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Kidney Path - Glomerular disease
path of glomerular disease
37
Pathology
Graduate
02/09/2011

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Cards

Term
Which congenital kidney problem results in oligohydraminos?
Definition
Bilateral renal agenesis
Term
Which congenital kidney problem can cause urinary tract obstruction?
Definition
horseshoe kidney
Term
Which disorder has no visible basement membrane changes, fused epithelial foot processes, lipid accumulation in rental tubular cells?
Definition
Minimal change disease
Term
What are the morphologic findings of focal segmental glomerulonephritis? Nephritic or nephrotic?
Definition
no visible basement membrane changes, segmental sclerosis of scattered juxtamedullary glomeruli, nephrotic
Term
Name the nephrotic disorder - basement membrane markedly thickened by intramembranous and epimembranous (subepithelial) immune complex deposits, granular immunofluorescence, spike and dome appearance. Nephritic or nephrotic?
Definition
membranous glomerulonephritis, nephrotic
Term
What are the morphological findings of diabetic nephropathy?
Definition
Basement membrane markedly thickened, diffuse or nodular mesangial accumulations of basement membrane-like material
Term
Name the disorder - protein deposits identified by Congo red staining, birefringence under polarized light, or electron microscopic criss-cross fibrillary pattern. Nephritic or nephrotic?
Definition
renal amyloidosis, nephrotic
Term
What are the morphologic findings of lupus nephrosis? Nephritic or nephrotic?
Definition
immune complex deposition in subendothelial location, may manifest as membranous glomerulonephritis, nephrotic but some cases may present with nephritic symptoms
Term
Which nephritic disorder has subepithelial electron-dense humps, or "lumpy-bumpy" immunofluorescence?
Definition
post-strep glomerulonephritis
Term
Name the disorder - crescents, ANCA-negative forms with immune complexes or antiglomerular basement membrane antibodies, ANCA-positive (pauci-immune) form with Wegener granulomatosis
Definition
Rapidly progressive (crescentic) glomerulonephritis
Term
What are the morphological characteristics of Goodpasture syndrome?
Definition
Linear immunofluorescence caused by anti-GBM antibodies
Term
Which disorder has the morphological characteristic of a split basement membrane?
Definition
Alport syndrome
Term
What is Berger disease?
Definition
IgA nephropathy, with mesangial IgA deposits
Term
Which disorder has the morphological characteristic of a tram-track appearance, deposits of C3 and dense deposits (in one variant)
Definition
membranoproliferative glomerulonephritis
Term
Membranous glomerulonephritis should be suspected when nephrotic syndrome is accompanied by...
Definition
azotemia
Term
This disorder is seen in 10% of pts with SLE, is associated with some types of infectious disease, and can cause renal vein thrombosis
Definition
membranous glomerulonephritis
Term
What are Kimmelstiel-Wilson nodules, and which disorder are they associated with?
Definition
nodular accumulations of mesangial matrix material, diabetic nephropathy
Term
Which disorder is associated with an increase in mesangial matrix, with both diffuse and nodular glomerulosclerosis?
Definition
diabetic nephropathy
Term
Renal amyloidosis is associated with which other conditions?
Definition
chronic inflammatory disease like rheumatoid arthritis and plasma cell disorders like multiple myeloma
Term
What are the types of lupus nephropathy?
Definition
Type 1 - no renal involvement, Type 2 - mesangial form, Type 3 - focal proliferative form, Type 4 - diffuse proliferative form, Type 5 - membranous form
Term
Which disorder is associated with a wire-loop abnormality?
Definition
Type 4 lupus nephrosis, from immune complex deposition and gross thickening of the GBM
Term
What are the major clinical findings of nephritic syndrome?
Definition
Oliguria, azotemia, hypertension, hematuria
Term
What type of disease is post-strep glomerulonephritis? What is the antigen?
Definition
immune complex disease, the antigen is of streptococcal origin
Term
Name the disorder - intense inflammatory reaction, innumerable punctuate hemorrhages on kidney surfaces, enlarged hypercellular swollen bloodless glomeruli, GBM of normal thickness and uniformity, subepithelial electron-dense humps, lumpy bumpy immunofluorescence
Definition
post-strep glomerulonephritis
Term
Where are the crescents in RPGN? How do they happen?
Definition
Crescents are between the Bowman capsule and glomerular tuft, from deposition of fibrin and proliferation of parietal epithelial cells
Term
What are some of the immune complex forms of RPGN?
Definition
Post-strep (50%), SLE, IgA
Term
RPGN with non-streptococcal anti-GMB antibodies are seen in 10% of cases, these often present clinically as...
Definition
Goodpasture syndrome
Term
What is the pauci-immune type of RPGN (type III)?
Definition
No immune complex deposition (as in Type II) or anti-GBM antibodies (as in Type I), associated with ANCA
Term
Name the disorder: nephritic syndrome, hemorrhagic pneumonitis, men in mid-20d, RPGN crescents, linear immunofluorescence
Definition
Goodpasture syndrome
Term
How does focal glomerulonephritis differ from FSGN?
Definition
it is focal and segmental, but changes are inflammatory and proliferative rather than sclerotic. Most often it is an immune complex disease, occurring with SLE, SBE, PAN, Goodpasture, Wegener, IgA, can also be idiopathic
Term
Which syndrome is a hereditary nephritis associated with nerve deafness and ocular disorders like lens dislocations and cataracts?
Definition
Alport syndrome
Term
What causes Alport syndrome?
Definition
mutation in gene for alpha-5 chain of Type IV collagen
Term
Which disorder is most often characterized by benign recurrent hematuria in children, usually following an infection, lasting 1-2 days, and most often of minimal significant?
Definition
IgA nephropathy (Berger disease)
Term
Berger disease can also be a component of which other disease?
Definition
Henoch-Schonlein disease
Term
Name the disorder - characterized clinically by slow progression to CRD, histologically by both basement membrane thickening and cellular proliferation, duplication of GBM into two layers with a tram-track appearance on silver stains?
Definition
Membranoproliferative glomerulonephritis
Term
What is Type I membranoproliferative glomerulonephritis?
Definition
immune complex nephritis, unknown antigen, striking tram-track appearance
Term
What is Type II membranoproliferative glomerulonephritis?
Definition
characterized by irregular electron-dense material deposited within the GBM, C3 is demonstrable and adjacent to but not within the dense deposits, serum C3 characteristically reduced. Possibly caused by IgG autoantibody (C3 nephritic factor) with specificity for C3 convertase of the alternate pathway
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