FSGS - FSGS FSGS was first described and illustrated in...

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Unformatted text preview: FSGS FSGS was first described and illustrated in 1925 During the 1970s, the differences between FSGS and minimal change in nephrotic patients were emphasized as it became obvious that the prognosis of FSGS was in general poorer Later in the 1980s, however, discussion swung back to emphasizing the many similarities between patients with the two appearances, prompted by the fact that one appearance could evolve into the other in serial biopsies Cameron. Nephrol Dial Transplant (2003) FSGS accounts for up to 20% of dialysis patients The diagnosis of FSGS requires the presence of areas of glomerular sclerosis that are both focal and segmental The clinical hallmarks include proteinuria, nephrotic syndrome and frequently the progressive loss of renal function In all forms of FSGS injury is directed to or originates within the podocyte D'Agati Curr Opin Nephrol Hypertens. 2008 Clinical features The significance of a finding of FSGS cannot be interpreted in detail without information on the clinical setting Proteinuria is almost invariable Secondary forms of FSGS rarely present with a full nephrotic syndrome, even if in the presence of quite heavy proteinuria In primary FSGS, macroscopic hematuria is very rare, but microscopic haematuria usual and hypertension common Stewart. Nephrol Dial Transplant 2003 FSGS shows a remarkable racial predominance accounting for almost two- thirds of AA adults with a nephrotic syndrome Clinically, the most important prognostic feature is whether or not proteinuria resolves, almost always in association with treatment Patients in whom this happens, even if they relapse repeatedly, almost always do well Stewart. Nephrol Dial Transplant 2003 The only other features that have prognostic value are renal function at the time of investigation and the amount of proteinuria Age, sex, race and hypertension do not seem to affect prognosis In renal biopsy specimen the major prognostic factor is the presence and extent of tubulo- interstitial damage Stewart. Nephrol Dial Transplant 2003 Histologic variants Collapsing variant There is at least one glomerulus with tuft collapse and overlying visceral epithelial cell hypertrophy and hyperplasia Characterized by: Black racial predominance Severe markers of nephrotic syndrome Poor response to steroid A rapidly progressive course to renal failure D'Agati VD Curr Opin Nephrol Hypertens. 2008 Causes HIV Parvovirus B19 Acute cytomegalovirus infection Erythrophagocytosis syndrome Interferon therapy Pamidronate toxicity Acute vaso-occlusive injury Rare familial forms D'Agati VD Curr Opin Nephrol Hypertens. 2008 Tip variant Defined by at least one segmental lesion involving the tip domain Is more common in Caucasian adults Tends to present with abrupt onset of full nephrotic syndrome There is usually less tubulointerstitial injury Responds better to steroids and there is usually preservation of renal function Most cases are idiopathic D'Agati VD Curr Opin Nephrol Hypertens. 2008 Perihilar...
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This note was uploaded on 12/24/2011 for the course STEP 1 taught by Professor Dr.aslam during the Fall '11 term at Montgomery College.

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FSGS - FSGS FSGS was first described and illustrated in...

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