Pathology COPY Flashcards
nephrotic syndromes
- minimal change disease
- FSGS
- membranous nephropathy
- diabetic nephropathy
- amyloidosis
nephritic syndromes
-post-strep
glomerulonephritis
-berger’s (IgA) nephropathy
-diffuse proliferative glomerularnephritis
-crescentic GN (RPGN)
-Alport syndrome
-Membranoproliferative glomerulonephritis (MPGN)
nephrotic w/ foot process effacement
minimal change disease
FSGS
immune complex deposition
membranous nephropathy
membranoproliferative glomerulonephritis
mesangium deposition
diabetic nephropathy
amyloidosis
SLE related nephrotic syndrome
membranous nephropathy
SLE related nephritic syndrome
diffuse proliferative glomerulonephritis
*MOST COMMON SLE SYNDROME
thin/split BM
eye/ear disturbances
ALPORT SYNDROME
type IV collagen defect, nephritis
selective proteinuria
minimal change disease
loss of albumin due to heparin sulfate loss
exquisite sensitivity to steroids
minimal change disease
minimal change sx but no response to steroids
FSGS
HIV, sickle cell, heroin
FSGS
- thick BM
- subEPITHELIAL IC deposits (spike and dome)
- absence of hypercellularity
- (good prog in child, bad prog in adults
membranous nephropathy
anti-phospholipase A2 receptor Abs
membranous nephropathy
FSGS response to steroids
POOR, prog to chronic renal failure
membranous nephropathy response to steroids
POOR, prog to chronic renal failure
- recurrent hematuria
- thick BM, focal sclerosis (mesangial)
- nodular sclerosis (KIMMELSTIEL-WILSON BODIES)
- preferential hyaline arteriosclerosis in EFF a.
diabetic nephropathy
diabetic nephropathy MOA
hyperglycemia –> nonenzymatic glycation of tissue proteins –> mesangial expansion
GBM thickening/inc perm
hyperfiltration (glomerular HTN and inc GFR) –> glomerular hypertrophy and scarring
most comm cause ESRD in US
diabetic nephropathy
deposits in mesangium (B-pleated sheets)
- congo red –> apple-green birefringence
amyloidosis
SLE
solid tumors
Hepatitis (B/C)
nephrotic syndrome
membranous nephropathy
nephrOtic sx
- proteinuria (>3.5 g/dl) –> FROTHY urine
- hypOalbuminemia
- hyperlipidiemia (fatty casts/oval bods)
- hypercoaguability (AIII loss)
- dec plasma oncotic P so fluid leaks into tissues –> dec ECV/GFR, inc RAAS –> EDEMA (LE, peri-orbital)
- infections (loss of Ig)
tx of which nephritic syndrome is usu supportive bc IC dissipate out
post-strep glomerulonephritis
M protein virulence fx
peripheral/periorbital edema
HTN
cola urine
post-strep glomerulonephritis
2-3 weeks post infection
post-strep glomerulonephritis
subEPITHELIAL humps ("lumpy-bumpy"), deposition capillary loops w/ neutrophils
post-strep glomerulonephritis
dec complement levels (C3) due to infection
post-strep glomerulonephritis
type II membranoproliferazive glomerulonephritis
nephrITIC syndrome
- INFLAMM process damaging ENTIRE glomeruli
- damaged filtration barrier –> dec GFR –> inc BUN/Cr
- oliguria
- inc hydrostatic P –> HTN, edema
- filtration barrier to RBCs/protein lost (HEMATURIA)
- ->salt retention (periorbital edema +HTN)
- EM: hypercellular (neutrophils)
- UA: RBC casts (glomerular bleeding), dysmorphic RBCs
all nephritic syndrome have sub_____ deposition, except _______, which has sub_____ deposition.
all nephritic syndrome have subENDOTHELIAL deposition, except POST-STREP GN, which has subEPITHELIAL deposition.
