Nephrotic syndromes Flashcards
Nephrotic characteristics?
1.RBC barrier mantained (NO RBC cast and NO HEMATURIA). No Glomerular endothelium inflamation.
2.Protein Barrier ruptured–>podocytes( >3.5 g/day )in a 24HR. urine test
3.Hyperlipedemia (increase liver activity)–>Fatty cast (oval bodies) or Maltose cross (polarized light)
4.DECR albumin
5.Edema (decr plasma oncotic pressure)
6.Decr Ig (incr risk infections)
7.Hypercoaguble state (decr, Anti-Thrombin3)
Nephrotic table….
Types of nephrotic syndromes
Primary (direct sclerosis and inflam to podocytes)
1.Minimal change, 2. Focal segmental, 3. Membranous Nephropathy
Secondary (Indirect)
1.Diabetic Nephropathy
2. Systemic amyloidosis
Light Microscopy (LM) and Immunoflorascense unchanged (IF)
Minimal change disease
(EFFACEMENT)=flatten out podocytes.
What type of Nephropathy present with SELECTIVE PROTENURIA?
MINIMAL CHANGE DISEASE (only damages the foot processes of podocytes and nothing else).
4 I’s Nephropathy + (Minimal Change disease)
**Idiopathic
recent Immnunization
**recent Infection
* Immune stimulus (Bee sting)
Hodkins Lymphomas; rel. of anbormal cytokines by reedsternberg cells. –>T cell cytokines rel
Hodkin Lymphoma cells and markers
CD 15+ and CD 30+ with 2 owls eye.
Minimal change disease other name
Lipoid nephrosis
Diagnosis
Child treated with corticosteroids with recent history of immunization that presented with: edema, >300mg cholesterol, >3.5g/ day protein and negative IF and LM.
Minimal change disease
-Ass. children most commonly
-Adults can have it
-Corticosteroid treatment is effective.
Why corticosteroids work in Minimal change disease?
Inhb. NF-KB (transcripton factor for potent cytokines like TNF alpha), Minimal change being related to cytokine release, greatly supress it.
Effacement child
Lipoid nephrosis
Histological Diag.
- <50% of biopsy glomerulus affected, only segments affected (not entirley) and thickening of glomerulus
- -Hyaline and effacement presentt.
(FSGS)Focal Segmental Glomerulosclerosis.
non-selective protenuria in Nephrotic syndromes
-Focal segmental glomerulosclerosis
-Membranous nephropathy
FSGS 1* and 2* associations.
1* Idiopathic.
2* Heroin, HIV infect, Congenital malformations, sickle cell, interferon tretament (Hepatitis B and C), obesity
mutations of nephrin and porocin protein located on slit diamphragm
1* FSGS association
1.LM:sclerosis + Hyalinosis
2.Pinkage color, what stain?
1.FSGS
2.PAS stain
FSGS
-Sgmental Focal thickening (protein deposits)+ Hyalinosis (PAS stain)
Prognosis od FSGS
within 10 yrs 50% develop ENRF
Membranous nephropathy other name
Membrane glomerulonephritis
Membranous glomerulonephritis 1*
Antibodies (IgG) towards phospholipase A2 (M TYPE),present serum
-phospholipase A2 are present in podocytes.
Where are phospholipase A2 recep located?
membrane of podocytes
Membranous nepgropathy 2*
-Drugs (NSAIDS, penincillamine, Gold–.R.A)
-Infection (Hepatitis B and C and syphylis)
-SLE
-Solid tumors (COLON most comm, lung , melanoma)
Membrabous Glomerulonephritis
Silver stain
Describe
Membranous Nephropathy
-Diffuse capillaries, GBM thickening (Complex deposition)+ NO HYPERCELLULARITY
SUB-EPITHELIAL DEPOSITS (imm complex) + spike and dome app
Membranous nephropathy
Membranous Nephropathy Prognosis
slow onset progression (many yr). Normal creatine .
-Exception is in Thrombosis (more quickly lose of function; ELEVATED creatinine)
site of thrombosis in Membranous Nephropathy
RENAL VEIN (quick onset of depression)
Membranous nephropathy response to corticosteroids
POOR
DIAGNOSIS
Systemic Amyloidosis
-Most common side of aggregation is kidney
-LM: Congo red stain (PINK AMYLOID DEPOSITS)
-Polorized ligth:apple green
-Locolized in mesangium
Most common cause of End stage renal disease in US
Diabetic Glomerulonephritis
Cause of Diabetic glomerulonephritis
Non-enzymatic glycation
Diagnosis
Wilson nodules+mesangial expansion+Glomerular basement membrane thickening.
