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351 Cards in this Set
- Front
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|
define azotemia
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elevation of BUN and Creatinine; related to a decrease in GFR; asymptomatic
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deifine uremia
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(basically) azotemia with symptoms
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three types of azotemia and major reasons for these types?
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1. pre-renal - reduced perfusion of the kidneys
2. renal - intrinsic renal disease 3. post-renal - obstruction of urine flow past the kidney |
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what metabolic changes will be present in renal failure? Why?
|
metabolic acidosis with an anion gap
(secondary to decreased bicarbonate and accumulation of acids) |
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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+) |
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which electrolyte disturbance is considered a "major life-threatening" disturbance?
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potassium
(causes arrythmias) |
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CV/Pulmonary manifestations of renal failure? (4)
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volume overload CHF/pulmonary edema
HTN pericarditis arrythmias |
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hematologic abnormalities seen in renal failure?
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normocytic, normochromic anemia
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manifestations of renal failure in bones? (3)
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renal osteodystrophy
secondary hyperparathyroidism altered calcium metabolism |
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three clinical signs of nephritic syndrome?
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1. gross hematuria
2. hypertension 3. proteinuria (mild to moderate) |
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four clinical signs of nephrotic syndrome?
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1. heavy proteinuria
2. hypoalbuminemia 3. severe edema 4. hyperlipidemia, lipiduria |
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general pathophysiology of nephritic syndrome?
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usually acute onset.
glomerular diseases, usually proliferative (inflammatory)in nature |
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general pathophysiology of nephrotic syndrome?
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alterations of glomerular function or structure. Effacement of foot processes is present.
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what does asymptomatic hematuria or proteinuria usually indicate?
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mild glomerular abnormalities
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what are the four "levels" of chronic renal failure?
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1. diminished renal reserve
2. renal insufficiency 3. renal failure 4. end stage renal disease |
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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 |
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what are the BUN and creatinine levels in diminished renal reserve?
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normal
(due to reserve capacity of the kidney) |
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what are the three clinical signs of renal tubular defects?
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1. polyuria
2. nocturia 3. electrolyte imbalances |
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how common are congenital anomalies of the kidney?
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common (~10% of all people)
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what is often seen along with bilateral renal agenesis?
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abnormal lung development in utero
(incompatible with life) |
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what constitutes renal hypoplasia?
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a small, unscarred kidney with fewer than six pyramids
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1. what is an ectopic kidney?
2. consequences? |
1. incorrect position of the kidneys (usually in pelvis)
2. -predisposed to infection and backflow |
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renal dysgenesis or agenesis causes what in regards to the amniotic fluid?
|
oligohydramnios
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common placental manifestation of oligohydramnios?
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amnion nodosum
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common fetal manifestations of oligohydramnios? (4)
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Potter sequence
1. lung hypoplasia 2. altered facies 3. defects in hands and feet 4. breech position |
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describe the most common shape abnormality of a malformed kidney.
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horseshoe kidney - two kidneys are fused at poles forming a single kidney
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genetic associations of autosomal recessive polycystic kidney disease (infantile)
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PKHD1 gene that encodes fibrocystin
*unknown mechanism* |
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pathologic features of autosomal recessive polycystic kidney disease (infantile)?
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enlarged, cystic kidneys
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complications of autosomal recessive polycystic kidney disease (infantile), fetus and infant?
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FETAL - pulmonary hypoplasia (fatal)
INFANTILE - hepatic fibrosis |
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typical outcome of autosomal recessive polycystic kidney disease (infantile)?
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stillborn or death in infancy or childhood
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differences in heredity between infantile and adult polycystic kidney disease?
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infantile - autosomal recessive
adult - autosomal dominant, high penetrance |
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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 |
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common clinical complication in autosomal dominant adult polycystic kidney disease?
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Berry aneurysms
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pathologic features in autosomal dominant adult polycycstic kidney disease?
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large multicystic kidneys
liver cysts |
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5 Clinical features of autosomal dominant adult polycystic kidney disease?
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1. hematuria
2. flank pain 3. UTI 4. renal stones 5. HTN |
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typical outcome of autosomal dominant adult polycystic kidney disease?
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chronic renal failure beginning at age 40-60
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inheritance of cystic renal dysplasia?
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NONE - there is no familial component
|
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what is cystic renal dysplasia?
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congenital malformation of kidney with formation of cysts and presence of abnormal histologic structures (ie. bone, cartilage). No renal organization.
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typical outcome of bilateral cystic renal dysplasia?
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fatal during fetal development
|
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typical outcome of unilateral cystic dysplasia?
