Study your flashcards anywhere!

Download the official Cram app for free >

  • Shuffle
    Toggle On
    Toggle Off
  • Alphabetize
    Toggle On
    Toggle Off
  • Front First
    Toggle On
    Toggle Off
  • Both Sides
    Toggle On
    Toggle Off
  • Read
    Toggle On
    Toggle Off

How to study your flashcards.

Right/Left arrow keys: Navigate between flashcards.right arrow keyleft arrow key

Up/Down arrow keys: Flip the card between the front and back.down keyup key

H key: Show hint (3rd side).h key

A key: Read text to speech.a key


Play button


Play button




Click to flip

351 Cards in this Set

  • Front
  • Back
define azotemia
elevation of BUN and Creatinine; related to a decrease in GFR; asymptomatic
deifine uremia
(basically) azotemia with symptoms
three types of azotemia and major reasons for these types?
1. pre-renal - reduced perfusion of the kidneys
2. renal - intrinsic renal disease
3. post-renal - obstruction of urine flow past the kidney
what metabolic changes will be present in renal failure? Why?
metabolic acidosis with an anion gap
(secondary to decreased bicarbonate and accumulation of acids)
describe the following electrolyte levels in renal failure
1. sodium
2. potassium
3. calcium
4. phosphorus
5. magnesium
1. dilutionally decreased
2. increased
3. decreased
4. increased (esp. in crush injuries)
5. increased (just like K+)
which electrolyte disturbance is considered a "major life-threatening" disturbance?
(causes arrythmias)
CV/Pulmonary manifestations of renal failure? (4)
volume overload CHF/pulmonary edema
hematologic abnormalities seen in renal failure?
normocytic, normochromic anemia
manifestations of renal failure in bones? (3)
renal osteodystrophy
secondary hyperparathyroidism
altered calcium metabolism
three clinical signs of nephritic syndrome?
1. gross hematuria
2. hypertension
3. proteinuria (mild to moderate)
four clinical signs of nephrotic syndrome?
1. heavy proteinuria
2. hypoalbuminemia
3. severe edema
4. hyperlipidemia, lipiduria
general pathophysiology of nephritic syndrome?
usually acute onset.
glomerular diseases, usually proliferative (inflammatory)in nature
general pathophysiology of nephrotic syndrome?
alterations of glomerular function or structure. Effacement of foot processes is present.
what does asymptomatic hematuria or proteinuria usually indicate?
mild glomerular abnormalities
what are the four "levels" of chronic renal failure?
1. diminished renal reserve
2. renal insufficiency
3. renal failure
4. end stage renal disease
What is the GFR for each of the following?
1. diminished renal reserve
2. renal insufficiency
3. renal failure
4. end stage renal disease
1. GFR ~ 50% of normal
2. GFR is 20-50% of normal
3. GFR is <20% of normal
4. GFR is <5% of normal
what are the BUN and creatinine levels in diminished renal reserve?
(due to reserve capacity of the kidney)
what are the three clinical signs of renal tubular defects?
1. polyuria
2. nocturia
3. electrolyte imbalances
how common are congenital anomalies of the kidney?
common (~10% of all people)
what is often seen along with bilateral renal agenesis?
abnormal lung development in utero
(incompatible with life)
what constitutes renal hypoplasia?
a small, unscarred kidney with fewer than six pyramids
1. what is an ectopic kidney?
2. consequences?
1. incorrect position of the kidneys (usually in pelvis)
2. -predisposed to infection and backflow
renal dysgenesis or agenesis causes what in regards to the amniotic fluid?
common placental manifestation of oligohydramnios?
amnion nodosum
common fetal manifestations of oligohydramnios? (4)
Potter sequence
1. lung hypoplasia
2. altered facies
3. defects in hands and feet
4. breech position
describe the most common shape abnormality of a malformed kidney.
horseshoe kidney - two kidneys are fused at poles forming a single kidney
genetic associations of autosomal recessive polycystic kidney disease (infantile)
PKHD1 gene that encodes fibrocystin
*unknown mechanism*
pathologic features of autosomal recessive polycystic kidney disease (infantile)?
enlarged, cystic kidneys
complications of autosomal recessive polycystic kidney disease (infantile), fetus and infant?
FETAL - pulmonary hypoplasia (fatal)
INFANTILE - hepatic fibrosis
typical outcome of autosomal recessive polycystic kidney disease (infantile)?
stillborn or death in infancy or childhood
differences in heredity between infantile and adult polycystic kidney disease?
infantile - autosomal recessive
adult - autosomal dominant, high penetrance
1. genetic connection in autosomal dominant adult polycystic kidney disease?
2. likely result of this defect?
1. mutations in polycystin 1 or polycystin 2 genes
2. result is alteration in Ca++ transport
common clinical complication in autosomal dominant adult polycystic kidney disease?
Berry aneurysms
pathologic features in autosomal dominant adult polycycstic kidney disease?
large multicystic kidneys
liver cysts
5 Clinical features of autosomal dominant adult polycystic kidney disease?
1. hematuria
2. flank pain
3. UTI
4. renal stones
5. HTN
typical outcome of autosomal dominant adult polycystic kidney disease?
chronic renal failure beginning at age 40-60
inheritance of cystic renal dysplasia?
