Nephrotic syndrome Flashcards
What is the definition of nephrotic syndrome ?
It is a glomerular destruction syndrome which is marked by > 3.5 gm of protein loose/ day.
What is the manifestation of hypoalbuminemia in nephrotic syndrome ?
Peripheral and periorbital edema due to reduced oncotic pressure.
What is the cause of hypercoagulability in nephrotic syndrome ?
Lose of anti-thrombin III leading to thrombo-embolic complications.
What is the cause of infections in nephrotic syndrome ?
Lose of immunoglobulins.
What is the cause of frothy and foamy urine production in nephrotic syndrome ?
Lose of lipids through the urine.
What is the composition and microscopic presentation of fatty casts seen in nephrotic syndrome ?
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.
What is the cause of hyperlipedimia in nephrotic syndrome ?
The liver increases lipoprotein synthesis in response to lipid lose through the urine this causes hyperlipedimia.
What is the key pathology of primary nephrotic syndromes?
direct sclerosis and detachment of podocytes on the glomerulus.
What are the three main types of primary nephrotic syndromes ?
- Minimal change disease
- FSGS
- Membranous nephropathy
What is the cause of minimal change disease ?
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.
What is the pathophysiology of minimal change disease?
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.
What is the protein loss phenotype of minimal change disease ?
Selective proteinurea marked by selective loss of albumin.
What is the microscopic features of minimal change disease ?
- Light microscopy- Normal
- Immunoflurafence microscopy -normal
- Electron microscopy- effacement of the foot process of the podcytes can be seen.
What is the only type of minimal change disease that can be treated with corticosteroids ?
ideopathic minimal change disease.
What is the FSGS epidemiology ?
This is the most common type of ideopathic adult onsent primary nephrotic syndrome in african and hispanic adults.
What are the non-ideopathic causes of FSGS?
- HIV, heroin abuse, interferon tx and congenital malformations.
What are the pathophysiolgic changes seen in FSGS ?
The exact pathophysiology is not know. However, there is pdocyte effacement and hyalinosis dueto deposition of lipids and proteins in sclerotic glomerulus.
What are the immunoflurafence findings in FSGS?
It can sometimes be positive for C3, C1, or IgM.
What is the response of FSGS to corticosteroids ?
Inconstant response ergo some patients may progress to CKD.
What is membranous nephropathy ?
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.
What is the pathophysiology of membranous nephropathy ?
Subepithelial deposition of immune complexes causing activates compliment system which damages Podocytes and mesangial cells.
What is the serum marker of membranous nephropathy ?
IgG antibody againts PLA2R.
What is the light microscopic presentation of membranous nephropathy ?
diffuse capillary and glomerular basement membrane thickening due to immune complex deposition.
What is the use of silver methenamine staining in membranous nephropathy ?
It can unravel the irregular expansion from the GBM.