repeated mucosal (resp/GI) infec –> Ig deposition in. mesangium –> repeated hematuria
slowly worsening renal func (BUN/Cr) over time
IgA nephropathy
nephritic syndrome days after infec
IgA nephropathy
cirrhosis
celiac
HIV
IgA nephropathy
IgA nephropathy w/ extra renal involvement
Henoch-Scholein Purpura
(HSPI)
deposition occurs outside kidney (GI, skin)
IgA nephropathy - glomeruli only
Berger disease
capillary loops THICKENED
subendothelial deposition
diffuse proliferative glomerulonephritis
fever/rash/arthritis
immune complex deposition (subendo)+thickened capillary loops
IgA nephropathy
post strep glomerulonephritis can progress to
crescentic GN (RPGN)
finding of inflammatory debris (FIBRIN + macrophages)
crescentic GN (rapidy progressive glomerulonephritis)
lupus nephritis
“full house deposition” (IgG/M/A, complement)
dx: ANA and anti-dsDNA
crescentic GN
Crescents composition/location
crescents in Bowman’s space
fibrin + macrophages
crescents + ribbon like IgG deposition
anti-GBM
Goodpasture
crescents + granular IgG deposition
PSGN
SLE
IgA
MPGN (idiopathic)
crescents + negative IF, ANCA+
pauci-immune Werger granulomatosis (c-ANCA), microscopic polyangitis or Churg-Strauss (p-ANCA)
type IV collagen defect (alpha 3,4,5)
alport syndrome
thick BM prolif mesangial cell matrix varying renal func chronic deposition of immune complexes double contours, hyper cellular, subendoethelial or within BM deposition
membranoproliferative glomerulonephritis (MPGN)
MPGN (types !, II)
membranous nephropathy
location of deposits?
MPGN I –> subENDOthelial
MPGN II –> w/i BM
membranous nephropathy –> subEPIthelial
tram track GBM
membranoproliferazive glomerulonephritis (MPGN)
thick BM prolif mesangial cell matrix tram tracks hepatitis B/C or idiopathic sub endothelial Ig depsoits w/ granular IF
membranoproliferazive glomerulonephritis (MPGN) type I
thick BM prolif mesangial cell matrix tram tracks low levels of circulating C3 IF: no IgG
membranoproliferazive glomerulonephritis (MPGN) type II
cyroglobulins
sign of active hep C
think MPGN
membranoproliferazive glomerulonephritis (MPGN) type II MOA
assoc w/ C3 nephritic factor (AutoAb) –> stabilizes C3 convertase –> alternative path –> overactive complement –> buildup in GBM + inflammation
LOW circulating C3 levels
palpable abd mass
hematuria
flank pain
usu clear cell histo
renal cell CA
loss of VHL (tumor suppressor)
renal cell CA
*can also be sporadic (male, smokers)
renal cell CA origin
PCT cells
yellow mass
highly vascular. often hemorrhage
renal cell CA
most common renal malignancy young child
Wilm’s tumor
nephroblastoma
epithelium, storm, blastema on histo
wilms tumor
WT1 tumor suppressor mut
Wilms tumor
assoc w/ wilms
wagr syndorme
beckwith wiedmann syndrome
denys-drash syndrome
WAGR
wilms tunmor aniridia (iris absence) genital anomalies mental retardation deletion of WT1 gene chrom 11
ped overgrowth disorder
- wilms
- neonatal hypoglycemia
- macrosomia
- muscular hemihypertrophy
- organomegaly (tongue)
beck with wiedemann
diffuse mesangial sclerosis
dysgenesis of gonads (male pseudohermaphroditism)
WT1 mut
Denys drash syndrome
hamartoma (vessels/smc/adipose)
benign tumor (young children)
cardiac rhabodomyomas
ash-leaf patches
angiomyolipma
tumor assoc w/ tuberous sclerosis
angiomyolipoma
benign epithelial neoplasm arising from CD
tumor oncocytic cells (eos+ granular cytoplasm)
NESTS/CORDS, myxoid stroma
oncocytoma
tan, well-circumscribed tumor
oncocytoma
malignancy assoc w/ L sided varicocele
renal cell CA
bladder (urothelial carcinomas)
older adults
infiltrative, difficult to completely excise
recurring
UA: hematuria w/o casts
transitional cell CA
most common
multifocal/recurrent (“field defect”) damage to entire urothelium
old/white/smoker
NO casts in urine
flat or papillary
tx: chemo (platinum), surgery, radiation
tumor that is ideally tx w/ surgery (non-responsive to chemo)
renal cell CA
rare, need chronic inflammation of BLADDER
risk fx:
mult kidney stones
UTI (schisto)
squamous cell CA( metaplasia –> dysplasia –> squamous cell CA)
rare, glandular prolif of bladder
risk fx:
urachal remnant (dome of bladder-umbilicus)
long hx cystitis
exstrophy
adenocarcinoma
types of urothelial CA
transitional cell CA
squamous cell CA
adenocarcinoma
tumor with large eos cells with abundant mitochondria without perinuclear clearing
oncocytoma