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benign course, asymptomatic
(may see increase in infections, reflux) |
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define medullary sponge kidney
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cystic dilation of collecting ducts in medulla
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clinical features of medullary sponge kidney?
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usually asymptomatic
may see - hematuria, UTIs, recurrent renal stones |
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Typical outcome of medullary sponge kidney?
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benign
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inheritance pattern of familial juvenile nephronophithisis (uremic medullary cystic disease)?
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autosomal recessive
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genetic pathogenesis behind familial juvenile nephronophthisis (uremic medullary cystic disease)?
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mutations in NPH1, NPH2, NPH3
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pathologic features of familial juvenile nephronophthisis (uremic medullary cystic disease)?
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corticomedullary cysts, shrunken kidneys
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typical outcome of familial juvenile nephronophthisis (uremic medullary cystic disease)?
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progressive renal failure beginning in childhood
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4 clinical features of familial juvenile nephronophithisis (uremic medullary cystic disease)?
|
1. salt wasting
2. polyuria 3. growth retardation 4. anemia |
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inheritance pattern of adult onset medullary cystic disease?
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autosomal dominant
|
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genetic pathogenesis behind adult onset medullary cystic disease?
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MCKD1 and MCKD2
|
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pathologic features of adult onset medullary cystic disease?
|
corticomedullary cysts, shrunken kidneys
(same as juvenile onset medullary cystic disease) |
|
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2 clinical features of adult onset medullary cystic disease?
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1. salt wasting
2. polyuria |
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typical outcome of adult onset medullary cystic disease?
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chronic renal failure beginning in adulthood
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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 |
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MC cause of acquired cysts?
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dialysis
(these cysts are small) |
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thing we should remember about von Hipple-Lindau disease?
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increased susceptibility to renal cell and other cancers
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what proportion of the total blood supply does the kidney receive?
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20%
|
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which side of the circulation are the glomerular capillaries (both efferent and afferent poles)?
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arterial side
|
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what is the name of the matrix that supports the glomerular tufts?
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mesangium
|
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what are the two types of cells found in the mesangium and what is their function?
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type 1 - contractile
type 2 - secretory |
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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 |
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what type of filter are the visceral epithelial cells (podocytes)?
|
size and charge filter
(anionic charged) |
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what structure separates adjacent podocytes?
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slit diaphragm
|
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four places type IV collagen is found?
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1. glomerular basement membrane
2. eye 3. ear 4. lung |
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what type of filter does the glomerular basement membrane act as?
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charge and size filter
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what type of filter does the endothelial cell act as?
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charge and size filter
- anionic charge - restricts proteins about the size of albumin |
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what happens to the ultrafiltrate once it reaches Bowman's space?
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enters tubule system, then is excreted in the urine
|
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what are the epithelial cells called that line Bowman's space?
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parietal epithelium
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what are the four terms used to describe the extent of glomerular disease?
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diffuse
focal segmental global |
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what does diffuse glomerular involvement mean?
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most or all the glomeruli in the kidney are affected
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what does focal glomerular involvement mean?
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only some of the glomeruli affected
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what does segmental glomerular involvement mean?
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only part of an individual glomerulus is affected
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what does global glomerular involvement mean?
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the entire glomerulus is affected
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what are the three general glomerular patterns of injury?
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1. Membranous patterns
2. Sclerosis 3. Poliferative (inflammatory) patterns |
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describe membranous patterns of glomerular injury
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diffuse, acellular thickening of the capillary loops without an increase in cellularity in the glomerulus. capillary loops remain patent.
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describe the sclerotic pattern of glomerular injury
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sclerotic changes and fibrosis in the glomerulus
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proliferative patterns of glomerular injury may result from what two general patterns?
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1. hypercellularity of glomerular cells (esp. in mesangium)
2. infiltration of inflammatory cells |
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clinical manifestations of segmental sclerosis, mesangioproliferative and membranous glomerular disease?
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hematuria and/or proteinuria
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clinical manifestations of crescentic glomerular injury?
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Acute renal failure
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clinical manifestation of chronic sclerosed glomerular disease?
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renal failure
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what is the mildest form of proliferative glomerular injury?
|
mesangioproliferative glomerulonephritis
(hypercellularity of mesangium only) |
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what is proliferative glomerulonephritis?
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hypercellularity of capillaries and mesangium (this may include inflammatory cells)
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what is acute proliferative glomerulonephritis?
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proliferative glomerulonephritis with a predominance of neutrophils
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what is necrotizing glomerulonephritis?
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necrosis of glomeruli and nuclear debris
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what is membranoproliferative glomerular disease?
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hypercellularity of the glomerulus WITH thickened capillary walls
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what is crescentic glomerulonephritis?
