| Focal segmental glomerulosclerosis | |
|---|---|
| Other names | Segmental glomerulosclerosis, focal sclerosis with hyalinosis, familial idiopathic nephrotic syndrome[1] |
 | |
| Light micrograph of focal segmental glomerulosclerosis, hilar variant. Kidney biopsy. PAS stain. | |
| Specialty | Nephrology |
| Symptoms | Foamy urine, swelling[2][1] |
| Complications | High blood pressure, kidney failure[2][3] |
| Types | Primary, secondary[3] |
| Causes | Unknown, genetics, certain medications, vesicoureteral reflux, obesity, obstructive sleep apnea, HIV/AIDS[1][3] |
| Diagnostic method | Kidney biopsy[2] |
| Differential diagnosis | Membranoproliferative glomerulonephritis, lupus, other causes of nephrotic syndrome[3] |
| Treatment | Immunosuppressants, blood pressure control[2][3] |
| Frequency | 7 per million people[4] |
Focal segmental glomerulosclerosis (FSGS) is term for a specific pattern of damage in the kidneys.[4] Symptoms may include protein in the urine and swelling.[1][2] Complications may include high blood pressure and kidney failure.[2][3]
The cause is often unclear.[1] Other cases may occur due to genetics, certain medications, vesicoureteral reflux, obesity, obstructive sleep apnea, or HIV/AIDS.[1][3] The underlying mechanism involves scaring of parts of the glomerulus due to injury of podocytes.[3] A kidney biopsy may confirm the diagnosis, but early in the disease a normal biopsy may not exclude it.[2]
Treatment involves the use of immunosuppressants such as steroids, tacrolimus, or rituximab.[3] Blood pressure may be managed with ACE inhibitors and swelling may be treated with diuretics.[2][3] If kidney failure occurs, dialysis or kidney transplant may be requires.[4]
FSGS affects about 7 per million people.[4] It is the cause of about 40% of cases of nephrotic syndrome (high levels of protein in the urine) in adults and 20% in children.[3] The condition was first described in 1925 by Fahr.[5]
References edit
- ^ a b c d e f "Focal segmental glomerulosclerosis | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov. Retrieved 24 January 2021.
- ^ a b c d e f g h "Glomerular Diseases | NIDDK". National Institute of Diabetes and Digestive and Kidney Diseases. Retrieved 24 January 2021.
- ^ a b c d e f g h i j k Guruswamy Sangameswaran, KD; Baradhi, KM (January 2020). "Focal Segmental Glomerulosclerosis". PMID 30335305.
{{cite journal}}: Cite journal requires|journal=(help) - ^ a b c d "Focal Segmental Glomerulosclerosis". NORD (National Organization for Rare Disorders). Retrieved 24 January 2021.
- ^ Jennette, J. Charles (2007). Heptinstall's Pathology of the Kidney. Lippincott Williams & Wilkins. p. 156. ISBN 978-0-7817-4750-9.