NONE - there is no familial component
what is cystic renal dysplasia?
congenital malformation of kidney with formation of cysts and presence of abnormal histologic structures (ie. bone, cartilage). No renal organization.
typical outcome of bilateral cystic renal dysplasia?
fatal during fetal development
typical outcome of unilateral cystic dysplasia?
benign course, asymptomatic
(may see increase in infections, reflux)
define medullary sponge kidney
cystic dilation of collecting ducts in medulla
clinical features of medullary sponge kidney?
usually asymptomatic
may see -
hematuria, UTIs, recurrent renal stones
Typical outcome of medullary sponge kidney?
inheritance pattern of familial juvenile nephronophithisis (uremic medullary cystic disease)?
autosomal recessive
genetic pathogenesis behind familial juvenile nephronophthisis (uremic medullary cystic disease)?
mutations in NPH1, NPH2, NPH3
pathologic features of familial juvenile nephronophthisis (uremic medullary cystic disease)?
corticomedullary cysts, shrunken kidneys
typical outcome of familial juvenile nephronophthisis (uremic medullary cystic disease)?
progressive renal failure beginning in childhood
4 clinical features of familial juvenile nephronophithisis (uremic medullary cystic disease)?
1. salt wasting
2. polyuria
3. growth retardation
4. anemia
inheritance pattern of adult onset medullary cystic disease?
autosomal dominant
genetic pathogenesis behind adult onset medullary cystic disease?
pathologic features of adult onset medullary cystic disease?
corticomedullary cysts, shrunken kidneys
(same as juvenile onset medullary cystic disease)
2 clinical features of adult onset medullary cystic disease?
1. salt wasting
2. polyuria
typical outcome of adult onset medullary cystic disease?
chronic renal failure beginning in adulthood
simple cysts are common and normally asymptomatic. If they are at all symptomatic, what might the symptoms be?
1. microscopic hematuria
2. large cysts may rupture or hemorrhage
MC cause of acquired cysts?
(these cysts are small)
thing we should remember about von Hipple-Lindau disease?
increased susceptibility to renal cell and other cancers
what proportion of the total blood supply does the kidney receive?
which side of the circulation are the glomerular capillaries (both efferent and afferent poles)?
arterial side
what is the name of the matrix that supports the glomerular tufts?
what are the two types of cells found in the mesangium and what is their function?
type 1 - contractile
type 2 - secretory
a capillary loop is a functional filtering unit of three layers. what are the three layers from the outside in?
1. Visceral epithelial cell (podocytes)
2. Glomerular basement membrane
3. Endothelial cell
what type of filter are the visceral epithelial cells (podocytes)?
size and charge filter
(anionic charged)
what structure separates adjacent podocytes?
slit diaphragm
four places type IV collagen is found?
1. glomerular basement membrane
2. eye
3. ear
4. lung
what type of filter does the glomerular basement membrane act as?
charge and size filter
what type of filter does the endothelial cell act as?
charge and size filter
- anionic charge
- restricts proteins about the size of albumin
what happens to the ultrafiltrate once it reaches Bowman's space?
enters tubule system, then is excreted in the urine
what are the epithelial cells called that line Bowman's space?
parietal epithelium
what are the four terms used to describe the extent of glomerular disease?
what does diffuse glomerular involvement mean?
most or all the glomeruli in the kidney are affected
what does focal glomerular involvement mean?
only some of the glomeruli affected
what does segmental glomerular involvement mean?
only part of an individual glomerulus is affected
what does global glomerular involvement mean?
the entire glomerulus is affected
what are the three general glomerular patterns of injury?
1. Membranous patterns
2. Sclerosis
3. Poliferative (inflammatory) patterns
describe membranous patterns of glomerular injury
diffuse, acellular thickening of the capillary loops without an increase in cellularity in the glomerulus. capillary loops remain patent.
describe the sclerotic pattern of glomerular injury
sclerotic changes and fibrosis in the glomerulus
proliferative patterns of glomerular injury may result from what two general patterns?
1. hypercellularity of glomerular cells (esp. in mesangium)
2. infiltration of inflammatory cells
clinical manifestations of segmental sclerosis, mesangioproliferative and membranous glomerular disease?
hematuria and/or proteinuria
clinical manifestations of crescentic glomerular injury?
Acute renal failure
clinical manifestation of chronic sclerosed glomerular disease?
renal failure
what is the mildest form of proliferative glomerular injury?
mesangioproliferative glomerulonephritis
(hypercellularity of mesangium only)
what is proliferative glomerulonephritis?
hypercellularity of capillaries and mesangium (this may include inflammatory cells)
what is acute proliferative glomerulonephritis?
proliferative glomerulonephritis with a predominance of neutrophils
what is necrotizing glomerulonephritis?
necrosis of glomeruli and nuclear debris
what is membranoproliferative glomerular disease?
hypercellularity of the glomerulus WITH thickened capillary walls
what is crescentic glomerulonephritis?
proliferating epithelial cells with inflammation in Bowman's space. forms concentric layers (crescents) around the glomerular tuft. Ominous sign
is chronic sclerosis reversible?
