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38 terms

Kidney 3: Glomerulonephritis

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Glomerulonephritis
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?
X-linked
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
Membranoproliferative
Mesangial deposits, microscopic/gross hematuria
IgA nepropathy
Not immune complex mediated diseases
MCM, Thin Basement Membrane Disease