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Renal

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nonspecificstchanges's version from 2017-02-28 17:36

Nephrotic & Nephritic Syndromes

Nephrotic Syndrome Basic Principles

Glomerular disorders characterized by proteinuria >3.5 g/day resulting in
Hypoalbuminemia --> pitting edema
Hypogammaglobulinemia --> Increased infection risk
Hypercoagulable state --> loss of ATIII (anti-thrombin III)
Hyperlipidemia & hypercholesterolemia --> may result in fatty casts in urine

Nephritic Syndrome Basic Principles

Glomerular disorders characterized by glomerular inflammation & bleeding
Limited proteinuria (<3.5/day)
Oligouria & Azotemia
Salt retention w/ periorbital edema & HTN
RBC casts & dysmorphic RBCs in Urine
Biopsy-> Hypercellular, Inflamed Glomeruli
Immune-Complex deposition activates Complement; C5a attracts neutrophils, which mediate damage.

Nephrotic/Nephritic Syndromes

SyndromeNephrotic/NephriticH&E Stain FindingsEM FindingsImmune Complexes/IFAssociationsClassic Pt populationAdditionalTx
Minimal Change Disease (MCD)NephroticNormal Glomeruli on H&E Stain; Lipid may be seen in proximal tubule cellsEffacement of Foot Processes on EMNo Immune complexes; Negative IFUsually idiopathic; Associated with Hodgkin LymphomaChildrenSelective proteinuria (loss of Albumin ; but NOT inmmunoglobulin)Excellent Response to Steroids (damage mediated by cytokines from T cells)
Focal Segmental Glomerulosclerosis (FSGS)NephroticFocal & Segmental sclerosis on H&E StainEffacement of Foot Processes on EMNo Immune complexes; Negative IFUsually idiopathic; Associated with HIV, Heroin use, Sickle Cell DiseaseHispanics & African AmericansProgresses to Chronic Renal FailurePoor Response to steroids
Membranous NephropathyNephroticThick Glomerular Basement membrane on H&E StainSubepithelial deposits with "Spike & Dome" on EMImmune complex deposition (subepithelial); Granular IFUsually idiopathic; Associated with HBV/HCV, Solid tumors, SLE, or drugs (eg NSAIDs)Caucasian AdultsProgresses to Chronic Renal FailurePoor Response to steroids
Membranoproliferative Glomerulonephritis Type INephroticThick Glomerular Basement membrane on H&E StainSubendothelial deposits "Tram-track" appearanceImmune complex deposition (subendothelial); Granular IFAssociated with HBV/HCVn/aProgresses to Chronic Renal FailurePoor Response to steroids
Membranoproliferative Glomerulonephritis Type IINephroticThick Glomerular Basement membrane on H&E StainIntramembranous dense depositsImmune complex deposition (intramembranous); Granular IFAssociated C3 nephritic factor (autoantibody that stabilizes C3 convertase -> overactivation of complement, inflammation, and Low levels of circulating C3n/aProgresses to Chronic Renal FailurePoor Response to steroids
Diabetes Mellitus (DM)NephroticSclerosis of Mesangium; Kimmelstiel-Wilson nodulesn/an/an/an/aHigh serum glucose -> nonenzymatic glycosylation of vascular BM -> Hyaline arteriosclerosis (leaky BM); Glomerular Efferent arteriole more effected -> High Glomerular Filtration Pressure -> MicroalbuminuriaACE Inhibitors slow progression of hyperfiltration induced damage
Systemic AmyloidosisNephroticAmyloid deposits in mesangium; Apple-green Birefringence under Polarized light (Congo Red Stain)n/an/an/an/an/an/a
Post Streptococcal Glomerular Nephritis (PSGN)NephriticHypercellular, inflamed glomeruli on H&E StainSubepithelial deposits "Humps" on EMImmune complex deposition; Granular IFAssociated with previous Group A B-Hemolytic streptococcal infection of skin (impetigo) or pharynx; May occur after Non-streptococcal infections as welln/aChildren rarely progress to Renal Failure; 25% of Adults develop Rapidly Progressive Glomerulonephritis (RPGN)Treatment is Supportive
Rapidly Progressive Glomerulonephritis (RPGN)NephriticCrescents in Bowman's Space on H&E Stain; cresents composed of Fibrin & Macrophagesn/aClinical Picture & IF help resolve etiologyn/an/aProgresses to Renal Failure in Weeks to monthsn/a
Good Pasture Syndrome (RPGN)NephriticCrescents in Bowman's Space on H&E Stain; cresents composed of Fibrin & Macrophagesn/aAntibody against collagen in glomerular & alveolar BMs; Linear (Anti-BM) IFHematuria & Hemoptysis (Lungs/Kidney)Young adult malesn/a
Diffuse Proliferative Glomerular Nephritis (RPGN)NephriticCrescents in Bowman's Space on H&E Stain; cresents composed of Fibrin & Macrophagesn/aAntigen-Antibody complex deposition, subendothelial; Granular IFSLEn/amost common renal disease in SLEn/a
Wegener Granulomatosis (RPGN)NephriticCrescents in Bowman's Space on H&E Stain; cresents composed of Fibrin & Macrophagesn/aNegative IF; C-ANCA (PR3-ANCA) Associated Nasopharynx, lung, kidney
Churg-Strauss (RPGN)NephriticCrescents in Bowman's Space on H&E Stain; cresents composed of Fibrin & Macrophagesn/aNegative IF; P-ANCA (MPO-ANCA)Associated with Granulomatous inflammation, Eosinophilia, asthman/an/an/a
Microscopic Polyangiitis (RPGN)NephriticCrescents in Bowman's Space on H&E Stain; cresents composed of Fibrin & Macrophagesn/aNegative IF; P-ANCA (MPO-ANCA)No symptoms of granulomas, eosinophilia, or asthma.
IgA Nephropathy (Berger Disease) (RPGN)Nephriticn/an/aIgA immune complex in mesangium of Glomeruli; IgA IFChildhood fallowing mucosal infectionsChildrenEpisodic gross or microscopic hematuria with RBC casts following mucosal infection (eg gastroenteritis); IgA production Increased during infection; May slowly progress to renal failuren/a
Alport SyndromeNephriticCrescents in Bowman's Space on H&E Stain; Thinning and splitting of Glomerular BMn/an/aPresents as isolated hematuria, sensory hearing loss, & occular disturbancesMore often Males (X Linked)X Linked inherited defect in type IV Collagenn/a
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