NO - this is the end stage of most glomerular and renal diseases
clinical correlation with mesangial cell hypercellularity?
clinical correlation with mesangial matrix increase?
decreased GFR
clinical correlation with effacement of epithelial foot processes?
what is the etiology of most glomerular diseases?
immune mediated
In an immune mediated injury which cells are found to increase in the glomerulus? (4)
mesangial cells
four soluble mediators released during glomerular immune-mediated injury?
growth factors
activation of coagulation cascade
anti GBM antigen is actually directed against what?
type IV collagen
which immunoglobulin is most commonly seen in in situ immune complex formation?
in Goodpastures syndrome the immunoglobulin deposition is what shape?
what is a "pauci-immune" glomerular disease and how is it distinguished from an immune complex disease?
pauci-immune means there appears to be an immune response mechanism, yet there are no immune complexes within the glomerulus.
ANCA stands for?
when does acute proliferative (post-strep) glomerulonephritis MC occur?
1-2 wks after recovery from infectious disease (usually strep pharyngitis)
acute proliferative (post-strep) glomerulonephritis MC occurs in which age group?
(does occur in adults and is more severe in adults)
gross findings seen in acute proliferative (post-strep) glomerulonephritis?
flea-bitten kidney (hyperemic glomeruli causes a punctate hemorrhagic appearance)
microscopic manifestations of acute proliferative (post-strep) glomerulonephritis?
* proliferation of mesangial and endothelial cells within the glomerulus
* neutrophils present
* +/- crescents
immunoflourescence manifestations of acute proliferative (post-strep) glomerulonephritis?
see granular deposition of IgG and C3. "lumpy-bumpy"
EM manifestations of acute proliferative (post-strep) glomerulonephritis?
"lumps and bumps"
large subepithelial immune complexes
two hallmark clinical presentations of acute proliferative (post-strep) glomerulonephritis?
1. nephritis with oliguria
2. periorbital edema
features of a laboratory diagnosis of acute proliferative (post-strep) glomerulonephritis? (4)
1. hematuria with RBC casts
2. decreased C3
3. elevated strep antibodies
4. renal biopsy if necessary
prognosis of acute proliferative (post-strep) glomerulonephritis?
most recover in 6-8 wks.
adults - tend to progress to rapidly progressing glomerulonephritis (RPGN) and chronic renal insufficiency
what is the proposed etiology of crescentic (rapidly progressive) Glomerulonephritis?
leakage of fibrin and fibrinogen into Bowman's space, this stimulates proliferation.
*seen as a complication of many types of glomerulonephritis*
microscopic manifestations of crescentic (rapidly progressive) Glomerulonephritis? (3)
1. crescents in Bowmans space
2. epithelial cells, neutrophils, macrophages in Bowmans space
3. may have focal necrosis
clinical presentation of patients with crescentic (rapidly progressive) Glomerulonephritis? (2)
1. nephritic syndrome
2. progressive renal failure
Lab test used to diagnose crescentic (rapidly progressive) Glomerulonephritis?
renal biopsy
prognosis of crescentic (rapidly progressive) Glomerulonephritis?
most progress to irreversible renal failure
therapy for crescentic (rapidly progressive) Glomerulonephritis? (3)
1. corticosteroid
2. immunosuppressive
3. dialysis and/or renal transplant in the case of renal failure
what are the two main characteristics of membranoproliferative glomerulonephritis (MPGN)?
1. basement membrane thickening
2. mesangial proliferation
which age group is afflicted by membranoproliferative glomerulonephritis (MPGN)?
children and young adults
which type of MPGN is more often associated with systemic diseases?
type I
describe the location of the deposits seen in:
1. type I MPGN
2. type II MPGN
1. subendothelial deposits (occasional mesangial and subepithelial)
2. deposits within basement membrane
etiology of type II MPGN?
activation of alternate compliment pathway
which glomerular disorder is characterized by "tram tracks" on a silver stain?
MPGN types I and II
which immunological molecules are present in:
1. type I MPGN
2. type II MPGN
1. membrane and mesangial IgG, C3, early compliment
2. lots of C3 deposition in bands of GBM. scant IgG, NO early compliment
other name for type II MPGN?
Dense Deposit Disease (DDD)
prognosis of MPGN?
*nearly all progress to renal failure
*may recur in transplant
laboratory diagnostic techniques/values used for MPGN?
1. renal biopsy to distinguish from other GN
2. persistent low C3 levels
3. hematuria
4. azotemia
describe the new subtype (type III) of MPGN
deposits on both epithelial and endothelial sides of basement membrane
describe the general microscopic characteristics seen in the membranoproliferative pattern of glomerular diseases.
hypercellular, thick membrane
which immunological component is always present in the membranoproliferative pattern of glomerular diseases?
which glomerular disease is a major cause of nephrotic syndrome in adults?
membranous glomerulopathy
4 clinical characterstics of nephrotic syndrome?
