Focal segmental glomerulosclerosis

Other Names: FSGS; Glomerulosclerosis, focal; Segmental glomerulosclerosis; Focal sclerosis with hyalinosis; Familial idiopathic nephrotic syndrome; Familial idiopathic steroid-resistant nephrotic syndrome

Focal segmental glomerulosclerosis (FSGS) is a type of kidney disorder. It is characterized by scar tissue that forms in some of the glomeruli in the kidney. The glomeruli serve as filters that help the body get rid of harmful substances. Each kidney has thousands of glomeruli. "Focal" means that some of the glomeruli become scarred. Others remain normal. "Segmental" means that only part of an individual glomerulus is damaged.

Cause

In many cases the cause of FSGS can not be determined. Some cases are thought to be associated with congenital kidney defects, urine backing up into the kidneys, obesity, obstructive sleep apnea, sickle cell anemia, or viruses (e.g., HIV).

Known causes include:

• Drugs such as heroin, bisphosphonates, anabolic steroids
• Infection
• Inherited genetic problems
• Obesity
• Reflux nephropathy (a condition in which urine flows backward from the bladder to the kidney)
• Sickle cell disease
• Some medicines

Riskfactors

The condition affects both children and adults. It occurs slightly more often in men and boys. It is also more common in African Americans. Focal segmental glomerulosclerosis causes up to a quarter of all cases of nephrotic syndrome.

Signs and symptoms

Symptoms may include:

• Foamy urine (from excess protein in the urine)
• Poor appetite
• Swelling, called generalized edema, from fluids held in the body
• Weight gain

Diagnosis

The health care provider will perform a physical exam. This exam may show tissue swelling (edema) 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

Treatments may include:

• Medicines to reduce the body's inflammatory response.
• Medicines to lower blood pressure. Some of these medicines also help reduce the amount of protein that spills into the urine.
• Medicines to get rid of excess fluid (diuretic or "water pill").
• Low sodium diet to reduce swelling and lower blood pressure.

The goal of treatment is to control the symptoms of nephrotic syndrome and prevent chronic kidney failure. These treatments may include:

• Antibiotics to control infections
• Fluid restriction
• Low-fat diet
• Low- or moderate-protein diet
• Vitamin D supplements
• Dialysis
• Kidney transplant

Prognosis

A large portion of people with focal or segmental glomerulosclerosis will develop chronic kidney failure.

Possible Complications Complications may include:

• Chronic kidney failure
• End-stage kidney disease
• Infection
• Malnutrition
• Nephrotic syndrome

NIH genetic and rare disease info

• NIH info on Focal segmental glomerulosclerosis

Focal segmental glomerulosclerosis is a rare disease.

Focal segmental glomerulosclerosis Resources
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