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proliferating epithelial cells with inflammation in Bowman's space. forms concentric layers (crescents) around the glomerular tuft. Ominous sign
|
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is chronic sclerosis reversible?
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NO - this is the end stage of most glomerular and renal diseases
|
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clinical correlation with mesangial cell hypercellularity?
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hematuria
|
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clinical correlation with mesangial matrix increase?
|
decreased GFR
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clinical correlation with effacement of epithelial foot processes?
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proteinuria
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what is the etiology of most glomerular diseases?
|
immune mediated
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In an immune mediated injury which cells are found to increase in the glomerulus? (4)
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mesangial cells
neutrophils platelets monocytes |
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four soluble mediators released during glomerular immune-mediated injury?
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cytokines
growth factors compliment activation of coagulation cascade |
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anti GBM antigen is actually directed against what?
|
type IV collagen
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which immunoglobulin is most commonly seen in in situ immune complex formation?
|
IgG
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in Goodpastures syndrome the immunoglobulin deposition is what shape?
|
linear
|
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what is a "pauci-immune" glomerular disease and how is it distinguished from an immune complex disease?
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pauci-immune means there appears to be an immune response mechanism, yet there are no immune complexes within the glomerulus.
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ANCA stands for?
|
Anti-Neutrophil-Cytoplasmic-Antibodies
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when does acute proliferative (post-strep) glomerulonephritis MC occur?
|
1-2 wks after recovery from infectious disease (usually strep pharyngitis)
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acute proliferative (post-strep) glomerulonephritis MC occurs in which age group?
|
children
(does occur in adults and is more severe in adults) |
|
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gross findings seen in acute proliferative (post-strep) glomerulonephritis?
|
flea-bitten kidney (hyperemic glomeruli causes a punctate hemorrhagic appearance)
|
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microscopic manifestations of acute proliferative (post-strep) glomerulonephritis?
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* proliferation of mesangial and endothelial cells within the glomerulus
* neutrophils present * +/- crescents |
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immunoflourescence manifestations of acute proliferative (post-strep) glomerulonephritis?
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see granular deposition of IgG and C3. "lumpy-bumpy"
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EM manifestations of acute proliferative (post-strep) glomerulonephritis?
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"lumps and bumps"
large subepithelial immune complexes |
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two hallmark clinical presentations of acute proliferative (post-strep) glomerulonephritis?
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1. nephritis with oliguria
2. periorbital edema |
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features of a laboratory diagnosis of acute proliferative (post-strep) glomerulonephritis? (4)
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1. hematuria with RBC casts
2. decreased C3 3. elevated strep antibodies 4. renal biopsy if necessary |
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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 |
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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* |
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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 |
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clinical presentation of patients with crescentic (rapidly progressive) Glomerulonephritis? (2)
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1. nephritic syndrome
2. progressive renal failure |
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Lab test used to diagnose crescentic (rapidly progressive) Glomerulonephritis?
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renal biopsy
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prognosis of crescentic (rapidly progressive) Glomerulonephritis?
|
poor
most progress to irreversible renal failure |
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therapy for crescentic (rapidly progressive) Glomerulonephritis? (3)
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1. corticosteroid
2. immunosuppressive 3. dialysis and/or renal transplant in the case of renal failure |
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what are the two main characteristics of membranoproliferative glomerulonephritis (MPGN)?
|
1. basement membrane thickening
2. mesangial proliferation |
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which age group is afflicted by membranoproliferative glomerulonephritis (MPGN)?
|
children and young adults
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which type of MPGN is more often associated with systemic diseases?
|
type I
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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 |
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etiology of type II MPGN?
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activation of alternate compliment pathway
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which glomerular disorder is characterized by "tram tracks" on a silver stain?
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MPGN types I and II
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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 |
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other name for type II MPGN?
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Dense Deposit Disease (DDD)
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prognosis of MPGN?
|
poor
*nearly all progress to renal failure *may recur in transplant |
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laboratory diagnostic techniques/values used for MPGN?
|
1. renal biopsy to distinguish from other GN
2. persistent low C3 levels 3. hematuria 4. azotemia |
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describe the new subtype (type III) of MPGN
|
deposits on both epithelial and endothelial sides of basement membrane
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describe the general microscopic characteristics seen in the membranoproliferative pattern of glomerular diseases.
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hypercellular, thick membrane
|
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which immunological component is always present in the membranoproliferative pattern of glomerular diseases?
|
C3
|
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which glomerular disease is a major cause of nephrotic syndrome in adults?
|
membranous glomerulopathy
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4 clinical characterstics of nephrotic syndrome?