1. proteinuria (heavy)
2. hypoalbuminemia
3. hyperlipidemia, lipiduria
4. severe edema, anascara
3 glomerular diseases in which you see nephrotic syndrome?
1. minimal change disease
3. membranous glomerulopathy
explain the pathogenesis behind the hyperlipidemia seen in nephrotic syndrome
*glomerular damage causes protein to be excreted in urine
* therefore protein loss in blood
* liver compensates and synthesizes proteins (including liproteins)
* result is hyperlipidemia
in which age group is membranous glomerulopathy MC seen?
middle aged adults
5 possible etiologies of membranous glomerulopathy?
1. chronic immune complex disease (most idiopathic)
2. hepB
3. drugs (gold, penicillamine)
4. SLE
5. occult cancer
site primarily involved in membranous glomerulopathy?
basement membrane of glomerulus
microscopic characteristics of membranous glomerulopathy? (3)
1. normal cellularity
2. thickening of GBM
3. "spikes" on silver stains
EM characteristics of membranous glomerulopathy?
small deposits in GBM
IF characteristics of membranous glomerulopathy?
granular C3 and IgG along GBM
3 complications of membranous glomerulopathy?
1. renal failure in 1/3 (over 10-15 yr. course)
2. renal vein thrombosis
3. complications of nephrotic syndrome
therapy for primary membranous glomerulopathy? (2)
membranous glomerulonephritis is similar to?
"Heymann nephritis"
(experimental model for immune mediated GN)
what is the most frequent cause of nephrotic syndrome in childhood?
minimal change disease
miscroscopic findings seen in minimal change disease?
IF findings seen in minimal change disease?
EM findings seen in minimal change disease?
effacement of epithelial cell foot processes
villous hyperplasia
swelling of glomerular epithelial cells
complications of minimal change disease?
(infections, renal vein thrombosis, interstitial nephritis)
treatment for minimal change disease?
minimal change disease is MC seen in which age group?
proposed etiology of minimal change disease?
immunologic basis with possible cytokine release and T cell dysfunction
how are minimal change disease and focal segmental glomerular nephritis (FSGN) differentiated?
FSGN gets worse despite corticosteroid therapy, minimal change disease regresses.
in which population is focal segmental glomerulosclerosis more common and severe in?
what is FSGS characterized by?
collapse, sclerosis and hyalinosis of focal glomerular segments
prognosis of FSGS?
progresses to renal failure within 10 yrs
etiology of FSGS?
most idiopathic or part of another renal disease (ie. reaction to chronic proteinuria)
* HIV, heroin use?
microscopic manifestations of FSGS?
FIBROSIS - segmental collapse of glomerular tufts with sclerosis and hyalinosis
EM manifestations of FSGS?
folding and thickening of basement membrane with effacement of foot processes
which immune related molecules are found in FSGS and where are they found?
IgM and C3 are found in areas of sclerosis
clinical presentation of FSGS?
nephrotic syndrome unresponsive to corticosteroids.
three main therapies for FSGS?
what is the most common nephropathy worldwide?
IgA nephropathy
(Berger's disease)
IgA nephropathy may be the sequelae to?
respiratory or GI illness
which populations are most and least afflicted with IgA nephropathy?
most - Native Americans
least - blacks
peaks 2nd-3rd decade
what is the site involved in IgA nephropathy?
mesangium (IgA deposits are here)
IF manifestations of IgA nephropathy?
demonstrates a predominance of IgA
* also see strong C3
* no C1q, C4
EM manifestations of IgA nephropathy?
see mesangial immune deposits.
what, if seen in EM evaluation of IgA nephropathy, is an ominous sign for progression to renal failure?
hyaline thickening of the arterioles
does IgA nephropathy recur in renal transplants?
Yes (in about 50% of them)
IgA nephropathy is the most common cause of what clinical sign?
gross or microscopic hematuria
which sydrome is believed by some to be a manifestation of IgA nephropathy, not its own disease?
Henoch-Schonlein Purpura
what are familial nephropathies characterized by? (ie. what do they present with)
microscopic hematuria without progression.
in which familial nephropathy is the basement membrane 1/2 to 1/3 normal size?
thin membrane disease
progression of thin membrane disease?
may progress to sclerosis and/or renal failure
Alports syndrome is a defect in?
type IV collagen gene
presenting s/s of a patient with Alports syndrome? (4)
1. recurrent hematuria
2. some proteinuria
3. progressive inner-ear deafness
4. ocular disorders (lens dislocation, cataracts, corneal dystrophy)
what is the end stage of renal failure with sclerotic glomeruli called?
