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Renal disease which glomerulus is primary site of involvement and nature of disease is inflammation

Histologic signs of glomerular inflammation

-glomerular hypercellularity
-fibrinoid necrosis
-glomerular crescents

Acute Nephritic Syndromes

Hematuria, azotemia, hypertension,

Acute Nephritic syndromes are characteristic of what two renal diseases?

1.acute proliferative glomerulonephritis
2.crescentic glomerulonephritis

Two types of Acute Proliferative Glomerulnephritis

1.poststreptococcal glomerulonephritis
2.postinfectious glomerulonephritis

Group A beta-hemolytic streptococcus

Poststreptococcal glomerulonephritis

Glomerular disease 1-4 weeks following streptococcal infection of pharynx, impetigo. typically children 6-10

poststreptococal glomerulonephritis

Positive ASO titer test

poststreptococal glomerulonephritis, produced by group A beta-hemolytic strep bacteria

Subepithelial "humps" on EM

Acute Proliferative Glomerulnephritis
-single deposit, not numerous

Granular IgG and C3 deposits - lumpy bumpy

Acute Proliferative Glomerulnephritis

LM: diffuse proliferation and neutrophilic infiltration

poststreptococal glomerulonephritis

glomerular crescents, rapid and progressive loss of renal function, end stage renal disease short period of time

Rapidly Progressive (Crescentic) Glomerulonephritis

3 groups of RPGN

1. anti GBM
2. immune complex mediated
3.Pauci-immune GN

Linear staining of IgG

Anti-GBM disease of RPGN

What GBM antigen is responsible for Anti-GBM disease in RPGN?

noncollagenous domain of the alpha 3 chain of type IV collagen

Anti-GBM with pulmonary hemorrhage (hemoptysi) and renal failure - what disease?

Goodpasture syndrome

Weak or little immune deposit in glomerulus, ANCA high,

Pauci-immune GN of RPGN

systemic vasculitis (Wegener granulomatosis, microscopic polyarteritis, or Churg-Strauss syndrome)

Pauci-immune GN of RPGN

IgA deposits in the mesangium, gross or microscopic hematuria, common type of GN worldwide

IgA nephropathy

Typically isolated renal disease

IgA nephropathy

Systemic counterpart to IgA nephropathy

Henoch-schonlein purpura, vessel vasculitis with vessel wall IgA immune complex deposits

Morphology: mesangial widening, electron dense deposits in mesangial

IgA nephropathy

2 disease of hereditary syndromes of isolated hematuria

1. alport syndrome
2.thin basement membrane disease

Glomerular injury (hematuria and progressing to chronic renal failure), bilateral hearing loss, ocular lesions

Alport syndrome

What is most common inherited form of Alport syndrome?


Morphology: GBM shows irregular areas of thickening alternating with thinning, splitting and lamination of lamina densa (basket-weave appearance)

Alport Syndrome

What gene is mutated in alport?

COL4A5 gene, Alpha-5 chain of type IV collagen, interfere with assembly and architecture of type IV collagen

Persistent microscopic hematuria, non-progressive disease, normal renal function, diffuse thining of GBM (150-200) on EM, IF negative

Thin Basement Membrane Disease (Benign Familial Hematuria)

Mutations in what genes causes thin basement membrane disease, hetero or homo?

COL4A3, COL4A4, alpha a3 or a4 for type IV collagen, hetero

Homozygosity of COL4A3/COL4A4 results in what?

autosomal-recessive Alport syndrome and progression to renal failure

Causes of Chronic Glomeruonephritis, which one is most common?

Rapidly Progressive GN (crescentic)-most common
FSGS, Membranoproliferative GN, membranous GN, IgA, Poststreptococca GN, idiopathic

Kidneys reduced in size, global glomerulosclerosis (hyalinized), damages in glomeruli, tubues, intersititum (fibrotic), vessels (sclerotic -narrowed)

Chronic Glomerulonephritis

ESRD, relentless progressive, without dialysis or transplant = death

Chronic glomerulonephritis

Large subepithelial humps, neutrophils in capillary lumens

Postinfectious GN

Small, complexes on subepithelial side, proteinuria, nephrotic syndrom, low albumin, peripheral adema

Membranous nephropathy

Deposits in subendothelial side, double contouring Jones stain


Mesangial deposits, microscopic/gross hematuria

IgA nepropathy

Not immune complex mediated diseases

MCM, Thin Basement Membrane Disease

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