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This Concept Map, created with IHMC CmapTools, has information related to: nephrotic syndrome, Primary: Minimal Change Focal Segmental Glomerulosclerosis - most cmn in US Membranous Nephropathy - most cmn in world include Minimal Change, aka lipoid nephrosis aka nil disease, Minimal Change, aka lipoid nephrosis aka nil disease Pathology "oval fat bodies" in urine, Nephrotic Syndrome signs hyperlipidemia, Nephrotic Syndrome signs urine sediment: oval fat body casts, Focal Segmental Glomerulosclerosis pathogenesis more severe form of MCD, Focal Segmental Glomerulosclerosis imp variant collapsing glomerulopathy Clinical: A variant of FSGS with a particularly poor prognosis with severe proteinuria, rapid loss of renal function and no responsiveness to steroid treatment. There is an African-American pre-dominance. Represents about 5% of idiopathic FSGS biopsies. Can recur in renal transplants., Primary: Minimal Change Focal Segmental Glomerulosclerosis - most cmn in US Membranous Nephropathy - most cmn in world include Focal Segmental Glomerulosclerosis, Minimal Change, aka lipoid nephrosis aka nil disease features usu acute onset usu follows viral URI usu normal GFR nephrotic syndrome usu w/ proteinuria&edema urine sediment:oval fat bodies, Pathogenesis: damage to podocytes(visc epi cell) - by abn lymphocyte clone - dcrsd siaoprotein and heparan sulfate proteoglycans -> loss of GBM anionic charge rare mutation in actinin, podocin and nephrin structural alterations-> Min Change or FSGS evidence for immunologic basis incrsd incidence in Hodgkin disease drug-induced hypersensitivity rxtns disease response to steroids, Focal Segmental Glomerulosclerosis etiology Primary: idiopathic, Nephrotic Syndrome signs hypoalbuminemia, Minimal Change, aka lipoid nephrosis aka nil disease incidence most cmn nephrotic syn in children 10-15% in adults 2ndary causes: drugs - NSAIDs, Li toxins - bee sting, Hg, Pb infections - immztns, mono tumors: Hodgkins, Nephrotic Syndrome Tx ACEI/ARB's salt restriction diuretics statins steroids cytotoxic agents, Focal Segmental Glomerulosclerosis pathology segmental collapse of glomerular tuft hyaline lesions & foam cells in sclerotic lesions -due to trapping of lrg molecules from blood, Focal Segmental Glomerulosclerosis etiology Secondary: Solitary kidney Unilateral renal agenesis Post nephrectomy Morbid Obesity IV drug use (Heroin Nephropathy) HIV Nephropathy Associated with a collapsing variant of FSGS Prevalent in AA Rapid clinical course to renal failure (months not years) HTN Reflux nephropathy, Minimal Change, aka lipoid nephrosis aka nil disease Tx highly steroid responsive, freq relapses remission in ~75% if steroid resistant, maybe FSGS, collapsing glomerulopathy Clinical: A variant of FSGS with a particularly poor prognosis with severe proteinuria, rapid loss of renal function and no responsiveness to steroid treatment. There is an African-American pre-dominance. Represents about 5% of idiopathic FSGS biopsies. Can recur in renal transplants. pathology Glomerular tuft collapse, usually global, with proliferation of overlying visceral epithelial cells over the collapsed tuft. Tubules show microcystic changes disproportionate to the extent of sclerosis of glomeruli. Pathogenesis: Parvovirus infection is more frequent in collapsing glomerulopathy than in the usual type of FSGS., Focal Segmental Glomerulosclerosis clinical course Usually steroid resistant 20-40% may respond to steroids ESRD develops 5-20 years after presentation Patients with collapsing variants develop ESRD in 2-3 years Increase risk of recurrence in renal allograft in patients with idiopathic FSGS, Minimal Change, aka lipoid nephrosis aka nil disease signs child sudden-onset edema (pitting) normal BUN, Cr albumin 1mg/dL (low, nrm 4) serum cholesterol 280mg/dL 4+ proteinuria 8g protein (150mg nrm), Minimal Change, aka lipoid nephrosis aka nil disease Pathology lipid in kidney give yellow color - lipoid nephrosis incrsd protein reabsrptn in prox tubules