Chronic glomerulosclerosis
what do the kidneys look like in chronic glomerulosclerosis?
small and contracted
what do the glomeruli look like in chronic glomerulosclerosis?
sclerotic and hyalinized
is chronic glomerulosclerosis reversible?
patients are offered dialysis or renal transplants
what is the differential of a child presenting with nephrotic syndrome? (3)
1. membranoproliferative glomerulonephritis (type I)
3. Minimal change disease
(all more common in children, all nephrotic presentation)
what is the differential of a child presenting with nephritic syndrome? (2)
1. acute proliferative (post-strep) glomerulonephritis
2. membranoproliferative GN(I)
what is the differential of a child presenting with microscopic hematuria? (3)
2. Thin basement membrane disease
3. Alports syndrome
what is the differential of an adult presenting with nephrotic syndrome? (5)
1. MPGN (I or II)
2. membranous glomerulopathy
4. IgA nephropathy
5. minimal change disease (less likely, seen MC in kids)
what is the differential of an adult presenting with nephritic syndrome? (4)
1. Crescentic glomerulonephritis
3. IgA nephropathy
4. Less likely: acute proliferative (anti-strep) GN
what is the differential of an adult presenting with microscopic hematuria? (4)
2. IgA nephropathy
3. thin membrane disease
4. Alports syndrome
which disease is associated with the following EM finding:
"Humps" - large subepithelial deposits of immune complexes
Acute Proliferative (Post-Strep) Glomerulonephritis
which disease is associated with the following EM finding:
mesangial immune complexes
IgA nephropathy
which disease is associated with the following EM finding:
effacement of podocytes
minimal change disease
which disease is associated with the following EM finding:
discrete immune deposits within glomerular basement membrane loops
Membranous glomerulopathy
membranoproliferative glomerulonephritis
what am i?
a syndrome consisting of purpuric skin lesions of the extensor surfaces of the extremities and buttocks; abdominal pain, vomiting and intestinal bleeding, nonmigratory arthritis and renal abnormalities?
henoch-schonlein purpura (HSP)
what is always seen in an HSP renal biopsy?
HSP is most commonly found in what age group?
3-8 yrs
(can be found in any age group)
how does HSP manifest differently in an adult as compared to a child?
adult has more severe renal manifestations
children have a better prognosis
any etiology of HSP?
strong history of atopy
skin manifestations of HSP?
subepidermal hemorrhages and vasculitis with IgA deposition
GI manifestations of HSP?
focal vasculitis with IgA deposition
Microscopic renal manifestations of HSP?
IgA deposits in mesangium
prognosis for HSP?
excellent (esp. in children)
poorer if nephrotic syndrome or crescents are seen.
therapy for HSP?
how often are renal manifestations seen in SLE patients?
(>70% of the time)
renal manifestations of SLE are more severe in what populations?
1. blacks
2. children
3. males
what is the etiology of renal manifestations in SLE?
circulating immune complexes are deposited within the glomerulus.
besides glomerular involvement, what are three other renal manifestations of SLE?
1. interstitial inflammation and fibrosis
2. focal vasculitis
3. tubular atrophy
what immune particles are present in the glomerulus in an SLE patient?
"full house"
IgG, IgM, IgA, C3, C1q
what are the 6 classifications of SLE as seen under the light microscope?
Class I: normal
Class II: immune deposits in mesangium
Class III: <50% proliferative
Class IV: >50% proliferative
Class V: membranous (capillary loop thickening w/o proliferation)
Class VI: end stage sclerotic disease
complications of renal manifestations of SLE?
renal failure
(cause of death in 1/3 of SLE patients)
therapy for SLE?
how do renal manifestations of diabetes progress to proteinuria and renal failure?
there is accumulation of basement membrane material in the capillaries and mesangium
what type of diabetic is more likely to have renal manifestations that progress to renal failure?
poorly controlled diabetes. (Duration and severity of hyperglycemia are related to renal manifestations and prognosis)
what is another name for the renal manifestations of diabetes?
Kimmelstiel-Wilson disease
(nodular diabetic glomerulosclerosis)
what are the sites involved in the renal manifestations of diabetes?
(Basically everything)
glomerulus, mesangium, capillary basement membranes, tubular and vascular channels of the kidney.
regarding renal manifestations of diabetes:
what would you see in the efferent and afferent arterioles?
hyaline arteriosclerosis
prognosis of nodular diabetic glomerulosclerosis?
poor (if hyperglycemia nor controlled)
-changes are irreversible
- if hyperglycemia not managed -> progresses to renal failure w/i 6 yrs of presentation
renal manifestations of bacterial endocarditis?
deposition of immune complexes in the glomerulus (and subsequent reaction).
*treat endocarditis and this usually resolves*
what would be seen in an EM of amyloid deposits?
beta-pleated sheets, fibrillary material
are patients with amyloid candidates for a renal transplant?
amyloid will recur
where in the kidney does amyloid deposit? (3)
1. glomerulus
2. interstitium
3. around renal blood vessels
microscopic description of multiple myeloma in the kidney?
casts in tubules
how does essential mixed cryoglobulinemia alter the kidney?
produces thrombi in glomerular capillaries
renal manifestations of Waldenstroms macroglobulinemia?
IgM-kappa protein deposits in glomeruli
goodpastures disease produces what kind of antibodies?
anti-glomerular basement membrane
(actually antibodies to collagen IV)
IF pattern of goodpastures?
