Light micrograph of focal segmental glomerulosclerosis, hilar variant. Kidney biopsy. PAS stain.
Specialty
Nephrology
Focal segmental glomerulosclerosis (FSGS) is a histopathologic finding of scarring (sclerosis) of glomeruli and damage to renal podocytes.[2][3] This process damages the filtration function of the kidney, resulting in protein presence in the urine due to protein loss.[3] FSGS is a leading cause of excess protein loss—nephrotic syndrome—in children and adults.[4] Signs and symptoms include proteinuria and edema.[2][5] Kidney failure is a common long-term complication of the disease.[5][6] FSGS can be classified as primary, secondary, or genetic, depending on whether a particular toxic or pathologic stressor or genetic predisposition can be identified as the cause.[7][8][9] Diagnosis is established by renal biopsy,[2][10] and treatment consists of glucocorticoids and other immune-modulatory drugs.[11] Response to therapy is variable, with a significant portion of patients progressing to end-stage kidney failure.[5] An American epidemiological study 20 years ago demonstrated that FSGS is estimated to occur in 7 persons per million, with males and African-Americans at higher risk .[12][13][7]
^ ab"focal segmental glomerulosclerosis" at Dorland's Medical Dictionary.
^ abcRosenberg, Avi Z.; Kopp, Jeffrey B. (2017-03-07). "Focal Segmental Glomerulosclerosis". Clinical Journal of the American Society of Nephrology. 12 (3): 502–517. doi:10.2215/CJN.05960616. ISSN 1555-9041. PMC 5338705. PMID 28242845.
^ abD'Agati V. The many masks of focal segmental glomerulosclerosis. Kidney Int. 1994 Oct;46(4):1223–41. doi: 10.1038/ki.1994.388. PMID 7861720.
^Kitiyakara C, Eggers P, Kopp JB. Twenty-one-year trend in ESRD due to focal segmental glomerulosclerosis in the United States. Am J Kidney Dis. 2004 Nov;44(5):815–25. PMID 15492947.
^ abcRydel JJ, Korbet SM, Borok RZ, Schwartz MM. Focal segmental glomerular sclerosis in adults: presentation, course, and response to treatment. Am J Kidney Dis. 1995 Apr;25(4):534–42. doi: 10.1016/0272-6386(95)90120-5. PMID 7702047.
^Korbet SM, Schwartz MM, Lewis EJ. Primary focal segmental glomerulosclerosis: clinical course and response to therapy. Am J Kidney Dis. 1994 Jun;23(6):773–83. doi: 10.1016/s0272-6386(12)80128-4. PMID 8203357.
^Haas M, Meehan SM, Karrison TG, Spargo BH. Changing etiologies of unexplained adult nephrotic syndrome: a comparison of renal biopsy findings from 1976–1979 and 1995–1997. Am J Kidney Dis. 1997 Nov;30(5):621–31. doi: 10.1016/s0272-6386(97)90485-6. PMID 9370176.
^Cite error: The named reference :6 was invoked but never defined (see the help page).
^Kiffel J, Rahimzada Y, Trachtman H. Focal segmental glomerulosclerosis and chronic kidney disease in pediatric patients. Adv Chronic Kidney Dis. 2011 Sep;18(5):332–8. doi: 10.1053/j.ackd.2011.03.005. PMID 21896374; PMCID: PMC3709971.
^Campbell KN, Tumlin JA. Protecting Podocytes: A Key Target for Therapy of Focal Segmental Glomerulosclerosis. Am J Nephrol. 2018;47 Suppl 1(Suppl 1):14–29. doi: 10.1159/000481634. Epub 2018 May 31. PMID 29852493; PMCID: PMC6589822.
^Tucker JK. Focal segmental glomerulosclerosis in African Americans. Am J Med Sci. 2002 Feb;323(2):90–3. doi: 10.1097/00000441-200202000-00006. PMID 11863085.
^Kitiyakara C, Kopp JB, Eggers P. Trends in the epidemiology of focal segmental glomerulosclerosis. Semin Nephrol. 2003 Mar;23(2):172–82. doi: 10.1053/snep.2003.50025. PMID 12704577.
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