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Minimal-change nephropathy and focal segmental glomerulosclerosis 

Minimal-change nephropathy and focal segmental glomerulosclerosis
Chapter:
Minimal-change nephropathy and focal segmental glomerulosclerosis
Author(s):

Moin Saleem

, and Lisa Willcocks

DOI:
10.1093/med/9780198746690.003.0484
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date: 28 February 2021

Minimal-change nephrotic syndrome (MCNS) is an immune-mediated condition, usually of unknown cause. On light microscopy the glomeruli appear normal, and on electron microscopy there is effacement of epithelial cell foot processes over the outer surface of the glomerular basement membrane. MCNS is the cause of about 75% of cases of nephrotic syndrome in children and 17% in adults.

Management and prognosis—treatment in adults is with prednisolone at an initial dose of 80 mg/day, then tapering. This leads to complete remission in 90 to 95% of patients, but 50 to 75% of glucocorticoid-responsive adults will have a relapse. Progression to renal failure is not expected and would call the diagnosis of MCNS into question. Focal segmental glomerulosclerosis (FSGS) is not a specific disease entity but a histological lesion, often of unknown aetiology, which is characterized by segmental areas of glomerular sclerosis. It may be (1) primary—either due to genetic mutation, or associated with an unknown circulating protein factor that causes an increase in glomerular permeability; or (2) secondary—the end product of a variety of pathological processes including glomerular hyperfiltration, healed glomerulonephritis, viral (including HIV) infection, or parasitic infection. Management and prognosis—corticosteroid and immunosuppressive therapy should be considered only in patients with primary FSGS and nephrotic syndrome. The steroid regimen is as used for MCNS, but with lesser success. Steroid-resistant cases are treated with ciclosporin (for which there is most published evidence), mycophenolate mofetil, or cyclophosphamide. Prognosis depends on histology and response to treatment.

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