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63 Cards in this Set
- Front
- Back
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Glomerular filtration apparatus composition
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Negatively charged
Endothelial cells Epithelial cells (podocytes) Mesangial cells Basement membrane |
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Immune complex damage to glomerulus
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In situ -- anti-GBM and membraneous
Circulating - lupus, post infectious |
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Focal glomerular damage
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Some of the glomeruli are involved
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Diffuse glomerular damage
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All of the glomeruli are involved
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Global glomerular damage
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Whole glomerulus is damaged
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Segmental glomerular damage
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Part of the glomerulus is damaged
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Pathologic examination of kidney biopsy
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Light
Immunoflorescence EM |
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Nephrotic syndrome associated diseases
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Minimal change disease
Focal segmental glomerulosclerosis Membraneous glomerulonephropathy Diabetes mellitus Amyloidosis All have little to any cellular proliferation but show changes in the filtering apparatus |
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Hematuria associated diseases
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Thin basement membrane disease
Alport's disease IgA nephropathy |
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Nephritic syndrome associated diseases
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Immune complex related with proliferative features
Acute postinfections glomerulonephritis Membraneoproliferative glomerulonephritis Lupus nephritis Mesangioproliferative glomeruloneprhitis Necrotizing glomerulonephritis |
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Glomerular crescents
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Proliferation of epi and mononuclear cells plus fibrin
Sign of severe glomerular injury Categorized by IF |
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Categories of crescent disease of glomerulus
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Anti-GBM
Immune complex Pauci immune (often ANCA positive) |
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Structure of glomerulus
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Tuft of interconnected capillaries invaginating into a blind pouch extension of the proximal tubule
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Glomerular endothelium
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Cytoplasm perforated by fenestra
Cell surface carries a negative charge from polyanionic glycoproteins |
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Glomerular basement membrane
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Type IV collagen, negatively charged proteoglycans (heparin sulfate), laminin
Made by endothelial and viseral epithelial cells Serves as charge and size filtration barrier |
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Glomerular visceral epithelium
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Podocytes
Cytoplasmic extensions form feet that intertidigitate Gaps are spaned by filtration slit diaphragms Contractile proteins for active role in filtration regulation |
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Glomerular mesangium
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Mesangial cells and basement membrane like material
Structural support of capillary loops Contractile to regulate flow Phagocytic Reactant to glomerular injury (migrate, proliferate, elaborate) |
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Factors leading to glomerular filtration of a solute
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Size of molecule
(>68K MW is too big) Charge -- negative is repelled Shape and flexibility Hemodynamic forces |
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Most common cause of glomerulonephritis?
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Immune complexes
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anti-GBM disease
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In situ antibody deposition
Linear IF Ag is part of Type IV collagen <1% of cases of glomerulonephritis |
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Membraneous glomerulonephritis pathogenesis
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In situ immune complex formation
Ag is epithelial cell membrane protein (phospholipase A R) Immune complexes shed to form subepithelial deposits |
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IF in immune complex nephritis
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Granular
Deposits are usually mesagial or subendothelial, rarely subepithelial Localized based on charge mesangium - neutral subepi - cationic subendo - anionic |
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Cells that mediate glomerular damage
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PMNs
Monocytes Platlets Glomerular cells |
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Soluble mediators of glomerular damage
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Chemotactic complement factors
Ecosinoids, NO, endothelin - vessel changes Il-1, TNFalpha PDGF - mesangial growth TGFbeta - ECM deposition, glomerulosclerosis |
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Glomerular hypercellularity
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>3 mesangial cells per area
Mesangial and endocapillary Extracapillary - crescents |
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Linear IF in glomerulus
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anti-GBM disease (confirm with serology)
Also: light chain deposition disease, mebranoproliferative GN, non-specifically in diabetic |
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Granular IF in glomerulus
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Immune complexes disease
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EM is necessary to
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Diagnose minimal change disease
Also helpful for finding electron dense depositis, basement membrane alterations, looking at podocytes |
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Increase in glomerular size seen in
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Diabetes
Obesity Compensatory hypertrophy others |
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Decrease in glomerular size seen in
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Ischemia
Nephrosclerosis Aging |
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Endocapillary hypercellularity in glomerulus can be
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Proliferative - mesangial or endothelial cells
Exudative - influx of inflammatory cells |
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Endocapillary hypercellularity in glomerulus can cause
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Narrowing or closure of capillary lumen
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Glomerular crescent
