Focal segmental glomerulosclerosis
(Redirected from Glomerulosclerosis, focal)
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| Focal segmental glomerulosclerosis | |
|---|---|
| Synonyms | FSGS |
| Pronounce | N/A |
| Specialty | N/A |
| Symptoms | Proteinuria, edema, hypertension, kidney failure |
| Complications | Chronic kidney disease, end-stage renal disease |
| Onset | Variable |
| Duration | Chronic |
| Types | N/A |
| Causes | Idiopathic, secondary to other conditions (e.g., HIV, heroin use, obesity, sickle cell disease) |
| Risks | Genetic predisposition, African American ethnicity |
| Diagnosis | Urinalysis, kidney biopsy |
| Differential diagnosis | Minimal change disease, membranous nephropathy, diabetic nephropathy |
| Prevention | N/A |
| Treatment | Corticosteroids, immunosuppressants, ACE inhibitors, angiotensin II receptor blockers |
| Medication | N/A |
| Prognosis | Variable; can lead to chronic kidney disease |
| Frequency | 7 per million per year |
| Deaths | Varies depending on progression to end-stage renal disease |
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
| Disease of the kidney glomerules | ||||||
|---|---|---|---|---|---|---|
|
NIH genetic and rare disease info
Focal segmental glomerulosclerosis is a rare disease.
| Rare and genetic diseases | ||||||
|---|---|---|---|---|---|---|
|
Rare diseases - Focal segmental glomerulosclerosis
|
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Contributors: Deepika vegiraju, Prab R. Tumpati, MD