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Minimal Change Disease, "nil" disease; lipoid nephrosis -85-90% of nephrotic syndrome in children -Children 2-8 yoa, M:F 2:1 -20% of nephrotic syndrome in adults -Bland urinary sediment -Bx: light microscopy normal; EM shows fusion of podocyte (visceral epithelial cell) foot processes -Highly responsive to corticosteroids (in children) ---85-90% of children respond to steroids ---Adults may require longer courses of steroids -70% have one or more relapses ---Frequent relapsers = "corticosteroid dependent" ---May benefit from cytotoxic drugs, cyclosporine -Increased risk for pneumococcal and Haemophilus -Renal failure does not occur unless other disease process Focal Glomerulosclerosis (FGS) -10-15% children with nephrotic syndrome -15-20% of adults with nephrotic syndrome -Heavy proteinuria, variable hematuria, normal C3 -Biopsy: focal & segmental collapse of capillary loops -HIV-related ___: tubuloreticular structures -One third response to corticosteroids; most progress to chronic renal failure after years -Heroin use another secondary cause of ___ -accounts for about a sixth of cases of nephrotic syndrome in adults and in children. Membranous Glomerulopathy -"Bland" urinary sediment -Causes: ---I. Primary (idiopathic) ---II. Secondary: hepatitis B, syphilis, carcinomas, gold therapy, D-penicillamine, captopril; collagen vascular diseases -About 50% progress to end-stage renal disease by 5-10 years; may have spontaneous remissions Diabetic Nephropathy -Persistent albuminuria (>300 mg/24 hr), progressive decline in GFR, elevated BP -Urinary albumin >30 mg/24 h strongly predicts development of ____ -Diabetic retinopathy found in >90% of patients with type I DM with ____ -_____ single most important cause of ESRD in U.S., 40% of all dialysis patients -Time course: rare during first 5 years of DM -Peak incidence after 15-20 years DM -Classic lesion on bx: *"Kimmelstiel-Wilson"* nodular glomerulosclerosis, a nodular increase in hyaline material that expands the mesangium; only found in 15-20%. Arteriolosclerosis of affect and efferent arterioles. -Treatment: vigorous control BG; treat HBP; ACE inhibitors have an antiproteinuric effect; may help in microalbuminuria even with normal BP. Kimmelstiel-Wilson disease -Advanced diabetic nephropathy including nephrotic syndrome ---Once nephrotic syndrome develops, ESRD within 3-7 years -Named for two pathologists who described the typical features in 1930s -Pathologic feature: intercapillary glomerulosclerosis -On biopsy, nodules of pink hyaline material forms in regions of glomerular capillary loops, with a marked *increase in mesangial matrix* from damage due to non-enzymatic glycosylation of proteins -Nodular glomerulosclerosis in a patient with diabetic nephropathy: *increased mesangial matrix* due to non-enzymatic glycosylation of proteins Post-Streptococcal GN (PSGN) -A form of post-infectious GN -Follows an infection with group A ß-hemolytic streptococcus of the throat, upper respiratory tract or skin -Latent period 8-14 days for respiratory tract, and 21-28 days for skin infection -ASO titer is positive in 90% following upper respiratory infection and 50% following skin -Serum complement levels are low -Most common cause for acute nephritic syndrome in children, but can also be seen in adults -Biopsy: diffuse proliferative GN; IgG + C3 deposits -Treatment supportive: diuretics, Rx. Hypertension -Antibiotics if Streptococcus cultured -Complete recovery occurs 90-95% -Proteinuria/hematuria may persist for 1-2 years -immunofluorescence demonstrating coarsely granular capillary loop deposits of IgG Wegener's Granulomatosis -Small-vessel vasculitis associated with necrotizing granulomatous inflammation affecting the upper and lower respiratory tracts and kidneys -Renal pathology not specific for __ ---ANCAs positive in 65-90% -Clinical features: fever, malaise, purpuric rash, pulmonary hemorrhage, sinusitis (septal perforation, saddle nose deformity) -Prognosis poor without treatment (90% mortality 2 years) ---Steroids and cytotoxic agents can produce remission in about 75% ---11% progress to ESRD -Vasculitis involving Kidneys and lungs -The cold that did not get better -Saddle nose-sinus involvement -It is not uncommon IgA Nephropathy, Berger's Disease -Key finding on biopsy: mesangial IgA deposits -*Most common form of glomerulonephritis worldwide* -Typically presents as gross hematuria after a viral (respiratory) illness -Men affected more than women 3:1 -Renal biopsy required for diagnosis -This immunofluorescence pattern demonstrates positivity with antibody to IgA. Note that the pattern is that of mesangial deposition in the glomerulus. -IgA Nephropathy is the final diagnosis in 50% of patients with asymptomatic hematuria submitted to renal biopsy -Slow progressive course; renal failure develops in 20-30% after 20 years -No demonstrated effective therapy -IgA deposits may recur in transplant Polyarteritis nodosa -one of the causes of acute nephritic syndrome ---with normal serum complement level -rapidly progressive glomerulonephritis (rpgn) ---idiopathic or associated with other systemic vasculitis (_______, & wegeners) Henoch-Schonlein purpura one of the causes of acute nephritic syndrome with *normal serum complement level* -IgA nephropathy associated with systemic vasculitis -Small vessel vasculitis most often seen in children. -Higher incidence in winter and