Norton4 Flashcards
Duplication of BM. New BM synthesis b/c of subendothelial immune complex deposits. Interposition of Mesangial cells into duplicated BM gives appearance of split BM
Tram track appearance.
Membranoproliferative glomerulonephritis
See C3 only on Immunofluorescence. No IgG, C1q, or C4.
Membranoproliferative glomerulonephritis type 2
EM > deposition of dense material into GBM. Lamina densa becomes irregular and extremely dense, ribbon like
Membranoproliferative glomerulonephritis - type 2
Affects children, teenagers, and young adults. Slowly progressive, unremitting. Nephrotic syndrome with hematuria and proteinuria. 50% > chronic renal failure in 10 yrs. 90% > recurrence after organ transplant
Membranoproliferative glomerulonephritis both types
Genetic or acquired abnormality of immune regulation
IgA nephropathy (Berger disease)
Berger disease
IgA nephropathy
Increased IgA synthesis after UTI, respiratory or GI infection
IgA nephropathy (Berger disease)
In IgA nephropathy (Berger disease), IgA1 (nephritogenic form) deposits in
Mesangium
Alternative complement pathway activated causing glomerular injury
IgA nephropathy (Berger disease)
Light microscopy> normal glomeruli or Mesangial and endocapillary proliferation
IgA nephropathy (Berger disease)
Immunofluorescence > Mesangial deposition of IgA. C3, IgG, and IgM.
EM > dense deposits in mesangium
IgA nephropathy (Berger disease)
Mainly older children and young adults. Hematuria (grossly or microscopic). Maybe proteinuria.
IgA nephropathy (Berger disease)
Lasts several days and subsides. Recurs every few months. Slow progression to chronic renal failure
IgA nephropathy (Berger disease)
Hereditary. Can be AD, AR, or X-linked
Alport Syndrome
Most cases are X-linked.
Alport Syndrome
Hematuria w/ progression to chronic renal failure, nerve deafness (ears), eye d/o
Alport Syndrome
Abnormal alpha3-alpha5 chains of collagen 4
Alport Syndrome
X linked has problems with alpha
5
Autosomal has problems with alpha
3 and 4
Glomeruli always involved. Defective assembly of collagen 4. Hematuria and RBC casts in urine
Alport Syndrome
Initial sxs > 5-20 yrs.
Renal failure > 20-50 yrs
Alport Syndrome
EM necessary for early lesions.
Early > GBM thinning.
Later > alternating thick and thin GBM. Spluttering and layering of lamina densa.
See nothing on Immunofluorescence
Alport Syndrome
Common. Asymptomatic hematuria, maybe proteinuria
Thin BM lesion (benign familial hematuria)
Renal function preserved > excellent prognosis.
Thinning of GBM.
Thin BM lesion (benign familial hematuria)
Activation of alternative complement pathway only.
Membranoproliferative glomerulonephritis type 2
