Nephrotic syndrome Flashcards
significant proteinuria
nephrotic syndrome
hematuria and glomerulonephritis
nephritic syndrome
definition of proteinuria
> 3.5 gm in 24 hr
definition of hypoalbuminemia in salbumin
< 3.5 gm/dl
what do we see in nephrotic syndrome
- proteinuria
- hypoalbuminemia
- Edema
- hyperlipidemia and lipiduria
urine sediment in nephrotic syndrome
negative- non-active nl kidney function and blood pressure
Morbidity associated with Nephrotic syndrome
- Edema and effusions
- loss of IgG and complement
- Loss of ATIII
- loss of Vitamin D
- Hyperlipidemia and vascular disease
pathogenesis of proteinuria
injury to podocytes by either genetics, depositions or direct injury by ab, cytokines and toxins
3 primary glomerular diseases with nephrotic syndrome
- MCD- minimal change disease
- FSGS- focal segmental glomerulosclerosis
- MN- membranous glomerulopathy
secondary glomerular diseases with nephrotic syndrome
- diabetes
- SLE
- Amyloidosis
- medications- NSAIDS + Lithium
- Tumors/ infections/ vaccinations
three major causes of ESRD
- diabetes
- HTN
- Glomerular disorders
what are the advantages and disadvantages of dipstick ruine
- Pro:
1. quick
2. semi-quantitive
3. detects mainly labumin - Cons:
1. does not detect microalbumin
2. does not detect abnormal proteins
spot urine
protein to creatinine ratio
what do we expect to see in light microscopy for a pt. that has minimal change disease?
normal glomerulous
what do we see in electron microscopy with a pt. that hasa minimal change disease
abnormal podocytes: flat and unhappy
MCD:
- most common cause of
- peak incidence in children occur at ages
- in adults it is caused by the use of _____ which lead to immune dysfunction and cytokine/ direct toxicity
- hereditary chances
- response to steroids?
- prognosis?
- most common cause of NS in children
- peak incidence in children occur at ages 2-6
- in adults it is caused by the use of NSAIDS which lead to immune dysfunction and cytokine/ direct toxicity
- hereditary cases are rare and they are due to nephrin mutations
- responsive to steroids 6. good prognosis
FSGS:
- most frequent in
- at risk populations
- possible genetic component?
- most common cause of
- primary form is
- secondary form is ________ due to
- prognosis?
- recurrence?
- most frequent in adults
- at risk populations- hispanics/ high BMI/ African Americans/ HIV +
- possible genetic component- yep
- most common cause of NS in the US
- primary form is idiopathic with some semblance to MCD
- secondary form is hyperfiltration due to obesity/solitary kidney/ transplant kidney
- prognosis is poor with progression to ESRD
- recurrence is possible in transplants
Membranous nephropathy:
- occurs mostly in
- _____ renal function at presentation
- autoimmune disease
- rare cases associated with __________ (1 in 10 pt) , ___________ and _____________
- disease is acute or chronic
- response to steroids
- occurs mostly in adults 4-5th decade
- normal renal function at presentation
- autoimmune disease: most cases IgG class 4 against phospholipase A2-receptor (anti-PLA2R)
- rare cases associated with cancer associated ab (1 in 10 pt) , collagen- vascular disease and infection
- disease chronic
- response to steroids is poor
Diabetic nephropathy:
- most common cause of
- up to 1/3 of DMI and DMII will
- first sign of diabetic nephropathy
- ________ develops in about 20yr
- most common cause of ESRD in the US (diabetes mellitus)
- up to 1/3 of DMI and DMII will develop diabetic nephropathy and kidney failure
- first sign of diabetic nephropathy is microalbuminuria
4 overt proteinuria develops in about 20yr
course of Diabetic glomerulosclerosis
- hyperglycemia related to insulin deficiency
- non-enzymatic glycosylation of proteins
- biochemical changes of GBM
- increased synthesis of collagen IV
- GBM thickening and increased mesangial matrix
- Glomerulosclerosis
apple green Birefringence
amyloidosis
amyloidosis:
- deposition of
- disease of
- deposition in the glomeruli leads to
- deposition in heart leads to
- major types: ______ associated with lymphoma and _______ associated with Liver
- deposition of abnormally folded proteins- amyloid
- disease of adults
- deposition in the glomeruli leads to NS and kidney failure
- deposition in heart leads to CHF
- major types: AL which is deposition of immunoglobulin light chains associated with lymphoma and AA- deposition of serum amyloid-A-protein associated with Liver
oval fat bodies
focal segmental glomerulosclerosis-FSGS
focal segmental glomerulosclerosis-FSGS
membranous nephropathy- uniformly thick GBM
silver stain for collagen 4- not really distinct pt. has membranous
immunofluorescence with IgG showing capillary loops in patient with membranous n
notice the subepithelial electron dense deposits- membranous nephropathy
membranous nephropathy pathogenesis
nodular sclerosis seen in patients with advanced diabetes- nodular diabetic glomerulosclerosis
nodular diabetic glomerulosclerosis- kimmelstiel wilson disease
EM of Diabetic nephropathy
pt. with amyloidosis
congo red stain of pt. with amyloidosis
polarized light of amyloidosis
fibrillary deposits in EM seen in amyloidos
