycystic kidney disease (ADPKD). In 61 molecular genetic examination confirmed locus PDK1. The average size of the kidneys in children with ADPKD was significantly larger than in controls. The right kidney was enlarged as compared with normal in 38%, the left one in 23%. The volume was enlarged on the right in 23%, on the left in 31%. Pathological enlargement of the kidney (corrected for age) was accentuated with advancing age. Cysts were found in 95.8% of the children, in 66% of the group bilateral a total of more than 10 cysts were found, which is a major affection. In case of a larger number of cysts the echogenicity of the parenchyma was also increased. Doppler examination of the group did not reveal any pathological findings. A significant correlation was found between the number of cysts and the length of the kidney and the systolic and diastolic blood pressure. The authors did not provide evidence that the number of cysts has an adverse effect on proteinuria and the concentrating capacity. The authors draw attention to the fact that although ADPKD is associated with a more marked pathology only in adult age, it is possible to detect already in children surprisingly extensive morphological renal changes and in some cases significant proteinuria, hypertension and impaired concentrating capacities are present.

        Key words: autosomal dominant polycystic kidney disease, early presence of cysts and enlargement of the kidneys, proteinuria, hypertension