Renal Cystic Diseases In Children

Renal cystic diseases is a group of different diseases found in children quite often. Renal cysts is the common sign of these diseases, but they can be either of congenital, or genetic or aquired origin. In some cases it is only renal disease, in others - they are a symptom of multisystem pathology. The significant progress has recently been made in the understanding of the origin, mechanisms and diagnotics of renal cystic diseases. During the period 1967-1998 we observed renal cystic diseases in 47 patients aged from 1,7 to 16 years. In ten cases it was dysplastic renal disease, in eleven cases it was autosomal dominant polycystic kidney disease (ADPK) and in 16 patients it was autosomal recessive polycystic kidney disease (ARPK). The other patients had Fanconi nephronophtisis, renal cysts in tuberous sclerosis, a «sponge» kidney and the cystic diseаse which could not be differentiated. Diagnostics was made by means of ultrasound and radioraphy methods. In some cases renal biopsy was used. It was proved to be very important in cases of combination of dysplastic renal disease and glomerulonephritis wich was seen in some children. In this study we describe the typical clinical features of the dysplastic renal cystic disease, ADRK and ARPK. Their characteristic symptoms were mild or moderate proteinuria, renal tubular function’s impairment, physical retardation and osteoporosis. Arterial hypertension, anemia and polyuria were less common.

поликистоз почек, аутосомно-доминантная форма, аутосомно-рецессивная форма, кистозная дисплазия почек, фиброз печени

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