renal robbins

Question 1

the study of kidney diseases is facilitated by dividing them into those that affect the four basic basic morphological components,

Answer 1

glomeruli, tubules, interstitium, and blood vessels

Question 2

most glomerular diseases are ___ mediated, whereas tubular and interstitial disorders are frequently caused by ___

Answer 2

immunologically, toxic or infectious agents

Question 3

azotemia

Answer 3

a biochemical abnormality that refers to an elevation of blood urea nitrogen(BUN) and creatitine levels, and is related largely to a decreased glomerular filtration rate(GFR). manifestation of acute or chronic kidney injury

Question 4

prerenal azotemia

Answer 4

when there is hypoperfusion of the kidneys(hemorrhage, hypotensive shock, voume depeltion of any cause that impairs renal function in the absence of primary renal paryenchymal damage, like CHF, liver cirrhosis)

Question 5

postrenal azotemia

Answer 5

seen whenever urine flow is obstructed distal to the kidney. removal of obstruction corrects the azotemia

Question 6

uremia

Answer 6

azotemia plus a constellation of clinical findings and biochemical abnormalities resulting from renal damage. secondary involvements of GI, peripheral nerves, heart(fibrinous pericarditis). table 20-1

Question 7

nephritic syndrome(basic def)

Answer 7

due to glomerular disease, presents with either visible hematuria or microscopic hematuria with dysmorphic red cells and red cell casts on urinalysis. diminished gfr, mild to moderate proteinuria, and hypertension

Question 8

nephrotic syndrome(basic def)

Answer 8

also due to glomerular disease, heavy proteinuria more than 3.5 g a day, hypoalbuminea, severe adema, hyperlipidemia, and lipiduria (lipid in the urine)

Question 9

asymptomatic hematuria or proteinuria

Answer 9

another manifestation of kidney pathology

Question 10

acute kidney injury

Answer 10

rapid decline in GFR(hours or days), most severe forms exhibit oliguria or anuria(reduced urine flow), can result from glomerular, insterstitial, vascuar, or tubular injury, dysregulation of fluid electrolyte balance and waste retention of urea and creatinine. can be reversible or progress to CKD

Question 11

chronic kidney disease(CKD)

Answer 11

chronic renal failure, mild, clinically silent, diminshed gfr less than 60ml/minute/1.73m^2 for at least 3 months or persistend albuminuria, CKD is irreversible. end result of all chronic renal parenchymal diseases

Question 12

end stage renal disease(ESRD)

Answer 12

GFR is less than 5 percent of normal, end stage of uremia

Question 13

rapidly progressive glomerulonephritis

Answer 13

signs of nephritic syndrome with rapid decline in GFR. implies severe glomerular injury

Question 14

isolated urinary abnormalities

Answer 14

glomerular hematuria and/or subnephrotic proteinuria

Question 15

difference between primary and secondary glomerular disease

Answer 15

primary stems from the kidney itself, secondary: diabetes, sle, vasculitis, amyloidosis(table 20-2)

Question 16

what is the most common cause of CRF/ESRD

Answer 16

diabetes. hypertension is second. also lupus

Question 17

describe the pthwy of filtration from the capillary to the urinary space

Answer 17

fenestrated endothelium, GBM-lamina rara interna, lamina densa(electron dense), lamina rara externa, podocyte filtration slits

Question 18

what kind of collagen is the gbm made of

Answer 18

4

Question 19

what does goodpasture syndrome/anti-gbm ab attach to

Answer 19

noncollagenous regions of collagen type 4

Question 20

mesangial cells

Answer 20

lie between the capillaries, embedded in mesangial matrix, they are contractile, phagocytic, and capable of proliferation, laying down matrix and collagen and secrete biologically active mediators. important in many forms of glomerulonephritis

Question 21

what can pass through the glomerular filtration barrier

Answer 21

small and cationic. albumin is large and negatively charged

Question 22

visceral epithelial cell

Answer 22

podocyte. slit diaphragm presents a size selective diffusion barrier to the filtration of proteins, synthesizes gbm components. slit diaphragms are inbetween foot processes. again, size elective diffusion barrier to proteins. damage to these results in proteinuria

Question 23

what are the four basic tissue responses to injury

Answer 23

hypercellularity, basement membrane thickening, hyalinosis and sclerosis,

Question 24

hypercellularity

Answer 24

increase in the number of cells in the glomerular tufts. results from one or more of the following: proliferation of mesangial or endothelial cells. infiltration of leukocytes, and that along with with swelling and proliferation of mesangial and/or endothelial cells, called endocapillary proliferation. formation of crescents

Question 25

crescents formation

Answer 25

proliferation of epithelial cells, mostly parietal, infiltrating leukocytes, after immune/inflammatory injury involving capillary walls, plasma proteins leak into urinary space, fibrin deposition

Question 26

hyalinosis

Answer 26

accumulation of homogenous, eosiniphilic material, amorphous, extracellular. usually a consequence of endothelial or capillary wall injury, and is typically the end result of various forms of glomerular damage

Question 27

sclerosis

Answer 27

deposition of extracellular collagenous matrix. may also result in obliteration of capillary lumens in affected glomeruli.

