The contribution of ultrasound for the differential diagnosis of congenital and infantile nephrotic syndrome.
The aim of this study was to determine whether high-resolution ultrasound is able to differentiate between the various diseases associated with nephrotic syndrome (NS). We reviewed the US features of 15 patients less than 1 year presenting a NS whose exact type was defined by pathology nephrotic syndrome of Finnish type (NSFT, n=2); focal and segmental hyalinosis (FSH, n=3); minimal-change glomerular disease (MCGD, n=2); neonatal glomerulonephritis (n=1), and diffuse mesangial sclerosis (DMS, n=7). The US features studied included the size of the kidneys, cortical echogenicity, cortico-medullary differentiation (CMD), and borders. The images were reviewed on hard copies by two observers unaware of the final diagnosis. In each case a diagnosis was proposed based on the reading of the US features. Six patients with DMS displayed a peculiar US pattern: mild increase of renal size; and inhomogeneous (patchwork-like) parenchymal hyperechogenicity that included areas of the cortex and medulla. The NSFT and neonatal glomerulonephritis displayed some of the same US features: increased kidney size (+2 SD) and had homogeneous cortical hyperechogenity with persistent cortico-medullar differentiation. The kidneys in the 3 patients with SFH were sonographically normal (n=1) or displayed a mild cortical hyperechogenicity (n=2). Inhomogeneous parenchymal hyperechogenicity involving only segments of the cortex and medulla seems to be a specific US pattern for DMS. Ultrasound is less specific for the other types of CNS.
Department of Pediatric Imaging, University Hospital Queen Fabiola, Avenue J.J. Crocq 15, 1020 Brussels, Belgium. hanna.salame@skynet.be
Hanna
H
Salame
Nash
N
Damry
Katt
K
Vandenhoudt
Michèle
M
Hall
Fred E
FE
Avni
D003937:Diagnosis, Differential; D005260:Female; D005920:Glomerular Mesangium / Q000473:pathology; D005921:Glomerulonephritis / Q000000981:diagnostic imaging* / Q000473:pathology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009404:Nephrotic Syndrome / Q000151:congenital* / Q000000981:diagnostic imaging* / Q000473:pathology; D012598:Sclerosis; D012680:Sensitivity and Specificity; D014463:Ultrasonography