Robbins Ch 20 Part I Flashcards
Azotemia is mostly due to?
What is the BUN/Cr ratio for prerenal azotemia?
What is the ratio for postrenal azotemia?
1) Decreased GFR
2) BUN/Cr > 20
3) BUN/Cr < 20
How does nephritic syndrome differ from nephrotic syndrome?
Nephritic syndrome is associated with hematuria and HTN while Nephrotic syndrome is associated with severe proteinuria
Which syndrome shows classic presentations of acute poststreptococcal glomerulonephritis?
Which causes subepithelial deposits?
1) Nephritic syndrome
2) Nephrotic syndrome
What is acute kidney injury characterized as?
Chronic kidney disease?
1) Rapid decline in GFR (hours to days)
2) Diminished GFR that is persistently less than 60ml/min/1.73m2 for at least 3 months and/or persistent albuminuria
End stage renal disease is the terminal stage of?
Uremia
Urinary Tract infections are characterized by?
Bacteriuria and pyuria (bacteria and leukocytes in the urine, respectively)
The visceral epithelial cell layer of the glomerulus is important for the maintenance of?
Glomerular barrier function
In most forms of glomerular injury, loss of normal slit diaphragms is a key event in the development of?
Proteinuria
What is associated with focal nodular glomerulosclerosis?
Diabetes
What is characterized by an accumulation of material that is homogenous and eosinophilic by light microscopy?
Hyalinosis
What is characterized by deposition of ECM collagen and seen with a trichrome stain (blue)?
Sclerosis
Membranous Nephropathy is due to antibody binding to?
It forms characteristic deposits of immune complexes along what aspect of the basement membrane?
What appearance does it have on electron microscopy?
How does it respond to corticosteroids?
What is the principal immunoglobulin deposited in cases of primary membranous nephropathy?
1) PLA2R (phospholipase A2 receptor)
2) Subepithelial
3) Spike and Dome
4) Not well
5) IgG4
What causes simultaneous lung and kidney lesions resulting in hematuria and hemoptysis?
Goodpasture syndrome
What is the pattern of deposition on immunofluorescence for membranous nephropathy?
For Goodpasture syndrome?
1) Granular pattern of deposition
2) Linear pattern of deposition
What is the most common cause of nephritic syndrome in adults?
What is it treated with?
1) Focal Segmental Glomerulosclerosis
2) Renin-Angiotensin System Inhibitors
There is a strong correlation between the decline of renal function and the amount of?
Tubulointerstitial fibrosis
What are the original infections typically seen 1-4 weeks prior that can lead to nephritic syndrome?
1) Post-streptococcal A (pyogenes) Beta-Hemolytic Pharyngitis
OR
2) Skin Infection
What antibody has been localized to the “hump-like” deposits in the subepithelial space characteristic of PSGN?
Antibody against pyogenic exotoxin B (SpeB)
What is seen on light microscopy for nephritic syndrome?
What is seen on immunofluorescence?
What is seen on electron microscopy?
1) Enlarged and Hypercellular glomeruli
2) Granular deposits of IgG and C3
3) Electron dense deposit on the subepithelial side causing an appearance of “humps”
What do the labs show for nephritic syndrome?
1) Elevated ASO titers
2) Low serum complement levels
Post-infectious glomerulonephritis due to staphylococcal infections differs by sometimes producing immune deposits containing?
IgA rather than IgG
Rapidly Progressive Glomerulonephritis is characterized by?
Severe glomerular injury associated with the formation of crescents in most glomeruli
The crescents seen in rapidly progressive glomerulonephritis are proliferations of?
Parietal epithelial cells lining the bowman capsule
Type I rapidly progressive glomerulonephritis can be caused by?
What HLA is it associated with?
1) Goodpasture’s
2) HLA-DRB1 (MHC Class II)
Type II rapidly progressive glomerulonephritis can be caused by?
SLE or Henoch-Schonlein
Type III rapidly progressive glomerulonephritis can be caused by?
How is it diagnosed?
1) Granulomatosis with polyangiitis
2) C-ANCA
What pattern is found on immunofluorescence for Type I rapidly progressive glomerulonephritis?
For Type II?
For Type III?
1) Linear
2) Granular
3) None
Which type of rapidly progressive glomerulonephritis requires emergency plasmapheresis?
