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Focal segmental glomerulosclerosis
Definition
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.
Alternative Names
Segmental glomerulosclerosis; Focal sclerosis with hyalinosis
Causes
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:
- Heroin use
- HIV
- Inherited genetic problems
- Obesity
- Reflux nephropathy (a condition in which urine flows backward from the bladder to the kidney)
- Sickle cell disease
Symptoms
- Foamy urine (from excess protein in the urine)
- Poor appetite
- Swelling, called generalized edema, from fluids held in the body
- Weight gain
Exams 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:
- Kidney biopsy
- Kidney function tests (blood and urine)
- Urinalysis
- Urine microscopy
- Urine protein
Treatment
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.
See also:
In general, treatments may include:
- Antibiotics to control infections
- Anti-inflammatory medicines to reduce the immune response
- Dialysis
- 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
Outlook (Prognosis)
More than half of those with focal or segmental glomerulosclerosis develop chronic kidney failure within 10 years.
Possible Complications
- Chronic kidney failure
- End-stage kidney disease
- Infection
- Malnutrition
- Nephrotic syndrome
When to Contact a Medical Professional
You should call your doctor if you develop symptoms of this condition, especially if there is fever, pain with urination, or decreased urine output.
Prevention
No prevention is known.
References
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.
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.
