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Path Renal
KYCOM Block 13
131
Pathology
Graduate
01/25/2014

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Term
explain the barriers to nephron filtration 5
Definition
mesangium: supports vessels
endothelium: interior vessel size barrier
basement membrane: neg charge barrier
podocytes: visceral epithelium size barrier
nephrin: keeps podocytes spaced for size barrier, negative charge barrier
Term
explain the 4 ways the kidney is damaged
Definition
antigens: get stuck subepithelial

circulating immune complexes: get stuck subendothelial

Ig attack IV collagen: attack BM, complement follows

Complement damage: induces epithelial and endothelial release of proteases, GF, and cytokines which destory BM and kill cells. cause hypocomplementemia
Term
what are the 3 ways we can look at a kidney biopsy, what do each show us
Definition
electron microscope: location of immune complexes

light microscope: fibrosis, collagen, BM structure (stained with silver, PAS, trichrome)

immunoflourescence: tells type if Ig and Ag, exact location, and pattern (linear or granular)
Term
define uremia, what are the symptoms 6
Definition
illness with kidney failure

fatigue, weakness, nausea, itch, confusion, pericarditis, coma
Term
define oliguria
Definition
urine output <400mL/d
Term
define anuria
Definition
urine output <75mL/d
Term
what are the 3 types of azotemia, how do they occur
Definition
pre-renal: dec CO causes hypoperfusion in glomeruli and dec GFT which allows BUN to reabsorb into blood more (BUN:CR>15)

post-renal azotemia: UT obstruction below kidney increases pressure and dec GFR allowing BUN to be resorbed more (BUN:CR>15)

renal intrinsic azotemia: kidney damage won't allow BUN back into blood (BUN:CR<15, RBC cast, protien in urine)
Term
7 characteristics of nephritic syndromes
Definition
protein <3.5g/d (<+2)
little edema
oliguria
HTN due to protein
asotemia
hematuria: RBC casts
dysmorphic RBC
Term
7 characteristics of nephrotic syndromes
Definition
protein <3.5g/d (>+3)
general dependent edema
hypoalbumenia
hyperlipidemia
lipiduria (maltease cross)
fat thromboembolism
endothelial dammage (inflamm and clots)
dec plasma: ions, vitamins, hormones, drugs
Term
why is the hyperlipidemia, lipiduria, and fat thromboembolism in nephrotic syndromes
Definition
proteinuria causes liver to try and make more and it always sends protein out with fats so fats seen in blood and in urine as fatty casts. extra fats in blood give risk of thromboembolism
Term
what is the final stage of many glomerular diseases
Definition
chronic glomerulonephritis
Term
what renal diseases commonly end in chronic glomerulonephritis 3
Definition
FSGS, RBGN, MBGN
Term
symptoms of chronic glomerulonephritis 4
Definition
uremia; protein, azotemia
anemia
anorexia
Term
urinalysis 1, LM trichrome signs of chronic glomerulonephritis 6
Definition
waxy casts

fibrosis, dead cells, hyalinization, interstitial fibrosis, lymphocytic infiltrate, scaring
Term
prognosis and tx of chronic glomerulonephritis
Definition
death without dialysis and transplant
Term
what are the 4 nephritic diseases
Definition
acute proliferative (post-strep) GN
rapid proliferative (crecentric) GN
IgA nephropathy
hereditary nephritis - alport syndrome
Term
what are the age/most commons for the 4 nephritic diseases
Definition
acute proliferative (post-strep) GN: more commonin kids

rapid proliferative (crecentric) GN: male > female 20-40yo

IgA nephropathy: more common in kids, most common GN, most common hematuria

hereditary nephritis - alport syndrome: AD, AR, X linked. 20-40yo
Term
what is the pathological cause of acute proliferative glomerulonephritis 2
Definition
immune complexes with antigens to GBS 1-4wk after infection

immune complexes to SLE (lupus nephritis)
Term
what is the patholgical causes of the 3 rapid progressive (crecentric) GN
Definition
1. good pastures: anti-IgG to type IV collagen in glomeruli BM and aleolar capillary that recruits C3

