Focal segmental glomerulosclerosisDefinition:
Focal segmental glomerulosclerosis is scar tissue that forms in parts of the kidney called glomeruli. The glomeruli serve as filters, helping rid the body of unnecessary or harmful substances. Each kidney has thousands of glomeruli. One glomeruli is called a glomerulus.
"Focal" means that some of the glomeruli become scarred, while others remain normal. "Segmental" means that only part of an individual glomerulus is damaged.
Segmental glomerulosclerosis; Focal sclerosis with hyalinosis
Causes, incidence, and risk factors:
The cause of focal segmental glomerulosclerosis is usually unknown.
The condition affects both children and adults. Men and boys are affected slightly more often than women and girls, and it also occurs more often in African Americans. Focal segmental glomerulosclerosis causes about 20 - 25% of all cases of nephrotic syndrome .
Known causes include:
- Foamy urine (from excess protein in the urine)
- Poor appetite
- Swelling, called generalized edema , from fluids held in the body
- Weight gain
Signs and tests:
The health care provider will perform a physical examination. This may detect tissue swelling and high blood pressure. Signs of kidney (renal) failure and excess fluid may develop as the condition gets worse.
Tests may include:
Some patients will receive high doses of corticosteroids or a drug called cyclosporine to suppress their immune system.
The goal of treatment is to control the symptoms of nephrotic syndrome and prevent chronic kidney failure.
In general, treatments may include:
- Antibiotics to control infections
- Anti-inflammatory medicines to reduce the immune response
- Fluid restriction
- Kidney transplant
- Low-fat diet
- Low- or moderate-protein diet (1 gram of protein per kilogram of body weight per day)
- Medicines to treat high blood pressure, high blood cholesterol , and high triglyceride levels
- Salt-reduced diet
Vitamin D supplements
See also: Kidney disease - diet
More than half of those with focal or segmental glomerulosclerosis develop chronic kidney failure within 10 years.
No prevention is known.
Appel GB. Glomerular disorders and nephrotic syndromes. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 122.
Nachman PH, Jennette JC, Falk, RJ. Primary glomerular disease. In: Brenner BM, ed. Brenner and Rector's The Kidney. 8th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 30.
|Review Date: 6/8/2011|
Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Herbert Y Lin, MD, PhD, Nephrologist, Massachusetts General Hospital; Associate Professor of Medicine, Harvard Medical School. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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