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Thomas: Acute/subacute Renal Failure: Glomerular Disease

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Answer
Nephrotic Syndrome   Non-inflammatory Damage to the basement membrane Increased permeability Proteinuria >3.5 g/d  
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Increased premeability leads to...   ↓ albumin ↓ oncotic pressure ↓ effective circulating volume Loss of Antithrombin III  
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Nephritic Syndrome   Inflammatory Immune-mediated damage Infiltrates of neutrophils and phagocytes -Crescentic lesions -Rupture of GBM  
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Clinical and lab findings with nephritic syndrome   Hematuria Oliguria HTN RBC casts Proteinuria <3.5g/d  
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Glomerular diseases: Symptoms   Edema Weight gain Malaise Oliguria Hematuria Worsening HTN  
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Glomerular diseases: Past Medical Hx   HTN DM Hep B/C HIV Hyperlipidemia DVT/PE  
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Glomerular diseases: Family Hx   Kidney disease DM Autoimmune Dz  
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Glomerular diseases: Physical exam   Edema, JVD, crackles HTN Conjunctival pallor Signs of autoimmune dz Xanthomas  
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General labs/workup   Rule out pre/post-renal Urine protein/Cr ratio UA specifically for casts Cholesterol panel Albumin  
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What tests are used to help rule out pre/post-renal ARF?   Bladder scan UA Specific gravity FeNa Uosm  
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Nephrotic syndrome findings   Proteinuria >3.5 Serum albumin <3.5 Cholesterol >300 mg/dL Oval fat bodies on UA  
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Nephrotic syndrome findings (cont.)   ↓Ca due to ↓albumin Anemia due to loss of transferrin and erythropoietin Hypercoaguability due to loss of Antithrombin III  
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Nephrotic Syndrome Tx   Statins ACEI/ARBs Fluid and salt retention  
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Nephrotic Syndrome **BUZZWORDS**   Proteinuria Hypoabluminemia Edema Hyperlipidemia  
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Nephrotic syndromes   Minimal change disease Focal segmental GS Membranous GN Diabetic GS Amyloid  
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Minimal Change Disease   Most common type in kids 90% of nephrotic cases <10 Sudden onset Usually idiopathic  
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Minimal Change Disease: Association and Damage   A: Medications Lymphomas D: effacement of podocytes  
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Why do you get the nephrotic proteinuria with MCD?   Uniform thinning of GBM Subepithelial accumulation of immune complexes Disrupted filtration slits  
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Minimal Change Disease Clinical and Dx   Edema Proteinuria (up to 9g/d) Dx: biopsy shows effacement of podocytes  
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Minimal Change Disease Tx and prognosis   Tx: High dose steroids for 8 weeks Prognosis: Very good, few progress to ESRD  
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Focal Segmental Glomerulosclerosis (FSGS)   #1 cause of nephrotic syndrome in African Americans ~30% of adult nephrotic  
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FSGS Mechanism   HTN causes hyperfiltration Accumulation of ECM Leads to scarring and tamponade Epithelial cells damaged  
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FSGS Associations   ***HIV*** IV heroin Urethral reflux Massive obstruction Chronic pyelonephritis Interstitial kidney dz  
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FSGS Clinical   Usually asymptomatic Microscopic hematuria initially  
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FSGS Tx and prognosis   Tx High dose steroids Treat underlying cause Prognosis 50% → ESRD in 5 yrs  
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Membranous Glomerulonephropathy   From antibody/antigen deposition Up to 1/3 adult nephrotic 2:1 M:F Typical age 30-50  
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Membranous Glomerulonephropathy Mechanism   Damage to mesangial matrix Immune cells can't cross GBM so it's non-inflammatory  
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Membranous Glomerulonephropathy Causes   Usually idiopathic **Malignancies** -Breast, colon, stomach, kidney, lung Hepatitis B/C Syphilis, DM, SLE  
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Membranous Glomerulonephropathy Workup and Tx   Exclude SLE, RA Hepatitis serologies **age appropriate cancer screenings** Tx: Steroids and cytotoxic agents  
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Diabetic Glomerulosclerosis Developes within...   5-10yrs in DM II 10-20yrs in DM I ~50% diabetics  
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Diabetic Glomerulosclerosis Increased prevalence in...   Smokers Non-whites Pts with poor glycemic and BP control  