2. anti-IgG to SLE, IgA nephropathy, PGN that attacks nephron

3. serum ANCA attacks nephron in microscopic polyangitis, weagner's granulomatosis, or idiopathic pacui-immune type GN
Term
what do the vessels look like the goodpastures
Definition
parietal cells and monocytes cause crescent shape and push bowmans and cell tuft decreasing flow and killing cells

anti-IgG to IV collagen on GBM and pulmonary BM (1st) in LINEAR pattern
Term
symptoms of goodpastures 5
Definition
pulmonary hemorrhage
hemoptysis
plasma phoresis: removes Ab
oliguria
azotemia
Term
what do the vessels look like in type 2 rapid progressive glomerulonephritis
Definition
parietal cells and monocytes cause crescent shape and push bowmans and cell tuft decreasing flow and killing cells

anti- IgG from SLE/PGN/IgAN in granular pattern
Term
what do the vessels look like in type 3 rapid progressive glomerulonephritis
Definition
parietal cells and monocytes cause crescent shape and push bowmans and cell tuft decreasing flow and killing cells
ANCA
Term
prognosis and treatment of rapid progressive (crescentric) GN (all three types)
Definition
rapid progression to renal failure
<80% crescent cells is better prognosis
dialysis and transplant needed to death
Term
what is the cause of IgA nephropathy
Definition
genetic abnormality in IgA production or clearance
IgA is made to respiratory or GI infection then attacks the glomeruli
Term
what other conditions is IgA nephropathy associated with 3
Definition
cleaiac disease
liver disease
henoch-scholein purpura (probablly a varient of it)
Term
what does the LM, EM, and IF show in IgA nephropathy
Definition
LM: mesangial proliferation
EM: electron dense deposits in mesangium
IF: IgA in mesangium, C3 alternate compliment pathway activation and recruitment
Term
symptoms of IgA nephropathy 2
Definition
hematuria 1-2d post respiratory, GI, or urinary infection that reoccurs every few months

LOIN PAIN
Term
prognosis of IgA nephropathy
Definition
can sometimes progress to chronci renal failure in 20y
Term
cause of aloprt syndrome
Definition
GBM type IV collagen mutation
Term
what does the vessel look like in aloprt syndrome 3
Definition
thin BM
LM trichrome: sclerosis, fibrosis
Term
signs of alport syndrome 5
Definition
nerve deafness
lens dislocation
posterior cataract
cornea dystrophy
carriers: hematuria
Term
prognosis of alport syndrome
Definition
renal failure by 20-50yo
Term
what are the 4 nephrotic diseases
Definition
minimal change diesease
membranous glomerulonephritis
focal segmental glomerulonephritis
membranous proliferative GN
Term
what are the ages or common epidemology of the 4 nephrotic diseases
Definition
minimal change disease: most common nephrotic syndrome in kids

membranous glomeruonephritis: most common nephrotic syndrome in adults (30-50)

focal segmental GN: in all ages, more in AA, HIV, IV drugs

membranous proliferative Gn: nephritic, nephrotic, or mixed
Term
what is the cause of minimal change disease
Definition
T cells induce IL-8 and TNF which attacks the nephron
Term
what are the EM, IF, and LM changes in the glomerulus in minimal change disease
Definition
EM: loss of slit pores, damage to podocytes
IF: no complexes seen
LM: PCT filled with lipoprotein causes NECROSIS
Term
what is the clinical presentation of minimal change disease
Definition
previously healthy kid (1-7) gets respiratory infection or vaccine and activates this immune response

selective proteinuria (albumin)
Term
what disorders is minimal change disease associated with
Definition
atopic disorders
Term
prognosis and tx of minimal change disease
Definition
>90% respond to steroids
<5% have renal failure in 25y
change is reversible
Term
what is the cause of membranous glomerulonephritis 3
Definition
exogenous: Hep B, hep B, malaria, syphillis, penicillin, NSAID, gold, murcery lodge in the kidney and body sends antibodies to attack them

endogeous: SLE, malignant carcinoma from lung or colon (ANTI-CEA), melanoma, or DM cause auto-antibodies to the kidney

both induce C5-C9 MAC to cause endothelial and mesangial cells to release protease and oxidants that cause damage
Term
what are the LM, EM, and IF changes in membranous glomerulonephritis
Definition
LM: thick BM and spikes
EM: subepithelial deposits, spikes, podocyte loss
IM: C3, IgG along BM

