Norton4

Question 1

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

Answer 1

Tram track appearance.

Membranoproliferative glomerulonephritis

Question 2

See C3 only on Immunofluorescence. No IgG, C1q, or C4.

Answer 2

Membranoproliferative glomerulonephritis type 2

Question 3

EM > deposition of dense material into GBM. Lamina densa becomes irregular and extremely dense, ribbon like

Answer 3

Membranoproliferative glomerulonephritis - type 2

Question 4

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

Answer 4

Membranoproliferative glomerulonephritis both types

Question 5

Genetic or acquired abnormality of immune regulation

Answer 5

IgA nephropathy (Berger disease)

Question 6

Berger disease

Answer 6

IgA nephropathy

Question 7

Increased IgA synthesis after UTI, respiratory or GI infection

Answer 7

IgA nephropathy (Berger disease)

Question 8

In IgA nephropathy (Berger disease), IgA1 (nephritogenic form) deposits in

Answer 8

Mesangium

Question 9

Alternative complement pathway activated causing glomerular injury

Answer 9

IgA nephropathy (Berger disease)

Question 10

Light microscopy> normal glomeruli or Mesangial and endocapillary proliferation

Answer 10

IgA nephropathy (Berger disease)

Question 11

Immunofluorescence > Mesangial deposition of IgA. C3, IgG, and IgM.
EM > dense deposits in mesangium

Answer 11

IgA nephropathy (Berger disease)

Question 12

Mainly older children and young adults. Hematuria (grossly or microscopic). Maybe proteinuria.

Answer 12

IgA nephropathy (Berger disease)

Question 13

Lasts several days and subsides. Recurs every few months. Slow progression to chronic renal failure

Answer 13

IgA nephropathy (Berger disease)

Question 14

Hereditary. Can be AD, AR, or X-linked

Answer 14

Alport Syndrome

Question 15

Most cases are X-linked.

Answer 15

Alport Syndrome

Question 16

Hematuria w/ progression to chronic renal failure, nerve deafness (ears), eye d/o

Answer 16

Alport Syndrome

Question 17

Abnormal alpha3-alpha5 chains of collagen 4

Answer 17

Alport Syndrome

Question 18

X linked has problems with alpha

Answer 18

5

Question 19

Autosomal has problems with alpha

Answer 19

3 and 4

Question 20

Glomeruli always involved. Defective assembly of collagen 4. Hematuria and RBC casts in urine

Answer 20

Alport Syndrome

Question 21

Initial sxs > 5-20 yrs.

Renal failure > 20-50 yrs

Answer 21

Alport Syndrome

Question 22

EM necessary for early lesions.
Early > GBM thinning.
Later > alternating thick and thin GBM. Spluttering and layering of lamina densa.
See nothing on Immunofluorescence

Answer 22

Alport Syndrome

Question 23

Common. Asymptomatic hematuria, maybe proteinuria

Answer 23

Thin BM lesion (benign familial hematuria)

Question 24

Renal function preserved > excellent prognosis.

Thinning of GBM.

Answer 24

Thin BM lesion (benign familial hematuria)

Question 25

Activation of alternative complement pathway only.

Answer 25

Membranoproliferative glomerulonephritis type 2