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Cellular proliferation of > 2 cell layers taking up space in Bowman's capsule
Epithelial proliferation in response to severe glomerular injury and leak of fibrin into space from capillary rupture Epithelial and inflammatory cells observed |
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Crescentic glomerulonephritis
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>50% of glomeruli show crescents
Usually presents as rapidly progressive glomerulonephritis with rapid decline in renal fnc Poor prognosis |
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Differentiating cause of crescentic glomerulonephritis
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Immunofluroescence
Anti-GBM Ab diseases Immune complex Pauci-immune - often ANCA postive |
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Proteinuria effect on podocytes
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Swelling
Microvillous transformation Foot process effacement |
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Mesangiolysis
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Dissolution of mesangium and degeneration of mesangial cells
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Minimal change disease of the glomerulus pathology
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Normal on light and IF
Effacement of foot processes on EM |
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Most common cause of nephrotic syndrome in children
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Minimal change disease
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Clinical aspects of minimal change GN
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Nephrotic
Often in kids Occasionally seen with NSAIDs and lymphomas Benign course and good response to steroids |
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Focal segmental glomerular sclerosis pathology
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Areas of sclerosis in some glomeruli
Nonspecific IgM and C3 in sclerotic areas Foot process effacement on EM |
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Focal segmental glomerular sclerosis clinical features
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Nephrotic
Progressive with poor response to Rx Primary or secondary to HIV, reflux, systemic disease, heroin, obesity, others |
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Membraneous glomerulonephropathy pathologic
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Spikes and holes in glomerular basement membrane seen on light
Diffuse granular IF around basement membrane (IgG, C3) Subepithelial deposits in capillary wall by EM Deposits evolving over time |
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Membraneous glomerulonephropathy clinical
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Nephrotic
Adults 85% primary 15% 2/2 drugs, tumors, SLE, infections, metabolic disorders |
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Most common cause of nephrotic syndrome in adults
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Membraneous glomerulonephropathy
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Diabetic glomerulonephropathy patholgy
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Early - glomerular hypertrophy, thickening of BM, mesangial expansion
Late - progressive thickening of BM, diffuse or nodular (KW) glomerulosclerosis, insudative lesions, microaneurysms, arteriolar hylanizaiton |
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Amyloidosis GN
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Most common is light chain
Variable messangial and GBM deposition of protein EM: 8-12 nm twisted fibrils |
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Post infectious glomerulonephritis pathology
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Diffuse instrinsic cell proliferation and leukocytic inflitrate
Swollen, hypercellular glomeruli Collapse of capillary lumens IF: granular deposits along BM (IgG, C3) EM: Subepithelial humps |
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Membranoproliferative glomerulonephritis clinical
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Nephritic or nephrotic
Often secondary to Hep C, autoimmune disease, dysproteinemias, other infections Often progresses to ESRD despite treatment |
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Membranoproliferative glomerulonephritis pathology
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Hypercellular glomeruli with leukocytes
Often lobular Tram-track appearance due to duplication of BM and mesangial interposition Subendothelial immune complex deposits |
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SLE nephropathy
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Nephritic
Wire loops, hyaline thrombi, fibrin necrosis, hematoxylin bodies IF: full house (Igs, C3, C1q, light chain +) EM: mesangial deposits, sometimes subendothelial and subepithelial |
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Classification of SLE nephropathy
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By light microscopy
Normal Mesangial Focal proliferative Diffuse proliferative Membraneous Diffuse sclerosis |
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Glomerular disease presenting primarily with hematuria
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IgA nephropathy
Alport syndrome Thin glomerular basement membrane syndrome |
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Most common glomerulonephropathy worldwide?
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IgA nephropathy
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IgA nephropathy
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Hematuria
Mesangioproliferative or normal appearance IF shows IgA deposits in paramesangium |
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What looks the same as IgA nephropathy pathologically?
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Henoch-Schonlein purpura
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Alport syndrome
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Nephritis, nerve deafness, ocular abnormalities
Defects in Type IV collagen Basketweave appearance of glomerular basement membrane |
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Thin basement membrane disease
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Benign familial hematuria
Persistent intermittent hematuria Thin basement membrane ?variant of alports, similar mutations |
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Goodpasture disease
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Anti-GBM antibody - nephrotic syndrome
Cross reaction with alveolar basement = hemorrhage |
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Major causes of pauci-immune crescentic glomerulonephritis
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Wegner's
microscopic angiitis Churg-Strauss |
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GNs normal by light micro
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Minimal change
Alport Thin basement membrane SLE type 1 |
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Antigen in anti-GBM disease
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Noncollagneous domain of the alpha3 chain of type IV collagen
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Antigen in membraneous glomerulonephritis
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M-type phospholipase A receptor on basal surface of epithelial cells
Binding-complement- shedding of the immune complexes |