DIFFUSE THICKENING OF CAPILLARIES
NECROSIS of kidney
Term
clinical signs of membranous glomerulonephritis
Definition
non-selective proteinuria
NECROSIS of kidney
Term
prognosis of membraneous glomerularnephritis
Definition
40% renal failure in 2-10y
10-30% benign/partial remission/full remission
Term
what is the cause of focal segmental glomerulonephritis 3
Definition
evolution from other glomerular nephritis
agressive form of minimal change disease
curculating mediators (IgM/C3)
Term
what are the LM, IF, EM changes in focal segmental GN
Definition
SEGMENTAL
LM: tubular atrophy, sclerosis, hyalinization, interstitial fibrosis
IF: IgM, C3
BM: broken, displaced, obliderated podocytes
Term
clinical signs of focal segmental GN
Definition
hx glomerulonephritis (esp IgA nephropathy)
non-selective proteinuria
hematuria
HTN
Term
prognosis of focal segmental GN
Definition
poor
kids > adults
50% renal failure in 10y
Term
cause of membranous proliferative GN 2
Definition
immune complexes lodge in kidney and recruit C3: HapB, HepC, SLE, AV shunt infection, CLL)

C3 nephritic factor (C3nF) activates alternate complement pathway stabilizing C3 convertase causing hypocomplementemia (except C1, 2, 4) and causing complement damage to glomeruli
Term
EM, IM, LM changes in membranous proliferative GN type 1
Definition
EM: subentotheloal deposits
IM: IgG, C1q, C4, GRANULAR
LM: lobular glomeruli, proliferation of mesangial cells, infiltrative WBC, TRAIN TRACK BM
Term
EM, IM, LM changes in membranous proliferative GN type 2
Definition
EM: deposits inside BM and subendothelial, GRANULAR
IF: C3 in BM and mesangium
LM: lobular glomeruli, proliferation of mesangial cells, infiltrative WBC, TRAIN TRACK BM
Term
clinical signs of membranous proliferative GN
Definition
mild proteinuria
Term
prognosis of membranous proliferative GN
Definition
slowly progressive (inc proteinuria) until renal failure
type 2 is worse
Term
what are the 4 systemic diseases that cause glomerulonephritis
Definition
diabetic glomeruloapthy
SLE
renal amyloidosis
multiple myeloma kidney disease
Term
what is the most common cause of renal failure in the uS
Definition
diabetic glomerulopathy
Term
what are the 3 risk factors for diabetic glomerulopathy
Definition
poor glycemic control
HTN
diabetic reinopathy - high correlation
Term
what are the changes to the vessels and golmeruli in diabetic glomerulopathy 7
Definition
non-enzymatic glycosulation causes athlerosclerosis and haline formation which cause hyperfiltration and increase GFR, and causes damage

glycosylation and endothelil damage cause sorbital activation which causes hyperfiltration, increases GFR, and causes damage

type IV collagen increases

proteinuria due to compormised layers

protein and type IV collagen deposits in mesangium

papillary necrosis: infarction of renal papillae cause puss in calyx, black papillae, death of papilae, and sloughing off
Term
what are the clinical signs of diabetic glomerulopathy 2
Definition
poor glycemia for 10d leads to microalbuminea
Term
what are people with diabetic glomerulopathy at risk for
Definition
chronic pyelonephritis
Term
how does SLE cause glomerulonephritis
Definition
immune complexes stick in mesangium, BM, capillaries