LINEAR staining of IgG and compliment
Goodpastures is more common in what population?
young males
renal microscopic characteristics of goodpastures?
crescentic or proliferative glomerulonephritis
clinical presentation of goodpastures?
nephritic syndrome, hematuria
rapid renal failure
prognosis and treatment of goodpastures?
rapidly fatal without treatment
*treat with immunosuppressive drugs and plasma exchange*
two other common systemic vasculitis diseases with renal manifestations?
1. Wegeners granulomatosis
2. Polyarteritis nodosa
why is the kidney so susceptible to ischemic damage?
*little to no collateral circulation
why does ischemia make the renal effects of hypertension worse?
ischemia stimulates the kidney to release vasoactive substances (renin)
*vasoconstriction worsens HTN and accelerates ischemic damage
hypertension is defined as a disease of what size vessels?
small vessels
renal disease that is caused by mild to moderate hypertension is called?
benign nephrosclerosis
(aka. arteriolonephrosclerosis)
3 risk factors for benign nephrosclerosis?
1. increased age
2. black
3. male
etiology of benign nephrosclerosis?
arteriolosclerosis causes parenchymal ischemia -> results in atrophy and small foci of fibrosis
which part of the kidney is affected by benign nephrosclerosis?
renal cortex
(small arteries and arterioles)
gross manifestations of benign nephrosclerosis?
* diffuse, regular granularity of cortical surface
* reduction of kidney size
in benign nephrosclerosis: what would be seen microscopically in the arteroles and small arteries?
hyaline thickening of walls
in benign nephrosclerosis: what would be seen microscopically in the glomeruli?
in benign nephrosclerosis: what would be seen microscopically in the tubules and interstitium?
tubules: tubular atrophy
interstitium: fibrosis
2 Lab characteristics for diagnosis of benign nephrosclerosis?
1. azotemia
2. granular or waxy casts in urine
malignant hypertension is defined as what pressure?
diastolic >130 mmHg
most common cause of death in malignant HTN?
malignant HTN is more commonly found in what population?
young to middle aged males
(more common in blacks)
malignant HTN is associated with very high levels of what 3 renal hormones?
1. renin
2. angiotensin
3. aldosterone
types of damage caused by malignant HTN?
significance of this damage?
1. vascular necrosis
2. endothelial cell damage
3. platelet thrombi
4. intravascular coagulation
*all produce ischemia -> more renin released -> cycle aggravated*
in malignant HTN: gross appearance of a kidney?
(multiple small hemorrhages on surface)
in malignant HTN: describe the microscopic finding of necrotizing arteriolitis
fibrinoid necrosis of the arterioles (see fibrin deposition and inflammatory infiltrate in arterioles)
in malignant HTN: describe the hyperplastic arteriolitis seen microscopically
intimal thickening
in malignant HTN: describe the necrotizing glomerulitis seen microscopically
necrotic glomeruli
- infiltrated with neutrophils
- thrombosed capillary loops
clinically, how does malignant HTN present?
visual impairments "aura"
what is a "Goldblatt" kidney?
an ischemic kidney (from RAS) that releases renin and other vasoactive substances
which population more commonly experiences Renal Artery Stenosis (RAS)?
young women
what exactly happens to cause the acquired stenosis seen in RAS?
fibromuscular dysplasia
(hypertrophy of a layer in the renal artery wall)
besides fibromuscular dysplasia, what is the other cause of RAS?
atheromatous plaque or emboli
which layer of the arterial wall most commonly undergoes fibromuscular dysplasia?
the media
what is a hallmark feature that characterizes fibromuscular dysplasia causing RAS?
renal artery looks like a "string of beads" on an arteriogram
prognosis for RAS?
*represents a rare form of curable HTN*
if diagnosed and treated promptly complete reversal of both HTN and renal damage occurs
what is the treatment for RAS caused by fibromuscular dysplasia?
surgical removal of affected vessel segments.
what is the common pathogenesis seen in all thrombotic microangiopathies?
endothelial cell damage and activation with intravascular thrombosis
which thrombotic microangiopathy follows a GI or respiratory illness in a young child?
Hemolytic Uremic Syndrome (HUS)
what are the three components of Hemolytic Uremic Syndrome?
1. microangiopathic hemolytic anemia
2. thrombocytopenia
3. acute renal insufficiency
what type of GI infection does HUS normally follow?
verocytotoxin producing E.coli gastroenteritis
how is HUS different when seen in older children and adults?
more severe
(higher morbidity and mortality)
appearance of skin in HUS?
purpuric lesions
microscopic appearance of HUS?
fibrin thrombi in small vessels throughout the body
(kidney = glomeruli, afferent arterioles, intralobular arteries)
in HUS: what clinical presentation would indicate a poor prognosis?
neurological symptoms
what would be seen in the lab results of an HUS patient? (5)
1. schistocytes
2. hemolytic anemia
3. azotemia
4. thrombocytopenia
5. reticulocytosis
therapy for HUS? (4)
1. plasma infusion
2. plasmaphoresis
3. vincristine
4. gamma globulin
what are the 4 clinical settings in which hemolytic uremic syndrome is seen?
