Focal segmental glomerulosclerosis (FSGS) is a cause of nephrotic syndrome in children and adolescents, as well as a leading cause of kidney failure in adults. It is also known as “focal glomerular sclerosis” or “focal nodular glomerulosclerosis.” It accounts for about a sixth of the cases of nephrotic syndrome. (Minimal change disease (MCD) is by far the most common cause of nephrotic syndrome in children: MCD and primary FSGS may have a similar cause.)
In children and some adults, FSGS presents as a nephrotic syndrome, which is characterized by edema (associated with weight gain), hypoalbuminemia (low serum albumin, a protein in the blood), hyperlipidemia and hypertension (high blood pressure). In adults it may also present as kidney failure and proteinuria, without a full-blown nephrotic syndrome.
Some general secondary causes are listed below:
- Glomerular hypertrophy/hyperfiltration
- Unilateral renal agenesis
- Morbid obesity
- Scarring due to previous injury
- Focal proliferative glomerulonephritis
- Toxins (pamidronate)
- Human immunodeficiency virus-associated nephropathy
- Heroin nephropathy
Focal segmental glomerulosclerosis may develop following acquired loss of nephron’s from reflux nephropathy. Proteinuria is nonselective in most cases and may be in subnephrotic range(nephritic range <3.0gm/24hr) or nephritic range.
There are currently several known genetic causes of the hereditary forms of FSGS.
- Blood tests — cholesterol
- Kidney biopsy
- American Kidney Fund: http://www.kidneyfund.org/
- National Kidney Foundation: https://www.kidney.org/
- Nephrotic Syndrome Study Network: http://www.neptune-study.org/
- NephCure Kidney International: https://nephcure.org/