Type II
What is characterised by derangement of glomerular capillaries with increased permeability to protein, with resultant severe proteinuria?
Nephrotic syndrome
Where is edema from nephrotic syndrome most prominent?
Periorbital regions
Nephrotic syndrome patients are at an increased risk of infection, especially for?
Staphylococcal and pneumococcal infections
A potential consequence of nephrotic syndrome is renal vein thrombosis which can cause?
Varicocele on the LEFT in males
What is the most common cause of nephrotic syndrome in kids?
Minimal change disease
What systemic disease are the most common causes of nephrotic syndrome in adults?
1) SLE
2) Diabetes
What is found in the visceral epithelial cells on electron microscopy for minimal change disease?
What is found on light microscopy?
1) Effaced foot processes (podocytes)
2) Normal glomerulus
How does minimal change disease respond to steroid therapy?
Very well
What does minimal change disease result in?
Selective proteinuria
What is the most common cause of nephrotic syndrome in adults in the US, especially black and Hispanic patients?
Focal Segmental Glomerulosclerosis
How does Focal Segmental Glomerulosclerosis differ from minimal change disease?
Focal Segmental Glomerulosclerosis has a poor response to corticosteroids
The possible mutations associated with Focal Segmental Glomerulosclerosis all localize to?
1) Slit diaphragm
2) Adjacent podocyte cytoskeletal structures
What autosomal recessive mutation is a cause of Focal Segmental Glomerulosclerosis?
What autosomal dominant mutation?
What mutation is associated with adult onset FSGS?
What mutation increases the risk of FSGS in African Americans?
1) Podocin (NPHS2)
2) Alpha-actinin 4
3) TRP6
4) Apolipoprotein L1 (APOL1)
What is characterized by proliferation and hypertrophy of glomerular visceral epithelial cells and is typically associated with prominent tubular injury with formation of microcysts?
Collapsing glomerulosclerosis
What should be done for definite diagnosis of Focal Segmental Glomerulosclerosis?
Biopsy
The severe form of the collapsing variant of Focal Segmental Glomerulosclerosis is called?
HIV-associated nephropathy
HIV-associated nephropathy is associated with high circulating levels of?
IFN-alpha
Which type of Membranoproliferative Glomerulonephritis is associated with the alternative complement activation?
70% of patients have what autoantibodies?
Electron microscopy shows a permeation of the lamina densa of the GBM by?
1) Type II (dense deposit disease)
2) C3 nephritic factor (C3NeF)
3) An extremely electron dense ribbon of material
In both types of Membranoproliferative Glomerulonephritis there is proliferation in the?
What does this show on light microscopy?
1) Mesangium and capillary loops
2) GBM has “double contour” or tram-track appearance
A secondary Membranoproliferative Glomerulonephritis due to a chronic immune complex disorder such as SLE, Hep B/C, or HIV is almost always which type?
Type I
What is the most common cause of glomerulonephritis worldwide?
IgA Nephropathy (Berger Disease)
Where are the IgA deposits found with IgA nephropathy?
They can form complexes and lead to the activation of?
1) Mesangium
2) Alternative complement pathway (C3)
How does IgA Nephropathy present most commonly?
Patients with recurrent hematuria following a respiratory or GI tract infection
What is the Alport Syndrome mnemonic?
A: “A” is for Alport
LP: “LP” is a record, that you listen to; reminds you of deafness
O: “O” is for ocular problems
R: “R” Renal failure
T: “T” Thickening of BM and Type IV Collagen
What is the inheritance pattern of Alport syndrome?
The defective glomerular basement membrane is a result of a defect in?
What is found on electron microscopy?
This leads to what appearance on EM?
1) X-linked disorder (85% of the time)
2) Type IV collagen synthesis
3) Alternating thickening and thinning of glomerular basement membrane
4) Basket weave
What is the most common presenting sign of Alport syndrome?
Gross or microscopic hematuria
What is the end point of all Nephrotic and Nephritic Syndromes?
Chronic Glomerulonephritis
Chronic glomerulonephritis is characterized by extensive hyalinization and fibrosis (obliteration) of the glomeruli, demonstrated by?
Large amount of collagen on a trichrome stain
