Block VII, WeekVI
Terms
undefined, object
copy deck
- 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?
-
potassium
(causes arrythmias) - CV/Pulmonary manifestations of renal failure? (4)
-
volume overload CHF/pulmonary edema
HTN
pericarditis
arrythmias - 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?
-
normal
(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?
- oligohydramnios
- 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?
- benign
- 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?
- MCKD1 and MCKD2
- 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?
-
dialysis
(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?
- 20%
- 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?
- mesangium
- 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?
-
diffuse
focal
segmental
global - 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?
- hematuria
- clinical correlation with mesangial matrix increase?
- decreased GFR
- clinical correlation with effacement of epithelial foot processes?
- proteinuria
- 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
neutrophils
platelets
monocytes - four soluble mediators released during glomerular immune-mediated injury?
-
cytokines
growth factors
compliment
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?
- IgG
- in Goodpastures syndrome the immunoglobulin deposition is what shape?
- linear
- 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?
- Anti-Neutrophil-Cytoplasmic-Antibodies
- 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?
-
children
(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?
-
poor
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?
-
poor
*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?
- C3
- 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
2. FSGS
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)
-
corticosteroids
cyclophosphamide - 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?
- NONE
- IF findings seen in minimal change disease?
- NONE
- 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?
-
rare
(infections, renal vein thrombosis, interstitial nephritis) - treatment for minimal change disease?
- corticosteroids
- minimal change disease is MC seen in which age group?
- children
- 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?
- blacks
- 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?
-
corticosteroids
cyclophosphamide
NSAIDs - 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
M>F
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?
-
NO!
patients are offered dialysis or renal transplants - what is the differential of a child presenting with nephrotic syndrome? (3)
-
1. membranoproliferative glomerulonephritis (type I)
2. FSGS
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)
-
1. FSGS
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
3. FSGS
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
2. MPGN
3. IgA nephropathy
4. Less likely: acute proliferative (anti-strep) GN - what is the differential of an adult presenting with microscopic hematuria? (4)
-
1. FSGS
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
FSGS -
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?
- IgA
- 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?
-
supportive
NSAID
prednisone - how often are renal manifestations seen in SLE patients?
-
often
(>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?
-
corticosteroids
immunosuppressants - 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?
-
NO
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?
-
hemoptysis
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?
- glomerulosclerosis
- 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?
- CVA
- 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?
-
"flea-bitten"
(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
-
"onionskinning"
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?
-
headaches
nausea
visual impairments "aura"
convulsions - 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