Jg. Blickman et al., AUTOSOMAL RECESSIVE POLYCYSTIC KIDNEY-DISEASE - LONG-TERM SONOGRAPHICFINDINGS IN PATIENTS SURVIVING THE NEONATAL-PERIOD, American journal of roentgenology, 164(5), 1995, pp. 1247-1250
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.