The contribution of ultrasound for the differential diagnosis of congenital and infantile nephrotic syndrome

Abstract

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.

Congenital Nephrotic Syndrome of the Finnish Type: A Greek Case Report

“…Furthermore, hyperechogenic kidneys are a classic but non-specific finding, in differential diagnosis among other disorders. Conversely, the detection of hyperechogenic, enlarged kidneys in combination with a thick placenta and intrauterine growth retardation should suggest but not confirm the diagnosis of CNF [1] [7] [10].…”

Ultrasonographic Findings in Cairn Terriers With Preclinical Renal Dysplasia

“…Medullary hyperechogenicity has been described in human neonates and is considered a normal variant if it disappears within 10 days 16 . Other conditions in which hyperechogenicity can be seen in the medulla include diffuse glomerulosclerosis and hypernatremic dehydration where the hyperechoic foci are thought to be crystal depositions 17,18 …”

Infant with gross hematuria and nephrotic syndrome: answers

“…DMS is usually diagnosed in the first 2 years of life, and progresses to end-stage renal failure in a mean of 3 years [12]. Renal ultrasound (US) can help differentiate DMS from other forms of NS in the first year of life, with DMS typically associated with mild increase in kidney size and inhomogeneous parenchymal hyperechogenicity in the cortex and medulla [13]. In the early stages, glomeruli show fibrillar increase in the mesangial matrix, no increase in mesangial cells, and widespread hypertrophy of podocytes [4].…”