spring -Palpable purpuric lesions over buttocks. -IgA + C3 deposits seen on biopsy of skin lesions -Hematuria, proteinuria, RBC casts, normal C3 levels -Self-limited course normally, but 10% progress to end-stage renal disease Rapidly Progressive Glomerulonephritis (RPGN), Anti-Glomerular Basement Membrane Glomerular Nephritis, Goodpasteur's Disease -Renal hematuria (RBCs, RBC casts) with renal failure developing over weeks to months -Diffuse glomerular crescent formation on biopsy ---*Crescents* are accumulations of macrophages, fibroblasts and fibrin within Bowman's space which indicate active, and usually severe glomerular injury. -Proliferating epithelial cells form crescents that compress the glomerular tufts -I. Anti-GBM mediated -II. Immune-complex mediated -III. Non-immune complex mediated Membranoproliferative Glomerulonephritis (MPGN) -_____ Type I- This silver stain demonstrates a double contour to many basement membranes, or the "tram-tracking" that is characteristic of _____________) type I that results from basement membrane reduplication. -May be idiopathic or associated with other diseases -Important association with Hepatitis C -Three types: ---Type I most common; immune complexes,  C ---Type II "dense-deposit disease", C3 nephritic factor in serum; activation of alternate C pathway ---Type III mixed features Lupus (SLE) Nephritis -"Butterfly" facial rash of __ -SLE is an autoimmune disease affecting skin, kidneys, joints, serous membranes & blood vessels -Up to 60% of adults and 80% of children develop some degree of renal involvement -___is more common in women aged 20-40 M:F 9:1 -Common presenting symptoms: fever, arthralgia, typical malar or butterfly rash; photosensitivity -Associated with anti-nuclear antibodies (ANA); low serum complement with renal involvement -Drug-induced: hydralazine, procainamide, sulfonamides -Anti-nuclear antibodies (ANA) -Antibodies to double-stranded DNA -Low serum complement with renal involvement -SLE Treatment Options ---High-dose I.V. corticosteroids (methylprednisolone) followed by oral ---Cytotoxic agents ex. Cyclophosphamide ---Dialysis and Transplantation: many patients experience improvement of other SLE-related symptoms after starting dialysis Hemolytic-uremic syndrome -A form of thrombotic microangiopathy: systemic platelet consumption with formation of intraluminal platelet thrombi -Classic childhood ___ is preceded by an acute diarrheal illness caused by an E. coli strain -Clinical features: renal failure, mental status changes preceded by 1 week of diarrheal illness -Lab: hemolytic anemia with abnormal RBCs (schistocytes) on peripheral blood smear, low platelet count -Treatment is supportive - Very similar to thrombotic thrombocytopenic purpura (TTP) is ______________). The two conditions may be difficult to tell apart. HUS can be a leading cause for acute renal failure in children. Ingestion of foods, such as poorly cooked ground beef, introduces a verotoxin-producing E. coli infection into the GI tract. Such strains are often identified by serotyping, typically type O157:H7. A bloody diarrhea is followed in a few days by renal failure caused by endothelial injury from the toxin, leading to the characteristic fibrin thrombi in glomerular and interstitial capillaries. Most patients recover in a few weeks with supportive dialysis. TTP: thrombotic thromocytopenic purpura -A form of thrombotic microangiopathy affecting mostly adults, especially black women -Clinical features: fever, CNS dysfunction, thrombocytopenia, hemolytic anemia, and renal failure -Major morbidities arise from CNS involvement and bleeding -Plasma exchange may be effective in 70-90% Amyloidosis -Fibril-like deposition of monoclonal Ig components -___________occurs secondary to circulation of abnormal precursor proteins -Fibrils stain with Congo Red -Sytemic deposition: heart, lungs, GI tract, CNS, kidneys Alport Syndrome - Hereditary -one of the hereditary forms of gomerulonephritis -Boys more affected than girls -Renal failure usually develops by age 30 -Biopsy shows thinned and thickened capillary loops with splitting of GBM -No effective treatment available -Usually presents in childhood with recurrent gross hematuria, mild proteinuria, nephrotic syndrome is rare -Dx suggested by family history of kidney disease, kidney failure, deafness and ocular abnormalities -80% of pedigrees show some X-linkage -This is a type of hereditary nephritis known as ______ in which patients may also manifest nerve deafness and eye problems. The renal tubular cells appear foamy because of the accumulation of neutral fats and mucopolysaccharides. The glomeruli show irregular thickening and splitting of basement membranes. Rapidly Progressive Glomerulonephritis (RPGN): Anti-GBM GN -____: linear capillary loop staining for IgG and C3, extensive crescents ---2/3 have Goodpasture's Syndrome: pulmonary hemorrhage ---Treatment: High dose steroids, cytotoxic agents, plasma exchange -Immunofluorescence demonstrating a linear pattern of IgG -Anti-GBM antibodies directed toward collagen -Pulmonary hemorrhage (cough, hemoptysis, dyspnea) 70% (Goodpasture's syndrome) -CXR: patchy or diffuse alveolar infiltrates -Renal failure: rapid, oliguric, hematuric with modest proteinuria -Treatment: aimed at reducing circulating preformed anti-GBM antibodies by daily plasma exchange for 14 days ---Cytotoxic therapy with cyclophosphamide and immunosuppression with corticosteroids