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Virtual Pediatric Hospital: Paediapaedia: Nephrotic Syndrome Paediapaedia: Genitourinary Diseases

Nephrotic Syndrome

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
Clinically they initially present with edema and are found to have proteinuria, hypoproteinemia, and hyperlipidemia. The peak age of incidence is 3-4 years old.

Etiology/Pathophysiology:
Site of disease is the glomerulus. Over time you progressively see glomerular sclerosis, renal fibrosis, atrophy and kidney failure. The causes of nephrosis are: (1) idiopathic - most common, (2) primary renal disease - glomerulonephritis, renal vein thrombosis, (3) systemic disease - systemic lupus erythematosus, diabetes mellitus, amyloidosis, and (4) infantile - microcystic disease of the kidney.

Pathology:
Not applicable

Imaging Findings:
Acutely see bilateral nephromegaly with thinning of the calyces and infundibulum due to the nephromegaly with the renal function being normal. Later on ultrasound you will see small, shrunken, echogenic kidneys.

DDX:
Diffuse nephromegaly

Bilateral shrunken kidneys

References:
See References Chapter.

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