FIGURE 3-11 (see Color Plate)

Finnish type of congenital nephrotic syndrome. Several disorders are responsible for nephrotic syndrome within the first few months to first year of life. The most common and important of these is known as congenital nephrotic syndrome of Finnish type because the initial descriptions emphasized the more common occurrence in Finnish families. This nephrotic syndrome is an inherited disorder in which infants exhibit massive proteinuria shortly after birth; typically, the placenta is enlarged. This disorder can be diagnosed in utero; increased a-fetoprotein levels in amni-otic fluid is a common feature. A, The microscopic appearance of the kidneys is varied. Some glomeruli are small and infantile without other alterations, whereas others are enlarged, more mature, and have diffuse mesangial hypercellularity. Because of the massive proteinuria, some tubules are microcystically dilated, a finding responsible for the older term for this disorder, microcystic disease. Because this syndrome is primarily a glomerulopathy, the tubular abnormalities are a secondary process and should not be used to designate the name of the disease. B, On electron microscopy, complete effacement of the foot processes of visceral epithelial cells is observed.

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