Sonographic Characteristic of Kidneys in Children with Autosomal Dominant Polycystic Kid-ney Disease (Correlation with Clinical and Laboratory Findings)

Overview

In a sonographic and clinical study the authors evaluated 71 children (29 boys and 42 girls), incl. 70 witha positive family history of autosomal dominant polycystic kidney disease (ADPKD). In 61 molecular geneticexamination confirmed locus PDK1. The average size of the kidneys in children with ADPKD was significantlylarger than in controls. The right kidney was enlarged as compared with normal in 38%, the left one in 23%. Thevolume was enlarged on the right in 23%, on the left in 31%. Pathological enlargement of the kidney (correctedfor age) was accentuated with advancing age. Cysts were found in 95.8% of the children, in 66% of the groupbilateral a total of more than 10 cysts were found, which is a major affection. In case of a larger number of cyststhe echogenicity of the parenchyma was also increased. Doppler examination of the group did not reveal anypathological findings. A significant correlation was found between the number of cysts and the length of the kidneyand the systolic and diastolic blood pressure. The authors did not provide evidence that the number of cysts hasan 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 onlyin adult age, it is possible to detect already in children surprisingly extensive morphological renal changes and insome cases significant proteinuria, hypertension and impaired concentrating capacities are present.

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