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Focal segmental glomerulosclerosis Causes and Risks:
The cause of focal segmental glomerulosclerosis is usually unknown. A minority of cases result from reflux nephropathy . Some (but not all) of the glomeruli become scarred. It affects about 1 out of 10,000 people, both children and adults. Men are affected slightly more often than women. It can occur in children.
The main result of focal segmental glomerulosclerosis is nephrotic syndrome . It causes about 10 to 15% of cases of nephrotic syndrome. Protein is persistently excreted in the urine, especially urine albumin , . Most cases will progress to chronic renal failure .
Although the disorder seems to be immune-system related, response to corticosteroid or immunosuppressive medications is inconsistent.
Prevention:
No prevention is known.
Symptoms:
Signs and Tests:
The examination is nonspecific other than for signs of edema and elevated blood pressure . Signs of chronic renal failure and associated fluid overload may develop as the disorder progresses.
Tests may include:
Treatment:
The goal of treatment is control of the symptoms associated with nephrotic syndrome and chronic renal failure . Treatment may be chronic and lifelong.
Corticosteroids and immunosuppressive medications may be prescribed to reduce the immune response .
Antihypertensive and diuretic medications may help control symptoms such as high blood pressure and edema . Antibiotics may be needed to control infections.
The treatment of high blood cholesterol and triglyceride levels, which are also common with this disorder, may be recommended to reduce the development of atherosclerosis . Dietary limitation of cholesterol and saturated fats may be of only limited benefit as the high levels seem to result from overproduction of cholesterol and triglycerides by the liver rather than the excessive intake of fats. Medications to reduce cholesterol and triglycerides may be recommended.
High-protein diets are of debatable value. In many patients, reducing the amount of protein in diet produces a decrease in urine protein . In most cases, a moderate-protein diet (1 gram of protein per kilogram of body weight per day) is usually recommended. In cases in which renal failure is present, a low-protein diet may be preferred.
The sodium (salt) in the diet and/or fluids may be restricted to help control swelling (see also renal disease - diet ). Vitamin D may need to be replaced if nephrotic syndrome is chronic and unresponsive to therapy.
Dialysis or kidney transplantation may be necessary to control renal failure.
Prognosis:
Over 50% of focal or segmental glomerulosclerosis cases develop chronic renal failure within 10 years.
Complications:
Call Your Healthcare Provider:
Call your health care provider if the symptoms indicating focal segmental glomerulosclerosis are present.
Call your health care provider if new symptoms develop, especially fever , pain with urination , or decreased urine output .
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