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78 Cards in this Set
- Front
- Back
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What is this disease?
Path findings - cysts that vary in size - undifferentiated mesenchyme such as cartilage, immature collecting ducts - disorganized architecture - dilated tubules wuth cuffs of primitive stroma |
cystic renal dysplasia
- associated lesions include ureterpelvic obstruction, ureteral agenesis/atresia |
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What disease is this? What genetic defects may be involved?
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Adult polycystic kidney disease (AD)
- 85% have PKD1 (polycystin-1) mutation: chromosome 16 - others have PKD2 (polycystin-2) mutation: chromosome 4 |
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What is this disease? What other symptoms may be present?
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Adult polycystic kidney disease
- bilateral kidney enlargement - insidious onset of hematuria, proteinuria, HTN - may also have cystic liver, berry aneurysm, mitral valve prolapse |
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What is this disease? pathogenesis?
- bilateral kidney enlargement - cysts filled with fluid - cysts also found in the liver - may also have berry aneurysm |
adult polycystic kidney disease
- mutations in PKD1 or PKD2 which encodes for polycystin1 and polycystin2 respectively. - altered epithelial growth and differentiation -> abnormal ECM, cell proliferation, fluid secretion |
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PKD1 or PKD2?
- more severe, end stage renal disease and death at age 53 |
PKD1
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What is this disease? What is its genetic defect? symptoms?
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Childhood polycystic disease (AR)
- mutation in PKHD1 (fibrocystin): chromosome6 - picture shows dilated channels at right angle with the cortex - symtoms: portal hypertension with splenomegaly |
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What is this disease?
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childhood polycystic kidney disease
- dilated collecting duct: cylindrical or saccular |
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What is this disease?
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Nephronopththisis (medullary cystic disease complex)
- medullary cysts at corticomedullary junction - cortical tubulointerstitial damage |
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What is this cystic disease?
- cysts in collecting duct - hematuria, UTI, recurrent stones |
medullary sponge kidney
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What type of glomerular lesion is this?
- proliferative/hypercellular - crescent - membranous - mesangial increase |
proliferative/hypercellular
- acute glomeruloneohritis - membranoproliferative glomerulonephritis - class IV SLE (diffuse proliferative glomerulonephritis) - bacterial endocarditis |
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What type of glomerular lesion is this?
- proliferative/hypercellular - crescent - membranous - mesangial increase |
crescent
- rapidly progressive glomerulonephritis |
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What type of glomerular lesion is this?
- proliferative/hypercellular - crescent - membranous - mesangial increase |
crescent
- rapidly progressive glomerulonephritis |
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What type of glomerular lesion is this?
- proliferative/hypercellular - crescent - membranous - mesangial increase |
membranous
- membranous glomerulopathy - class V SLE |
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What type of glomerular lesion is this?
- proliferative/hypercellular - crescent - membranous - mesangial increase |
mesangial increase
- acute glomeruloneohritis - IgA nephropathy - alport syndrome - ClassII SLE - HS purpura - diabetic glomerulosclerosis |
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What is this disease? pathogenesis?
Morphology - enlarged hypercellular glomeruli - widened mesangium - subepithelial immune complex deposits: subepithelial hump |
acute proliferative glomerulonephritis
- group A beta-hemolytic strep - circulating immune complex: cytoplasmic endostreptosin, cationic antigens |
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A 5 y/o kid presents with fever, malaise, hematuria (smoky/cocoa colored urine), RBC casts, and mild proteinuria. He has recently recovered from a sore throat more than 1 wk ago. ASO titer is positive and serum C3 level is low.
What does the kid have? What is his prognosis? What would his kidney biopsy show? |
1) acute proliferative glomerulonephritis
2) good prognosis, 95% recover 3) morphology - enlarged hypercellular glomeruli: leukocyte infiltrate, endothelial and mesangial proliferation - subepithelial hump - IgG, IgM, C3 deposits in mesangium and basement membrane |
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A 30 y/o male presents with acute onset on HTN, edema, hematuria. Lab showed elevated ASO titer, decreased C3.
What illness might he had about 2 wks ago? What does he have right now?What is his prognosis? |
1) strep throat.
2) He has acute proliferative glomerulonephritis right now 3) prognosis is not as good as in children: 60% recover, may progress to chronic glomerulonephritis, or rapidly progressive glomeruloneohritis. |
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Which type of rapidly progressive GN is this?
- linear deposits in GBM: IgG, C3 - HLA-DRB1 prevalence - antigen: NC1 portion of alpha3-chain of type IV collagen |
Type I: anti-GBM-antibody induced disease (Goodpasture syndrome)
- treat with palsmapheresis + steroids and cytotoxic drugs |
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Which type of rapidly progressive GN is this?
- granular fluorescence |
TypeII: complication of immune complex mediated disease
- post-infectious glomerulonephritis - SLE - IgA nephropathy - HS purpura |
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Which type of rapidly progressive GN is this?