diffuse proliferation of glomeruli
Term
how does renal amyloidosis cause glomerulonepheitis
Definition
depostits of amyloid in glomeruli (esp in dialysis), BM, vessels
Term
how is renal amyloidosis diagnosed
Definition
congo red shows yellow green bifringent amyloid in polarized light
Term
how does multiple myeloma kidney disease cause glomerulonephritis
Definition
Ig causes tubular casts and urinary obstruction
Term
what are the two types of renal vessel diseases
Definition
benign nephrosclerosis
malignant nephrosclerosis
Term
what is the cause of benign nephrosclerosis
Definition
renal changes due to benign h
Term
what are the two types of renal vessel diseases
Definition
benign nephrosclerosis
malignant nephrosclerosis
Term
what is the cause of benign nephrosclerosis
Definition
renal changes due to benign HTN causes hayline arteriosclerosis
Term
what is the cause of malignant nephrosclerosis
Definition
renal change due to malignant HTN causes hyperplastic arteriolosclerosis
Term
how does the external kidney look in benign nephrosclerosis
Definition
atrophy: grain leather, granular surface
Term
what changes in the arteries in benign nephrosclerosis
Definition
pink haylinethickening of vessels
Term
how does the kidney function change in benign nephrosclerosis
Definition
decreased GFR
decreased concentrating abilities
Term
how do the large arteries change in malignant nephrosclerosis
Definition
hyperplastic arteriolosclerosis (onion skin)
Term
how does the external kidney change in malignant nephrosclerosis
Definition
flea bitten kidney: pinpoint petechial hemorrhages on surface
Term
how do the small arteries change in malignant nephrosclerosis 3
Definition
fibroid necrosis
granular eosinophillia
necrotizing arteriolitis (inflammatory cells)
necrotizing glomerulitis: arteriolitis in glomeruli
Term
what are the clinical signs of malignant nephrosclerosis 6
Definition
diastolic >120 EMERGENCY
papilledema
encephalopathy
cardiovascular abnormalities
renal failure: proteinuria, hematuria
Term
what is the prognosis of malignant nephrosclerosis 3
Definition
QUICKLY progresses to renal failure
50% survive 5y
90% that die die from uremia
Term
what are the two types of kidney infections
Definition
acute pyelonephritis
chronic pyelonephritis
Term
what are the three types of kidney toxicities
Definition
acute tubular necrosis
acute drug induced nephritis
analgesic nephropathy
Term
who usually gets acute pyelonephritis
Definition
more commoni n females (short urethra, damage with sex)
Term
what microbes usually cause pyelonephritis 4
Definition
#1 E.colo
klebsiella
proteus
enterobacterium
Term
what are ways that microbes can get to the kidney 11
Definition
infection ascention
colonization and spread
insturmentation
vesicurorectal reflex incompetence
flaccid pladder (spinal injury)
hematogenous
outflow obstruction
stasis
incomplete voiding
benign prostatic hypertrophy
uterine prolapse
Term
where does pyrlonephritis infect the kidney
Definition
pelvis
tubules
interstitium
NOT glomeruli
Term
4 signs in kidney of pleonephritis
Definition
yellow external abscess
suppurative interstitial and tubular necrosis and abscess
pyonephrosis: exudate
papillary necrosis of pyramid tips
Term
what are 7 signs of pyelonephritis
Definition
fever
chills
CVA tenderness
WBC casts
dysuria
urgency
pyuria
Term
what is the most common cause of acute renal failure
Definition
acute tubular necrosis
Term
what are the two types of acute tubular necrosis, give examples of what could cause each
Definition
ischemic

nephrotoxic: AMINOGLYCOSIDES, IV RADIOCONTRAST, MYOBLOBIN VIA CRUSH INJURY, Mu, Ag, Pb, CCl3, ethylene glycol, mushrooms, pesticides
Term
what are 4 signs of ischemic acute tubular necrosis
Definition
BM rupture: cell dies and cannot regenerate
tubular necrosis
generalized edema of interstitium
tubular casts wash into urine
Term
what are 4 signs of nephrotixic acute tubular necrosis
Definition
some BM in tact
tubular necrosis
tubular casts in urine
generalized interstitial edema
Term
prognosis of acute tubular necrosis
Definition
reversible if survive the other symptoms of the toxicity
excellent prognosis
Term
signs of acute tubular necrosis in urinalysis
Definition
oliguria
dec BUN/CR (high in serum)
basic (acidic blood)
low k (high in blood)
dirty brown tubular casts
Term
what is the cause of chronic pleonephritis
Definition
recurrent infection due to chronic obstruction or reflux
Term
3 signs in kidney of chronic pleonephritis
Definition
scaring and fibrosis cause blunt calyces and retracted pelvis

fibrosis of interstitial tubules with PAS positive colloid casts
Term
what is seen on a pyelogram and biopsy of a kidney with chronic pleonephrosis
Definition
calyces and pelvis retraction

may see FSGN
Term
clinical signs of chronic pleonephrosis 2
Definition
unoticed until renal failure
HTN due to inflammation
Term
cause of acute drug induced nephritis
Definition
hypersensitivity to drug 15d after exposure (antibiotic, thiazide, NSAID) binds kidney elements OR look similar to kidney causing cross reactivity
Term
signs in kidney of acute drug induced nephritis 3
Definition
edema
eosinophils
PMN
Term
clinical signs of acute drug induced nephritis 7
Definition
fever
eosinophilia
rash
hematuria
leukocytouria
serum CR increase
oliguria esp in elderly
Term
tx and prognosis for acute drug induced nephritis
Definition
remove drug, recovery in months
Term
cause of analgesic nephropathy
Definition
analgesic + (phenacetin, aspirin, tyalnol, caffiene, codine) x long time
Term
how does aspirin damage kidney
Definition
stops prostaglandin synthesis, decreases vasodilation, causes papilla ischemia, necrosis, and shriveling
Term
how does tyanol causes kidney damage
Definition
binds and causes ROS damage
Term
how does phenacetin cause kidney damage
Definition
phenacetin breakdown cause yellow lipofuscion like exudate
Term
what is a sign of analgesic nephropathy in kidney no matter the cause
Definition
pas positive BM thickening of small papillae vessels
Term
clinical signs of analgesic nephropathy 3
Definition
HTN
anemia
Term
what are the three kidney cyst diseases
Definition
simple cysts
AD polycystic kidney disease
AR polycystic kidney disease
Term
who gets the three kidney cyst diseases
Definition
simple cysts - dialysis pt in end stage disease or long term dialysis, idiopathic