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1. proteinuria (heavy)
2. hypoalbuminemia 3. hyperlipidemia, lipiduria 4. severe edema, anascara |
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3 glomerular diseases in which you see nephrotic syndrome?
|
1. minimal change disease
2. FSGS 3. membranous glomerulopathy |
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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 |
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in which age group is membranous glomerulopathy MC seen?
|
middle aged adults
|
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5 possible etiologies of membranous glomerulopathy?
|
1. chronic immune complex disease (most idiopathic)
2. hepB 3. drugs (gold, penicillamine) 4. SLE 5. occult cancer |
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site primarily involved in membranous glomerulopathy?
|
basement membrane of glomerulus
|
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microscopic characteristics of membranous glomerulopathy? (3)
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1. normal cellularity
2. thickening of GBM 3. "spikes" on silver stains |
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EM characteristics of membranous glomerulopathy?
|
small deposits in GBM
|
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IF characteristics of membranous glomerulopathy?
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granular C3 and IgG along GBM
|
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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 |
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therapy for primary membranous glomerulopathy? (2)
|
corticosteroids
cyclophosphamide |
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membranous glomerulonephritis is similar to?
|
"Heymann nephritis"
(experimental model for immune mediated GN) |
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what is the most frequent cause of nephrotic syndrome in childhood?
|
minimal change disease
|
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miscroscopic findings seen in minimal change disease?
|
NONE
|
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IF findings seen in minimal change disease?
|
NONE
|
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EM findings seen in minimal change disease?
|
effacement of epithelial cell foot processes
villous hyperplasia swelling of glomerular epithelial cells |
|
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complications of minimal change disease?
|
rare
(infections, renal vein thrombosis, interstitial nephritis) |
|
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treatment for minimal change disease?
|
corticosteroids
|
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minimal change disease is MC seen in which age group?
|
children
|
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proposed etiology of minimal change disease?
|
immunologic basis with possible cytokine release and T cell dysfunction
|
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how are minimal change disease and focal segmental glomerular nephritis (FSGN) differentiated?
|
FSGN gets worse despite corticosteroid therapy, minimal change disease regresses.
|
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in which population is focal segmental glomerulosclerosis more common and severe in?
|
blacks
|
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what is FSGS characterized by?
|
collapse, sclerosis and hyalinosis of focal glomerular segments
|
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prognosis of FSGS?
|
progresses to renal failure within 10 yrs
|
|
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etiology of FSGS?
|
most idiopathic or part of another renal disease (ie. reaction to chronic proteinuria)
* HIV, heroin use? |
|
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microscopic manifestations of FSGS?
|
FIBROSIS - segmental collapse of glomerular tufts with sclerosis and hyalinosis
|
|
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EM manifestations of FSGS?
|
folding and thickening of basement membrane with effacement of foot processes
|
|
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which immune related molecules are found in FSGS and where are they found?
|
IgM and C3 are found in areas of sclerosis
|
|
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clinical presentation of FSGS?
|
nephrotic syndrome unresponsive to corticosteroids.
|
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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)
|
|
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IF manifestations of IgA nephropathy?
|
demonstrates a predominance of IgA
* also see strong C3 * no C1q, C4 |
|
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EM manifestations of IgA nephropathy?
|
see mesangial immune deposits.
|
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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)
|
|
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IgA nephropathy is the most common cause of what clinical sign?
|
gross or microscopic hematuria
|
|
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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.
|
|
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in which familial nephropathy is the basement membrane 1/2 to 1/3 normal size?
|
thin membrane disease
|
|
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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) |
|
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what is the differential of a child presenting with microscopic hematuria? (3)
|
1. FSGS
2. Thin basement membrane disease 3. Alports syndrome |
|
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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) |
|
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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
|
|
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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
|
|
|
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
corticosteroids (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
hematuria |
|
|
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?
|
initiation
maintenance recovery |
|
|
what is the "clue" during the initiation phase that signifies oncoming acute renal failure?
|
rising BUN w/o a decrease in urine output
(azotemia) |
|
|
what characterizes the maintenance phase of acute renal failure?
|
oliguria
(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?
|
diuresis
|
|
|
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 -or- 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 |
lead
|
|
|
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?
|
women
|
|
|
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?
|
"thyroidization"
(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?
|
Drugs
(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
cephalosporins sulfonamides |
|
|
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?
|
hypercalcemia
|
|
|
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?
|
anuria
*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?
|
pain
infection hematuria |
|
|
are renal stones more common in males or females?
|
males
|
|
|
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?
|
smooth
|
|
|
what are staghorn crystals?
|
casts of the calyces - associated with chronic pyelonephritis.
|
|
|
complications of renal stones? (3)
|
hydronephrosis
hydroureter 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
|
