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.
Salame
Hanna
H
Department of Pediatric Imaging, University Hospital Queen Fabiola, Avenue J.J. Crocq 15, 1020 Brussels, Belgium. hanna.salame@skynet.be
Damry
Nash
N
Vandenhoudt
Katt
K
Hall
Michèle
M
Avni
Fred E
FE
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