Nephrotic syndrome

Question 1

What is the definition of nephrotic syndrome ?

Answer 1

It is a glomerular destruction syndrome which is marked by > 3.5 gm of protein loose/ day.

Question 2

What is the manifestation of hypoalbuminemia in nephrotic syndrome ?

Answer 2

Peripheral and periorbital edema due to reduced oncotic pressure.

Question 3

What is the cause of hypercoagulability in nephrotic syndrome ?

Answer 3

Lose of anti-thrombin III leading to thrombo-embolic complications.

Question 4

What is the cause of infections in nephrotic syndrome ?

Answer 4

Lose of immunoglobulins.

Question 5

What is the cause of frothy and foamy urine production in nephrotic syndrome ?

Answer 5

Lose of lipids through the urine.

Question 6

What is the composition and microscopic presentation of fatty casts seen in nephrotic syndrome ?

Answer 6

These are hyline from the dead epithelial cells that contained fat globules. The presence of cholesterol in these fatty casts gives them malatese cross appearance under polarized light.

Question 7

What is the cause of hyperlipedimia in nephrotic syndrome ?

Answer 7

The liver increases lipoprotein synthesis in response to lipid lose through the urine this causes hyperlipedimia.

Question 8

What is the key pathology of primary nephrotic syndromes?

Answer 8

direct sclerosis and detachment of podocytes on the glomerulus.

Question 9

What are the three main types of primary nephrotic syndromes ?

Answer 9

• Minimal change disease
• FSGS
• Membranous nephropathy

Question 10

What is the cause of minimal change disease ?

Answer 10

This is the most common cause of primary nephrotic syndrome in children. The etiology is most often idiopathic, but can be triggered by recent infection, vaccination, bee sting etc.

Question 11

What is the pathophysiology of minimal change disease?

Answer 11

The T cell mediated immune system activation leads to the release of GPF which destroys the foot proceses of the podocytes leading to pdocyte effacement which disrupts the negative charge barrier that incurs the leakage of negatively charged albumin.

Question 12

What is the protein loss phenotype of minimal change disease ?

Answer 12

Selective proteinurea marked by selective loss of albumin.

Question 13

What is the microscopic features of minimal change disease ?

Answer 13

• Light microscopy- Normal
• Immunoflurafence microscopy -normal
• Electron microscopy- effacement of the foot process of the podcytes can be seen.

Question 14

What is the only type of minimal change disease that can be treated with corticosteroids ?

Answer 14

ideopathic minimal change disease.

Question 15

What is the FSGS epidemiology ?

Answer 15

This is the most common type of ideopathic adult onsent primary nephrotic syndrome in african and hispanic adults.

Question 16

What are the non-ideopathic causes of FSGS?

Answer 16

• HIV, heroin abuse, interferon tx and congenital malformations.

Question 17

What are the pathophysiolgic changes seen in FSGS ?

Answer 17

The exact pathophysiology is not know. However, there is pdocyte effacement and hyalinosis dueto deposition of lipids and proteins in sclerotic glomerulus.

Question 18

What are the immunoflurafence findings in FSGS?

Answer 18

It can sometimes be positive for C3, C1, or IgM.

Question 19

What is the response of FSGS to corticosteroids ?

Answer 19

Inconstant response ergo some patients may progress to CKD.

Question 20

What is membranous nephropathy ?

Answer 20

It can be primary or secondary to SLE drugs such as NSAIDs, Gold, Penicillamine. It can also occur secondary to HBV. HCV and syphilis. In addition, it can also occur secondary to solid tumors such as colorectal carcinoma.

Question 21

What is the pathophysiology of membranous nephropathy ?

Answer 21

Subepithelial deposition of immune complexes causing activates compliment system which damages Podocytes and mesangial cells.

Question 22

What is the serum marker of membranous nephropathy ?

Answer 22

IgG antibody againts PLA2R.

Question 23

What is the light microscopic presentation of membranous nephropathy ?

Answer 23

diffuse capillary and glomerular basement membrane thickening due to immune complex deposition.

Question 24

What is the use of silver methenamine staining in membranous nephropathy ?

Answer 24

It can unravel the irregular expansion from the GBM.

Question 25

What are the immune complex phenotypes found in membranous nephropathy ?

Answer 25

Under imunofluracence granular IgG and C3 can be seen.

Question 26

what is the response of Membranous nephropathy to corticosteroids ?

Answer 26

Very poor as patients may easily progress to CKD.

Question 27

What is the most common cause of ESRD ?

Answer 27

diabetic glomerulonephropathy.

Question 28

what is the pathophysiology of diabetic glomerulonephropathy ?

Answer 28

Increased circulating glucose sticks to the proteins in the blood this causes thickening of the basement membrane of the efferent renal arterioles which increases intraglomerular pressure. This will cause dilation of the afferent arteriole resulting in increased GFR or hyperfiltration. This will cause expansion of the glomerulus by structural matrix induction by the mesangial cells.

Question 29

What are Kimmelstiel- Willson nodules?

Answer 29

These are eosinophilic nodular glomerulosclerotic lesions seen in diabetic nephropathy.

Question 30

What are the light microscopic findings in diabetic glomerulonephropathy ?

Answer 30

• Mesangial expansion
• GBM hypertrophy
• Kimmelstiel - Willson nodules.

Question 31

What is the screening test for diabetic glomerulonephropathy ?

Answer 31

early screening for microalbuminurea.

Question 32

What is amyloid glomerulonephropathy ?

Answer 32

It is a secondary nephrotic syndrome due to the deposition of misfolded protein clumps that stain pink under light microscopy with congo red stain.

Question 33

What is the appearance of amyloids under polarized light ?

Answer 33

apple green deposits localised in the mesangium.

Question 34

What are the Tx in nephrotic syndrome ?

Answer 34

• reducing salt and fatty food intake.
  *Corticosteroids
• Immune modulator drugs such as cyclophosphamide, calcineurin inhibitors, and mycophenolate mofetil.
• RAAS inhibitors.
• Diuretics.