- no fluorescence - positive for p-ANCA |
TypeIII: pauci-immune type (small vessel vasculitis or polyangiitis)
- wegener granulomatosis - microscopic polyarteritis |
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What causes the ruptures of GBM in this EM?
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proliferating crescent -> obliterate bowmen space and compress the gloerular tuft
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What is the second most common nephrotic syndrome in adults?
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membranous glomerulopathy
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What class of disease are these?
- proteinuria - hypoalbuminemia - generalized edema - hyperlipidemia and lipiduria |
nephrotic syndrome
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What is this disease?
Morphology - diffuse thickening of glomerular capillary wall - granular subepithelial and mesangial deposits - sliver stain show spikes - no increase in cellularity - effacement of foot process |
membranous glomerulopathy
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Pathogenesis of idiopathic membranous glomerulopathy.
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direct C5b-C9 action -> activation of glomerular epithelial and mesangial cell -> release proteases and oxidants -> capillary wall injury -> protein leakage
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Give some secondary causes of membranous glomerulopathy.
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- drug: penicillamine, catapril, gold, NSAIDs
- malignant tumors - SLE: Class V - infections: chronic HepB,C, syphilis, schistosomiasis, malaria. |
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What is this disease?
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Membranous glomerulopathy
- subepithelial deposits - diffuse thickening of glomerular capillary wall |
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What is this disease?
Mophology - LM: normal glomeruli - EM: diffuse effacement of foot processes (shown) |
minimal change disease
- dramatic response to corticosteroids - highly selective proteinuria |
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What is this disease?
- massive proteinuria (highly selective) - intact renal function - may be associated with Hodgekin's disease - may follow NSAID therapy in association with acute interstitial nephritis |
minimal change disease
- immune dysfunction -> cytokines that damage visceral epithelial cells -> proteinuria - loss of glomerular polyanions - detachment of epithelial cells - often associated with respiratory infections and immunizations |
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What is the most frequent cause of nephrotic syndrome in children?
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minimal change disease
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What is the most common cause of nephrotic syndrome in adults?
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focal segmental glomerulosclerosis
- mutation in NPHS1 (CH19) which encodes nephrin - mutation in NPHS1 (CH1) which encode podocin - mutation in gene encoding podocyte actin-binding protein alpha-actinin4 - mutation in cell-cell and cell-matrix interaction mediated by alpha3beta1 integrins |
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Pathogenesis of focal segmental glomerulosclerosis.
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- mutation in NPHS1 (CH19) which encodes nephrin
- mutation in NPHS1 (CH1) which encode podocin: AR - mutation in gene encoding podocyte actin-binding protein alpha-actinin4: AD - mutation in cell-cell and cell-matrix interaction mediated by alpha3beta1 integrins |
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What is this disease?
Mophology - sclerotic areas in deep glomeruli (juxtamedullary glomeruli) - segmental hyaline deposition - mesangial matrix and cell proliferation - diffuse foot process effacement and detachment of epithelial cells from GBM |
focal segmental glomerulosclerosis
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What is this disease?
Symptoms - nonselective proteinuria - poor response to corticosteroids - may also have hematuria, HTN, low GFR |
focal segmental glomerulosclerosis
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Idiopathic focal segmental glomerulosclerosis is more common in what enthic group?
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- hispanic
- african americans |
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What is this disease?
- focal cystic dilation of tubule segments filled with proteinaceous material, inflammation, fibrosis - tubular inclusion in endothelial cells |
focal segmental glomeruloslcerosis associated with HIV:
- severe form of the collapsing variant of focal segmental glomerulosclerosis |
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What is this disease?
- adaptive response to loss of renal tissue (renal ablation) |
focal segmental glomeruloslcerosis
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What is this disease?
- secondary to IgA nephropathy |
focal segmental glomeruloslcerosis
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What type of membranoproliferative glomerulonephritis is this?
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Type I
- subendothelial deposits: C3 and IgG, C1q, C4 - activation of classical and alternative complement pathways |
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What type of membranoproliferative glomerulonephritis is this?
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Type2
- deposits in GBM proper: ribbon like - activation of alternative complement pathway |
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What is this disease?
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membranoproliferative glomerulonephritis
- hypercellular glomeruli - double contour ("tram track") - thick basement membrane - not responsive to steroids |
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What is this molecule?
- present in 70% of people who have MPGN type II - stabilizes alternative C3 convertase |
C3NEF
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What are some secondary causes of MPGN?
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- chronic immune complex disease
- alpha1-antitrypsin deficiency - CLL - hereditary deficiency of complement regulatory proteins |
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What is the most common GN worldwide?
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IgA nephropathy (Berger disease)
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What are some causes of secondary IgA nephropathy?
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- liver disease: defective hepatobiliary clearance of IgA complexes
- intestinal disease: celiac disease |
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Pathogenesis of primary IgA nephropathy.