1. infection associated
2. post-partum
3. secondary HUS (seen w/ other vascular renal diseases)
4. hereditary
prognosis for post-partum HUS?
grave. most die despite aggressive treatment
which population is thrombotic thrombocytopenic purpura (TTP) MC seen in?
young women
what is the difference between TTP and HUS?
*similar syndromes*
TTP - dominated by systemic effects (non-renal)
HUS - dominated by renal effects
what are the 5 classic symptoms of TTP:
1. fever
2. thrombocytopenia
3. microangiopathic hemolytic anemia
4. neurologic defects
5. renal failure
therapy for TTP?
exchange transfusions
(80% success rate, rest fatal)
a person with atherosclerotic disease has a risk of what following surgery or catheterization of the aorta?
atheroembolic renal disease
(emboli resulting in renal ischemia)
what are the clinical signs of a kidney infarction?
sharp flank or abdominal pain
what does sickle cell nephropathy result from?
accerated sickling in hypertonic medulla.
(decreased concentrating ability, hematuria, proteinuria seen)
which vascular disease of the kidney is a rare complication of massive hemorrhage (usually from an obstetric emergency)?
diffuse cortical nephropathy
manifestations of eclampsia in the kidney?
*swollen glomeruli with little blood.
* severe maternal HTN
define acute tubular necrosis
all forms of acute renal failure associated wtih the injury, functional loss and anatomic destruction of the tubular epithelial cells of the kidney.
define acute renal failure
syndrome associated with the acute suppression of renal function
*ofen accompanied by oliguria or anuria*
what are the three clinical phases ATN can be divided into?
what is the "clue" during the initiation phase that signifies oncoming acute renal failure?
rising BUN w/o a decrease in urine output
what characterizes the maintenance phase of acute renal failure?
(lasting 5-7 days)
when would a patient have non-oliguric renal failure?
following certain nephrotoxic agents (such as methoxyflurane)
which is more clinically benign: oliguric or non-oliguric acute renal failure?
non-oliguric renal failure
what characterizes the recovery phase of acute renal failure?
in which phase of acute renal failure do most fatalities occur?
maintenance (oliguric)
*2/3 of fatalities in this stage*
in which phase of acute renal failure is the patient most susceptible to infection?
recovery phase
ATN is classified into what two groups?
1. ischemic ATN
2. nephrotoxic ATN
what is pigment-induced ATN and which ATN category does it fall under?
*ATN that follows
1. massive hemolysis and release of heme proteins
2. severe skeletal injury with release of muscle myoglobin
*falls under ischemic ATN*
Ischemic ATN is most common after episodes of what?
severe circulatory collapse
describe the gross changes seen in ATN
*kidneys are swollen, heavy
*pale cortex, dark medulla
what is seen microscopically in both forms of EARLY ATN (3)
* hydropic changes
* accumulation of leukocytes in vasa recta
* tubular dilitation
what is seen microscopically in the maintenance phase of ATN (both types)? (3)
*epithelial cell damage and necrosis
* tubular dilitation
* casts
what is seen microscopically in the recovery phase of ATN (both types)? (2)
* epithelial cell regeneration
* restoration of ultrastructure
Two major microscopic differences between ischemic ATN and nephrotoxic ATN?
* basement membrane is ruptured in Ischemic ATN (tubulorrhexis); preserved in nephrotoxic ATN

* Skip lesions in ischemic ATN, uniform involvement of a given segment (with nephrotoxic ATN)
what is the most vulnerable part of the nephron?
straight proximal tubule
in which type of ATN do we see Tamm-Horsfall protein casts?
ischemic ATN
which part of the nephron, if affected, is most likely to be ischemic ATN?
distal tubule
in nephrotoxic ATN - at which part of the nephron would we look at to get a clue as to what the insulting agent was?
proximal tubule
describe the damaging agent if the following were observed in the proximal tubule:
large acidophilic inclusion, necrotic epithelial cells, prominent calcification
Mercury salts
describe the damaging agent if the following were observed in the proximal tubule:
neutral lipid inclusions, fatty change, necrosis without calcification
Carbon Tetrachloride
describe the damaging agent if the following were observed in the proximal tubule:
hydropic or vacuolar degeneration of the proximal tubule with calcium oxalate crystals in the tubular lumen
ethylene glycol
describe the damaging agent if the following were observed in the proximal tubule:
large eosinophilic nuclear inclusion bodies. little calcification, no crystal formation
describe the following theory in the pathogenesis of ATN:
persistent preglomerular arteriolar vasoconstriction
* activation of vasoconstrictive agents I(angiotensin II, throboxane, catecholamines)
* loss of vasodilator effects (prostaglandins)
**net result is decrease in renal blood flow -> decrease in GFR -> oliguria**
describe the following theory in the pathogenesis of ATN:
tubular backflow
tubular contents leak ino the interstitium (after destroyed tubular integrity) and further alter renal function and fluid flow
describe the following theory in the pathogenesis of ATN:
tubular obstruction
casts and proteinacous material obstruct. this increases intratubular pressure, produces decreased tubular flow and decreased GFR.
which sex is pyelonephritis more common in?
the pathogenesis of pyelonephritis is dependant on what four factors:
1. overgrowth of non-native flora
2. residual urine in bladder
3. reflux from bladder into renal pelvis and calices
4. entry of bacteria through papillae into renal parenchyma
four conditions in which reflux urine is more common?
