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Nephrotic Syndrome

Robert C. Kelsch MD1
Aileen B. Sedman MD2
1 Professor of Pediatrics and Communicable Diseases, University of Michigan Medical Center, Ann Arbor, MI.
2 Associate Professor of Pediatrics and Communicable Diseases, University of Michigan Medical Center, Ann Arbor, MI.

Renal disease of glomerular origin has three major clinical presentations: asymptomatic hematuria and/or proteinuria; acute nephritic syndrome, characterized by the sudden onset of hematuria, edema, hypertension, and azotemia; and nephrotic syndrome (NS), characterized by the insidious onset of edema with massive proteinuria and hypoalbuminemia. Hypercholesterolemia is considered a traditional part of the nephrotic complex, but its presence is not of diagnostic value.

NS may be primary or secondary. The term "primary" indicates that the disease is limited to the kidney and is of unknown etiology. Secondary NS is present when the disease has extrarenal manifestations not dependent on the renal abnormality or has a specific etiology. Table 1 provides a general classification with some prominent or typical examples in each category. This review will focus on primary NS and nephroticrange proteinuria in children. Patients with primary NS may have a variety of histopathologic descriptions, the most common of which are listed in Table 2. It is important, however, to keep in mind that different patients having renal diseases with identical etiology, histology, and therapeutic implications initially may present with any of the three major clinical patterns.

Epidemiology

The annual incidence of NS during the middle of the 20th century in western New York State was 4.4 cases per 100,000 children between 1 and 6 years of age and 2.0 cases per 100,000 children between 1 and 16 years of age.




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