Polycystic kidney: Pediatrician' s Point of view

Abstract

The mostly known cystic renal diseases are, with any doubt, autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD), even if “renal cysts” are often represented in very different clinical pictures with onset in paediatric age, especially in rare syndromic disease. In this paper, two main features concerning polycystic kidney disease in paediatric age have been treated. In the first part, we have considered compare and contrast of these two diseases with regard to incidence, inheritance, imaging and clinical signs, especially when ADPKD onsets very early and the differential diagnosis with ARPKD should be very difficult. In the second part, the incidence of hypertension in paediatric patients with ADPKD has been evaluated. Recently, it has been reported that the incidence of hypertension in paediatric patients with ADPKD is about 20%. Preliminary data from patients followed in Bambino Gesù Children's Hospital show that the incidence of hypertension is 19%, higher than currently accepted. To this end, we believe that new studies are needed to confirm this trend and to prevent the major hypertensive complications as left ventricular hypertrophy and hypertensive rethinopathy.