Diagnosis
Obtrs. efferent arterioles due to non enzymatic glycation–> afferent arteriole dilation , Further incr pressure->INCR GFR (Hyperfiltration)–>mesangial cell expansion
Diabetic Glomerulonephritis
Beta 2 microglobulin deposition
Amyloidosis
Ass syndrome with amyloidosis
Carpal Tunnel synddrome
Pathophysiology of carpal tunnel syndrome
-entrapment of median nerve–>parasthesia, but sensory is spared due to cutaneous branch enters hand external to carpal tunnel.
-Thenar muscle atrophy .
What related secondary nephroticc syndrome (Diabetic and Amyloidosis)
Both are associated with mesangium.
Tram Track app in silver stain
Membranous Glomerulonephritis.
Where do you see also Maltose cross?
Babbesia and Minimal change disease
Effacemnet do you see it where
Membronous nephropathy (Hyaline) and Minimal change.
Crecent shape of RPGN made up off what and what cell proliferates?
Made primarly of Fibrin but parietal cells over grow
Lumpy-bumby pattern on sliver stain
Focal segmental glomerulonephritis, most likely** HIV Nephropathy** telated FSGS
Lung-Hepatitis-Rheumatid Arthritis
Membranous Nephropathy 2*
Nephrin Factor nephropathy
Membranous Proliferative Type 2
What does your nephritic factor do?
Overactivates C3 convertase–>depletion of C3 in serum.
Diagnosis
Intramembronous deposition of a ribonlike homogenous extremly electron dense material of unknown material.
Membranous Proliferative Glomerulonephritis Type 2
MPGN ass…
Hepatitis C and B.
Pathogenesis of Type 1 MPGN..
-Chronic infection will promote formation of immune complexes causing deposition sub-Endothelial–>mesangial ingrowth “interposition”–>TRAM-TRACK app
-Activates classical and alternate pathways.
-All due to chronic localization of immune complex.
Pattern of deposition in Type 1 MPGN..
Sub-Endothelial with mesangial interposition, cretaing TRAM-TRACK app.
-Sub-Endothelial deposits with interposed mesangial cells cretaing TRAM-TRACK app.
-Homogenous dense electron deposits of unkonwn material
-MPGN Type 2
Mixed cryoglobulinic vasculitis ass with what nephritic syndrome?
MPGN Type 1
Diagnosis
Triad of weakness, arhthralgia, palbable purpura
-Biospy you see TRAM TRACK app.
Mixed Cryoglobulinic Vasculitis(ass with Hepatitis C)
Pathogenesis of Mixed Cryoglobulinic Purpura
Abn IgG antibodies that agglutinate RBC in cold temp–> small clot formation that may lead to skin cotting (palbable purpura), kidney clotting (glomerulonephritis), joint clotting (arthritis).
Pathogenesis of diabetus…
How does Uncontrolled Diabetus Mellitus cause Hyperfiltration.
-non ezymatic glycation of efferent arterioles cause narrow lumen–>decr blood supply to kidney–>afferent arteriole dilation–>combination of both leads to HYPERFILTRATION –>incr glomerular pressure–>glomerulat hyperthrophy+incr capillary surface area
What does sorbitol to Fructose consumes and effect?
NADPH.
-NO Gluthaione is reduced–>Incr ROS
How does INCR ROS cause damage?
Lipid peroxidation, Protein oxidative modfification,DNA damage.
How does hyperglycemia cause Retinpathy?
-by your Advanced Glycated products promoting release of VEGF –>leads to retinopathy.
How does hyperglycemia accelerate atherogenesis?
By** LDL trapping** (in your endothelial cells)
What product does hyperglycemia form?
-Advanced Glycated Products.
-via **Non-enzymatic Glycation. **
What product does hyperglycemia form?
-Advanced Glycated Products.
-via **Non-enzymatic Glycation. **
1.First onset of Diabetic kidney disease is what?
2.Timeline from diagnosis of Diabetus
1.micro-albuminuria (30-299mg/day).
2.5-10yr.
-Clinical diabetic kidney?
-Timeline from micro-albuminuria?
Clinical albiminuria (>300mg/day)
-5-10yrs from onset of micro-albuminuria.
What the role of angiotensin 2 in kidney?
-constricts efferent arterioles.
What drugs can we give to slow down progression from micro-albuminuria to clinical albuminuria?Why?
-ACE inhb. and ARB–>dilate efferent arterioles (reduce Hyperfiltration)
Diagnosis
Broad masses of eosinophilic acellular material in the glomerulus
Amyloidosis (NO basement membrane thickening)
Diagnosis
Chronic Hemodyalisis ass with signs of tingling in hands…
AMYLOIDOSIS (ass with Carpal Tunnel Syndrome)
