AUTOSOMAL RECESSIVE POLYCYSTIC KIDNEY-DISEASE - LONG-TERM SONOGRAPHICFINDINGS IN PATIENTS SURVIVING THE NEONATAL-PERIOD

OBJECTIVE. We studied the sonographic findings and the changes in rena l function seen on long-term follow-up of children who had the initial diagnosis of autosomal recessive polycystic kidney disease made in th e neonatal period. MATERIALS AND METHODS. The case records and sonogra ms of 14 children with biopsy evidence of autosomal recessive polycyst ic kidney disease were evaluated. Nine children who survived the neona tal period were followed up for a mean of 13 years (range, 5-19 years) after diagnosis and form the basis of this study. Serial changes in r enal size, echogenicity, and function were assessed sonographically, T he imaging findings were compared with those described in published re ports. RESULTS. The sonographic findings showed that five of the nine children had a decrease in renal size, and three had stable renal size over a minimum follow-up period of 5 years, Only one of the nine surv ivors showed progressive increase in renal size, All had increased cor tical echogenicity and large kidneys. Three patients showed a subjecti ve change in renal echogenicity over time, A change in the echogenic p attern to one that resembles autosomal dominant polycystic kidney dise ase was noted with no evidence of increase in size of the kidneys, Non e of the surviving children had renal stones or massively enlarged kid neys, The renal function of seven of the nine survivors has remained s table with creatinine clearance nearly normal (>60 ml/min/1.73 m(2)), and there was no correlation between renal size and renal function. CO NCLUSION. In patients with autosomal recessive polycystic kidney disea se who survive the neonatal period, kidney size as seen on sonograms d oes not continue to increase despite the patients' linear growth and m aintained normal renal function, Rather, a decrease in kidney size and change in echogenicity occurs, producing a pattern that is similar to that seen on sonograms of patients with autosomal dominant polycystic kidney disease but without the marked increase in kidney size that oc curs in that entity, This changing cystic pattern on follow-up sonogra ms may be the reason that previous descriptions of the sonographic fin dings in cases of autosomal recessive polycystic kidney disease have v aried and why a decrease in size may not herald deteriorating renal fu nction.