1. prostatic obstruction
2. neurogenic bladders
3. pregnancy
4. diabetic
what two things prevent vesiculo-ureteral reflux of urine?
1. steep angle of insertion of urethra into bladder wall
2. long course of ureter through bladder wall
*micturition occludes ureter*
what is the difference between a simple and compound papillae when it comes to pyelonephritis?
simple papillae - convex, resist urine reflux
compound papillae - concave, allow easier access or refluxed urine
when looking at a gross crossection of a kidney with pyelonephritis, what would indicate more severe damage? (3)
1. thinned cortex
2. blunted papillae
3. necrosis of papillary tips
what would be seen in the collecting ducts of an acute pyelonephrotic kidney?
neutrophils (in aggregates or casts)
what is seen upon the gross examination of a kidney of chronic pyelonephritis?
irregular and coarse corticomedullary scars
- overlie blunted, dilated and deformed calices.
- scars are more common at the poles of the kidney
what is characteristically seen upon microscopic examination of the chronic pyelonephritis kidney?
(dilated tubules filled with colloid material)
what is seen microscopically in xanthogranulomatous pyelonephritis
see foamy macrophages, plasma cells, occasional giant cells
(alse see large yellow-orange nodules resembling renal cell carcinoma)
Acute Allergic Interstitial Nephritis is most commonly caused by?
(damage is not caused by the drugs themselves but by the allergic rxn. to them)
how can acute allergic interstitial nephritis be differentiated from ATN?
allergic rxns. are not dose dependant
(in ATN they are)
*also there is more interstitial inflammation than in ATN
which drugs is acute allergic interstitial nephritis MC seen with? (3)
synthetic penicillins
in acute allergic interstitial nephritis, which cells comprise the majority of the immune response?
T lymphocytes
microscopically, what is seen in acute allergic interstitial nephritis? (3)
1. chronic inflammation
2. eosinophils
3. edema in interstitium
where in the kidney is most of the involvement in acute allergic interstitial nephritis?
corticomedullary junction
treatment for acute allergic interstitial nephritis?
early diagnosis, remove offending drug
analgesic nephropathy is associated with?
chronic use of phenacetin containing analgesic compounds
pathologic characteristic of analgesic nephropathy?
papillary necrosis
microscopic characteristic of analgesic nephropathy?
scant inflammation
brownish discoloration
clinical s/s of analgesic nephropathy?
1. anemia
2. premature aging
3. brown skin lesions
(symptomatic in late stages)
three types of renal disease caused by multiple myeloma?
1. amyloidosis
2. glomerular disease (from light chain deposits)
3. tubulointerstitial disease (light chain cast nephropathy)
What is urate nephropathy?
deposition of urate crystals in the tubules or interstitium
what are some examples of high urate levels that could cause urate nephropathy?
gout, chemotherapy, chronic lead intoxication, leukemia, polycythemia
what is a tophus?
urate crystals that are acompanied by an inflammatory reaction and giant cells.
what is nephrocalcinosis?
precipitation of calcium phosphate in renal tubules
what is a predisposing condition for nephrocalcinosis?
what exactly is damaged in hypokalemic nephropathy?
disturbance in tubular concentrating mechanism due to decreased interstitial sodium (this interferes with countercurrent exchange)
define hydronephrosis
dilation of the renal pelvis and calyces associated with progressive atrophy of the kidney due to obstruction of urine outflow
in ostructive uropathy, which part of the nephron is affected first and last?
first - tubules
last - glomeruli
what happens if there is unilateral obstructive nephropathy?
it is usually asymptomatic and not found until irreversible damage has occured
how does complete bilateral obstruction present?
*must be relieved for survival*
what is a urolithiasis?
kidney stone
(calculi within the renal pelvis, calyces or dislodged to lower UT)
how does a urolithiasis present?
are renal stones more common in males or females?
what do we think when a child has a renal stone?
hereditary cystinuria
which sites of the urinary tract are MC affected in urolithiasis?
NARROWING areas (renal pelvis, calyces)
what does a calcium phosphate or oxalate crystal look like?
hard, sharp edges
pale to dark brown
characteristic of uric acid crystals?
what are staghorn crystals?
casts of the calyces - associated with chronic pyelonephritis.
complications of renal stones? (3)
increased incidence of infections
which type of stone is visible on abdominal X-ray?
which type isn't?
calcium stones are visible
uric acid stones are not
three reasons a patient would be ineligible for a renal transplant?
1. renal disease (ie. amyloid)
2. co-morbid disease (ie. severe atherosclerosis)
3. advanced age
what causes a hyperacute kidney rejection?
INCOMPATIBLE KIDNEY - mismatched organ and blood types
what is the most common cause of an acute kidney rejection?
less than optimal dosage of anti-rejection drugs
(revered by increasing dosing)
is chronic rejection reversible?
NO (interstitial fibrosis)
which type of rejection accounts for the most common cause of long-term graft loss?
chronic rejection