Nephrotic Syndrome and FSGS

Identification of the gene or genes involved in primary idiopathic nephrotic syndrome could provide insight into the cause of the disease, as well as new treatment strategies.
Genes and Nephrotic Syndrome
Although the cause of primary nephrotic syndrome is not understood, it is generally thought to be due to an abnormality of the filtering units of the kidney called glomerulus. The incidence of primary nephrotic syndrome is variable and depends upon the ethnic background, which suggests a genetic tendency. The African-American population is especially susceptible to a particularly severe form of nephrotic syndrome called focal segmental glomerulosclerosis (FSGS). A particular type of nephrotic syndrome called congenital nephrotic syndrome of Finnish type (CNF) is especially common in the Finnish population and is a distinct entity.
FSGS
Focal Segmental Glomerulosclerosis (FSGS) is a rare disease that attacks the kidney’s filtering system (glomeruli) causing serious scarring. FSGS is one of the causes of a serious condition known as Nephrotic Syndrome.
Focal Segmental Glomerulosclerosis means:
Focal = some
Segmental = sections
Glomerulo = of kidney filters
Sclerosis = are scarred
Symptoms of Nephrotic Syndrome and FSGS
Common symptoms include edema, or swelling, especially in the legs, hypertension and high blood pressure, although in many cases, no clinical signs appear.
Complications
High protein levels in the urine can lead to high cholesterol and increased blood clotting. Complete renal failure, which requires a kidney transplant or dialysis, can occur when FSGS is left untreated.
Nephrotic Syndrome and FSGS facts
There are currently 19,306 people living with ESRD due to FSGS , in part because it is the most common cause of steroid resistant Nephrotic Syndrome in children, and it is the second leading cause of kidney failure in children. People of African ancestry are at a five times higher diagnosis rate of FSGS. About half of FSGS patients who do not respond to steroids go into ESRD each year, requiring dialysis or transplantation. Approximately 1,000 FSGS patients a year receive kidney transplants. However, within hours to weeks after a kidney transplant, FSGS returns in approximately 30-40% of patients.