Proteinuric and nephrotic conditions and secondary glomerular disease

Question 1

What is seen in nephrotic syndrome? (6)

Answer 1

1. massive proteinuria
2. hypoalbuminaemia
3. oedema
4. hyperlipidaemia
5. +/- lipiduria
6. not associated with increased cellularity

Question 2

What is the most common cause of nephrotic syndrome in children?

Answer 2

minimal change disease

Question 3

What is seen on light microscopy in minimal change disease?

Answer 3

normal

Question 4

What is seen on direct immunofluorescence in minimal change disease?

Answer 4

normal

Question 5

What is seen on electron microscopy in minimal change disease?

Answer 5

diffuse effacement of podocyte foot processes

Question 6

What is the proteinuria like in minimal change disease? (2)

Answer 6

1. highly selective proteinuria

2. small albumin&raquo_space;> others

Question 7

What is thought to be the cause of minimal change disease? (4)

Answer 7

1. some soluble permeability factor
2. immature T cells
3. CD34+ stem cells
4. cytokine mediated

Question 8

What is the prognosis of minimal change disease?

Answer 8

95% of children responds well to steroids

Question 9

What are the different settings of focal segmental glomerular sclerosis? (4)

Answer 9

1. primary/idiopathic
2. adaptive
3. secondary
4. glomerular tip lesion variant

Question 10

What is the cause of primary/idiopathic focal segmental glomerular sclerosis? (2)

Answer 10

1. cytokine

2. permeability factor

Question 11

What is the cause of adaptive focal segmental glomerular sclerosis?

Answer 11

hydrodynamic shear stress

Question 12

What is the cause of secondary focal segmental glomerular sclerosis? (5)

Answer 12

1. Heavy (obesity)
2. HIV
3. Heroin
4. Haematological - sickle cell
5. secondary to Hyerfiltration

Question 13

What is the proteinuria like in focal segmental glomerular sclerosis?

Answer 13

less selective than minimal change disease

Question 14

What is seen on light microscopy in focal segmental glomerular sclerosis?

Answer 14

1. juxtamedullary deep glomeruli

2. focal segmental sclerosis

Question 15

What is the prognosis of focal segmental glomerular sclerosis?

Answer 15

50% end stage renal failure in 10 years

Question 16

What is associated with membranous nephropathy? (7)

Answer 16

1. HBV
2. HCV
3. Lupus
4. Hodgkin’s lymphoma
5. syphilis
6. malaria
7. schistosomiasis

Question 17

What vessel is most susceptible to thrombosis due to membranous nephropathy?

Answer 17

renal vein

Question 18

Why is thrombosis a risk in membranous nephropathy?

Answer 18

due to loss of antithrombin III un urine

Question 19

What is the pathology of membranous nephropathy?

Answer 19

antigen-antibody in glomerular basement membrane and subepithelial to the podocytes

Question 20

What is seen on immunofluorescence with membranous nephropathy?

Answer 20

glomerular and subepithelial immune complexes made of C3 and IgG

Question 21

How does proteinuria occur in membranous nephropathy?

Answer 21

1. C3 is part of complement
2. membrane attack complex injures podocyte
3. non-selective proteinuria

Question 22

What type of proteinuria occurs in membranous nephropathy?

Answer 22

non-selective proteinuria with loss of albumin and globulin

Question 23

What is seen on light microscopy in membranous nephropathy? (2)

Answer 23

1. no increase in cellularity

2. thickened glomerular basement membrane

Question 24

What is seen on electron microscopy in membranous nephropathy?

Answer 24

1. subepithelial immune complex deposition
2. podocyte foot process effacement
3. thickened glomerular basement membrane in “spikes and domes”

Question 25

What is the aetology of membranous nephropathy?

Answer 25

1. primary - idiopathic

2. secondary to syphilis, HBV, HCV , malaria, schistosomiasis, cancer, hodgkin’s, SLE