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Wednesday, july 24, 2002

What is FSGS?
FSGS is a disease of the kidney. It can only be
FSGS usually causes protein in the urine diagnosed by a kidney biopsy. Its name describes (proteinuria). This may be mild enough that it Focal: Not all of the glomeruli (filtering units)
are involved; scarring in even 1 can make the produces swelling from the nephrotic syndrome. Segmental: Scarring involves only a portion of
nothing that could have prevented it. While it the filtering unit. If the whole glomerulus were may respond to treatment, many cases do not. About half of children with FSGS will develop Glomerulusclerosis: This is the medical term
permanent kidney failure within 5 years of their for scarring of the filtration units (glomeruli) of FSGS is more common now than it was 30 years Other findings may be present on the kidney ago, perhaps because of rising rates of childhood biopsy that will help predict how your kidneys are going to do.

How do you diagnose FSGS?
FSGS is diagnosed by kidney biopsy in patients
Other patients may have gene mutations that with unusual forms of nephrotic syndrome or contribute to the disorder. You may be asked to heavy proteinuria with no obvious cause. have blood tests to examine these possibilities. Some patients with FSGS have a protein in their blood that causes the kidney to leak protein.

How do you treat HUS?
The nephrotic syndrome can be treated with
mycophenolate (Cellcept™), another drug that suppresses the immune system. Another drug, hydrochlorothiazide (Diuril) or furosemide rituximab (Rituxan™), can be given by IV. It has (Lasix) to control swelling. If very severe, a produced long-lasting remissions in patients with protein called albumin may be given through the FSGS, even when the disease occurs after vein with furosemide to remove swelling. While transplant. This medication may not be covered these may control the swelling, they do not treat In about half of all patients, none of these Some cases of FSGS will resolve with treatment treatments work to cure the FSGS. In this case, with oral steroids (drugs such as prednisone). blood pressure medications are very important to Often this does not work and other agents are keep the kidneys working as long as possible. used including chemotherapy drugs such as Other drugs may be needed to control swelling drugs such as cyclosporine (Neoral™) or If the kidneys do fail, a kidney transplant can be tacrolimus (Prograf™). Control of high blood done. Unfortunately, FSGS may affect the pressure is also very important in preventing transplanted kidney and cause it to fail. This does kidney failure. Drugs such as ACE inhibitors or not happen every time, and when it does there others may be used to control this complication. are therapies that may help delay or reverse Some newer drugs may produce remission in patients with FSGS. These include


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