Focal segmental glomerulosclerosis (FSGS) in adults with nephrotic range proteinuria. FSGS accounts for about 15% to 20% of the nephrotic syndrome in older children as well. The frequency of FSGS in a given population of patients with the nephrotic syndrome is influenced by the racial composition of the population, because FSGS is more common in blacks than whites.
Children and young adults with FSGS nephrotic syndrome have large amounts of protein in the urine (proteinuria), which is the result of tissue degeneration and scarring that occurs in the kidney’s glomeruli, tiny clusters of looping blood vessels that filter the blood. The degeneration and scarring are characteristically limited to one part of the glomerulus and to a minority of glomeruli in the affected region. Non-selective proteinuria is both a valuable surrogate marker for measuring the severity of injury to the glomeruli and a risk factor for progressive loss of kidney function.
Symptoms of nephrotic syndrome include retention of fluid in body tissues (edema), retention of fluid in the abdominal cavity (ascites), a high level of blood lipids (hyperlipidemia), and formation of blood clots (thromboemboli). Most pediatric patients have renal biopsy findings consistent with the diagnosis of minimal change disease. Typically, these patients will have remission of clinical symptoms after treatment with a course of high-dose prednisone, and they rarely develop progressive disease that leads to end-stage renal disease (ESRD) and kidney failure.
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