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Diabetic Glomerulosclerosis Dx   Urine microslbumin test -microalb/Cr >30 Nodular sclerosis on biopsy **Kimmelstiel-Wilson nodules**  
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Diabetic Glomerulosclerosis Tx   Glycemic control BP control with ACEI/ARBs Intensive cholesterol control (LDL<100)  
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Nephritic disease   Inflammatory responce Immune mediated damage Crescentric lesions Plug up Bowman's Capsule Necrosis/thinning of GBM Rupture of GBM  
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Nephritic disease Clinical   *********** *Hematuria* *Oliguria * * HTN * ***********  
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Nephritic disease Labs   UA: Hematuria RBC casts Protein <3.5g/d  
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Nephritic Diseases   IgA Nephropathy Henoch Schonlein Purpura Post-infectious GN Membranoproliferative GN Anti-GBM/Goodpasture's Syndrome  
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What immunoglobulin plays a critical role in mucosal immunity?   IgA  
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IgA Nephropathy   #1 cause of GN worldwide 2-3 days post infection  
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Causes of IgA nephropathy   URT and GI infxn Causes defective mucosal immunity Overproduction and increased serum IgA  
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How does a pt with IgA nephropathy present?   1. Macroscopic or gross hematuria 2. Microscopic hematuria 3. High BP or kidney failure  
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IgA nephropathy Clinical findings, Dx and Tx   Macroscopic hematuria 40% have asymptomatic microscopic hematuria Normal complement (C3&4) Tx ACEI/ARBs, steroids  
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Henoch-Schonlein Purpura   Disease of kids Follows URI IgA in: -skin, GI tract, kidney, joints  
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Henoch-Schonlein Purpura Clinical findings and Tx   Lower extremity bruising Bloody diarrhea Hematuria/HTN Arthalgias Tx: supportive  
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Post-Infectious GN   Subendothelial antigen deposition 2-3 weeks post infxn Low C3 and C4  
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How is streptococcal pharyngitis diagnosed?   Rapid strep test ASO titer  
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Post-Infectious GN Dx and Tx   Dx: Hx, ASO titer, anti-DNAase B Tx: none, self-resolving  
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Membranoproliferative GN   Idiopathic cases uncommon Usually secondary to other dz  
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Membranoproliferative GN Associated with   Sjogren's Syndrome SLE Hep B/C WITH cryoglobulinemia Infective endocarditis  
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Membranoproliferative GN Labs and Tx   UA with dysmorphic RBCs, RBC casts, low C3 and normal C4 Treat underlying cause, steroids, antiplatelets  
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Anti-GB/Goodpasture's Syndrome   10-20% of cases of rapidly progressive GN Can be ANCA+ Antibodies to type IV collagen  
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Anti-GB/Goodpasture's Syndrome Clinical   Rapid onset of HTN Oliguria **Fatal if not treated promptly**  
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Anti-GB/Goodpasture's Syndrome Kidney and lung involvement   Kidney only More common in older women (Anti-GBM) Kidney/lung More common in young men (Goodpasture's syndrome)  
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Goodpasture's Syndrome   70% will have alveolar hemorrhage (hemoptosis)  
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Anti-GB/Goodpasture's Syndrome Dx and Tx   Dx Anti-GBM antibodies, biopsy Tx Cyclophosphamide+steroids Plasmapheresis if pulmonary hemorrhage  
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Anti-GB/Goodpasture's Syndrome Prognosis depends on...   Promptness of Dx and Tx Degree of kidney involvement  
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Small Vessel Vasculitis   Wegener's Granulomatosis -Lung, kidney, sinus Microscopic Polyangiitis  
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Small Vessel Vasculitis Clinical   Decreased appetite Weight loss Hematuria/proteinuria Polymyalgia  
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Small Vessel Vasculitis Dx and Tx   c-ANCA+ anti-PR3- Wegener's anti-MPO- Microscopic Cyclophosphamide+steroids Plasmapheresis for Wegener's  
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Nephrotic/Nephritic Lupus Nephritis   WHO classifications III focal nephritis IV diffuse nephritis V Membrane formation VI >90% of glomeruli sclerotic  
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Nephrotic/Nephritic Lupus Nephritis Clinical and Dx   Acute onset of hematuria Proteinuria Edema Pancytopenia Dx: anti-Sm, anti-Ds DNA  
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Nephrotic/Nephritic Lupus Nephritis Tx and prognosis   >Class III Cyclophosphamide+/-steroids Prognosis depends on Kidney fxn Histologic type Crescents  
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