AD polycistic kidney disease - adults 40yo

AR polycstic kidney disease - rare, in kids, perinatal/neonatal/infantile/juveline
Term
describe a simple cyst 4
Definition
1-5cm
in cortex
clear
filled with fluid
Term
why do we care about simple cysts if they dont do anything
Definition
they can differentiate into adenoma/adenocarcinoma
Term
what is the cause of AD polycystic kidney disease
Definition
AD polycistin (PKD) mutation (90% PKD1) of protein that is like other proteins that cause cell-cell-matrix adhesions
Term
clinical signs of AD polcystic kindey disease 5
Definition
flank pain
large palpable 4kg kidneys
HTN
hematuria
UTI
Term
signs in kidney of AD polycystic kidney disease 2
Definition
kidney failure
2-4cm cysts all over outside of kidney push on it and cause ischemia and atrophy
Term
signs outside kidney of AD polycystic kidney disease 3
Definition
berry anurysm in circle of willis
subarachnoid hemorrhage
asymptomatic liver cysts
Term
tx and prognosis of AD polycystic kidney disease
Definition
10% cohronic renal failure (>50yo)
SLOW progression
tx: transplant
Term
cause of AR polycystic kidney disease
Definition
2 mutated fibrocystin genes (PKhD1) to receptors on CD and biliary differentiating cells
Term
signs of AR polycystic kidney disease 3
Definition
cysts in cortex and medulla that spread from CD

sponge like kidney

cysts in liver and portal bile ducts
Term
prognosis of AR polycstic kidney disease
Definition
perinatal and neonatal die from pulmonary and respiratory failure

liver chirrhosis
Term
who gets urolithiasis
Definition
men>women
20-30yo
Term
general cause of urolithiasis
Definition
supersaturation of substance allows for percipitation
Term
size and location of urlithiasis
Definition
80% unilateral
usually 2-3 cm
Term
clinical signs of urolithiasis 4
Definition
flank pain towards groin (uteral colic)

gross hematuria

infections

hydronephrosis
Term
4 compounds causing renal stones, which is most common
Definition
Ca oxylate 70%
Mg ammonium phosphate
uric acid
cysteine
Term
how do Ca oxylate stones form
Definition
hypercalcuria without hypercalcemia (Ca goes right from GI to bladder)
renal wont absorb the Ca and uric acid wont enter because its too concentrated (so it cant break it up) so it percipitates
Term
why would someone have hypercalcuria and hypercalcemia in ueolithiasis 2
Definition
hyperparathyroidism
sarcoidosis
Term
how are Mg ammonium phosphate crystals formed, aka
Definition
struvite
bacteria poo out urea and it percipitates
Term
how do uric acid crystal form
Definition
gout increases uric acid or leukemia turns over cells and it spills out and gets high and then it is high in pee and percipitates when urine pH <5.5
Term
how do cysteine crystals form
Definition
genetic deficit in renal AA transporter for cys back into blood so it is stuck in pee in high concentration and percipitates
Term
what is a staghorn calculi
Definition
struvute in branches in casts of tubules
Term
what causes hydronephrosis
Definition
prostate enlargement, tumor, stone, anything that blocks the urine
Term
what are the changes in the kidney in hydronephrosis 6
Definition
BIG kidney
dilation of pelvis and calyces
obliterated papulae
flat pyramids
parenchyma compression, necrosis, and atrophy
coagulative necrosis (when acute)
Term
clinical signs of hydronephrosis 3
Definition
anuridia
polyruidia (decreased concentrating ability)
if unilateral may be asymptomatic for longer
Term
prognosis of hydronephrosis
Definition
reversible if un-obstructed within a few weeks
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