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- respiratory/GI mucosal infection -> IgA1 containing immune complex trapped in mesangium -> activate complement pathway
- defect in normal glycosylation -> IgA1 complex more likely to bind to mesangial antigens - increased IgA production by mucosa |
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Pathogenesis of Alport syndrome.
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X-linked
- mutation in alpha5 chain of type IV collagen - lesser alpha3 and alpha4 chain of type IV collagen: protective for goodpasture syndrome |
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What is this disease?
- hematuria - nephritis - nerve deafness - lens discoloration, posterior cataracts, corneal dystrophy |
Alport syndrome
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What is this disease?
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Alport syndrome
- mesangial increase - foam cells - lipid tubular cells - fetal-like glomerulus |
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What is this disease?
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Alport syndrome (basket weave appearance)
- lamination of lamina densa - focally attenuated basement membrane (splitting GBM) |
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What is this disease?
- normal renal function - hematuria - mutation in alpha3 or alpha4 chains of type IV collagen - GBM thinned to about 150-250nm |
thin basement membrane disease
- most common cause of benign familial hematuria |
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What is the most common cause of benign familial hematuria?
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thin basement membrane disease
- mutation in alpha3 or alpha4 chains of type IV collagen |
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What is this disease?
Gross - symetrically contracted small kidney with granular cortical surface - thinned cortex, increased pelvic fat Morphology - hyaline obliteration of glomeruli: bloodless - marked atrophy of associated tubules |
chronic glomerulonephritis
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What is this disease?
Clinical presentation - loss of appetite, anemia, vomiting, weakness - azotemia |
chronic glomerulonephritis
- insidious, slow progessing to renal failure - HTN, cerebral, CV manifestations |
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What are the following associated with?
- pericarditis - chronic anemia - uremic gastroenteritis - secondary hyperparathyroidism with nephrocalcinosis - LVH - diffuse alveolar damage |
uremia
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What are some etiology of chronic glomerulonephritis?
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- post-strep glomerulonephritis in adults
- rapidly progressive glomerulonephritis - membranous glomerulonephritis - focal glomerulosclerosis - membranoproliferative glomerulonephritis |
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What is the most common cause of death in SLE?
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renal failure
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Which class SLE is this?
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ClassII: mesangial lupus
- mild hematuria, proteinuria - mesangial/ subendothelial immune complexes: IgG, C3 - mesangial hypertrophy |
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Which class of SLE is this?
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ClassIII: focal proliferative GN
- hematuria, proteinuria - proliferation of mesangial and endothelial cells - neutrophils and fibrinoid deposits - capillary thrombi |
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Which class of SLE is this?
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ClassIV: diffuse proliferative GN
- proliferation of endothelial, mesangial, and epithelial cells (resemble acute proliferative GN) - subendothelial deposits |
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Which class of SLE is this?
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ClassV: membranous GN
- subepithelial deposits |
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Which class of SLE is this?
- no renal lesion |
Class I
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What is this disease?
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SLE
- LE body: degenerating nuclei of cells reacting with anti-nuclear antibodies |
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What is this? What disease may this be associated with?
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SLE
- virus particle |
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What is this skin lesion associated with?
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SLE
- linear deposits of IgG along epidermal-dermal junction. - liquefactive degeneration of basal keratinocytes and edema |
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What is this called? What disease is this associated with?
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SLE
- wire-loop lesion; subendothelial deposits |
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What is this disease?
- purpura on extensor surface of arms and legs, buttocks - abdominal pain - non-migratory arthralgia - often follows URI |
HS-purpura
- IgA deposits in mesangium - mesangial enlargement |
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What is this disease?
- IgA deposits in mesangium - mesangial enlargement |
HS-purpura
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What renal manifestation would bacterial endocarditis present?
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- hematuria, proteinuria
- diffuse proliferative GN with focal necrotizing GN |
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What is this disease?
- diffuse mesangial sclerosis - tubular basement membrane thickening |
Diabetic glomerulosclerosis
- increased GBM synthesis: typeIV collagen and fibronectin - glycosylated proteins damage glomerulus |
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What is this? What disease is this associated with?
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Diabetic glomerulosclerosis
- nodular glomerulosclerosis |
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Pathogenesis of diabetic glomerulosclerosis.
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- increased GBM synthesis with capillary thickening: type IV collagen and fibronectin
- glycosylated proteins: damage glomerulus |
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What are some types of amyloid proteins?
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- AL: Ig light chains
- AA: non-Ig synthesized by liver - beta amyloid: amyloid protein in alzhemier disease |
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What is this disease?
- fibrillary deposits in mesangium and capillary walls - do not stain with congo red |
fibrillay glomerulonephritis
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What is this disease?
- ciculating paraproteins - monoclonal Ig deposits in glomeruli |
immunotactoid glomerulonephritis
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What is this disease?
congo red stain |
amyloid
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What is this disease?
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amyloid
- yellow-green birefringence deposits under polarized light |
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What is this disease?
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amyloid
- amyloid fibrils in GBM |