Question 28

diffuse

Answer 28

involving all glomeruli in the kidney

Question 29

global

Answer 29

involves entirety of individual glomeruli

Question 30

focal

Answer 30

involves only a fraction of glomeruli in the kidney

Question 31

segmental

Answer 31

affecting a part of each glomerulus

Question 32

mesangial

Answer 32

affecting mesangial regions

Question 33

chronic glomerular responses to injury include

Answer 33

basement membrane thickening, hyalinosis, and sclerosis

Question 34

membranous nephropathy

Answer 34

immune complexes are formed locally by ab that react with intrinsic tissue ag. also, heymann nephritis. reacts to pla2r, which is a protein in glomerular epithelial cell membrane. complement activation and then shedding of the immune aggregates form the cell surface to form characteristic deposits of immune complexes along the subepithelila aspect of the bm. granular immunoflourescence, thickened bm on light microscapy

Question 35

what are some ag that can be planted in the gomerulus

Answer 35

cationic molecules that bind to anionic components of glomerulus, dna, other nuclear proteins

Question 36

where are heymann and pla2r membranous nephropathy complexes seen

Answer 36

subepithelial space/epimembranous space

Question 37

what immunoflourescence pattern do planted and in situ immune complex membranous nephritis have?

Answer 37

granular

Question 38

what pattern does anti gbm have

Answer 38

linear

Question 39

what other basement membranes do anti gbm abs cross react with

Answer 39

lung alveoli, kindey lesion(goodpasture syndrome

Question 40

in glomerulonephritis from deposistion of circulating immune complexes, what are examples of ag

Answer 40

endogenous; sle, iga nephropathy. exogenous: post strep, hepatitis,

Question 41

in immune mediated glomeronephritis, what is the mechanism of injury

Answer 41

local inflammation, engagement of fc receptors on leukocytes, complement

Question 42

membranoproliferative pattern

Answer 42

subendothelial

Question 43

dense deposit disease

Answer 43

membranoproliferative glomerulonephritis II(MPGN type 2). alternative complement pthway

Question 44

___ are a feature of multiple types of glomerular injury including focal and segmental gomerulosclerosis and diabetic nephropathy

Answer 44

loss of podocytes

Question 45

once any renal disease destroys functioning nephrons and reduces gfr to ___ percent normal, progression to end stage renal failure(ESRF) proceeds at a steady rate independent of the original stimulus or activity of the underlying disease

Answer 45

30 to 50

Question 46

two major hisotological characteristics of progressive renal damage are

Answer 46

FSGS and tubulointerstitial fibrosis

Question 47

fsgs

Answer 47

focal segmental glomerulosclerosis. progressive fibrosis involving portions of some glomeruli develops after many types of renal injury and leads to proteinuria and increasing functional impairment. asoc w/ compensoatory hypertoryphy, eventually leads to total glomerular sclerosis and uremia

Question 48

tubulointerstitial fibrosis

Answer 48

tubilar damage and interstitial inflammation, is a componenet of many acture and chronic glomerulonephritides

Question 49

renal function decline is best correlated with

Answer 49

tubulointersitital damage rather than glomerular injury

Question 50

nephritic syndrome

Answer 50

inflammation in the glomeruli. hematuria, red cell casts in the urine, azotemia, oliguria, and mild to moderate hypertension

Question 51

acute proliferative comerulonephritis

Answer 51

includes poststrep, postinfectious. characterized histologically by diffuse proliferation of glomerular cells associated with influx of leukocytes. typically caused by immune complexes

Question 52

poststrep glomerulonephritis

Answer 52

1 to 4 weeks after a strep infection or the skin or pharynx. assoc with overcrowding and poor hygiene. typically age 6 to 10. fomred by immune complexes containing strep ag and ab formed in situ. subpepithelial HUMP LIKE DEPOSITS. granular deposits of igg, igm, c3 in the mesangium and bm. neutrophil in lumen. enlarged glomeruli, marked hypercellularity with proliferation of endothelial and mesangial cells, leukocyte infiltration, crescent formation in severe cases

Question 53

what is the clinical for post strep glomerulonephritis

Answer 53

slide 95/96

Question 54

rpgn

Answer 54

rapidly progressing crescentic glomerulonephritis. syndrome assoc with severe glomerular injury, but does not denote a specific etiology. severe oliguria, nephritic syndrome. can lead to death from renal failure within weeks to months. The most common histological feature is crescents. proliferation of parietal epithelial, and infiltration of monocytes and macr.

Question 55

what are the three types of rpgn

Answer 55

anti gbm, immune complex deposition, pauci immune

Question 56

what is the hla haplotype assoc with goodpasture/rpgn

Answer 56

drb1`

Question 57

what is the morphology of rpgn

Answer 57

crescents, ruptures in the gbm, blood cell leaking through bm, wrinkling of glomerular bm,

Question 58

nephrotic syndrome

Answer 58

derangement in glomerular capillary walls resulting in increased permeablility to plasma proteins. manifestations: massive proteinuria, loss of 3.5 g or more, hypoalbuminemia, generalized edema, hyperlipidemia and lipiduria

Question 59

highly selective proteinuria

Answer 59

low weight proteins, like albumin

Question 60

membranous nephropathy

Answer 60

diffusie thickening of the glomerular capillary wall due to the accumulation of deposits containing ig along the subepithelial side of the basement membrane

Question 61

what are some causes of membranous nephropathy

Answer 61

drugs nsaids, malignat tumors, lung colon carcinoma, sle, infections, hla dq1

Question 62

what is the morphology of membranous nephropathy

Answer 62

uniform, diffuse thickening of the glomerular capillary wall without increase in cells. irregular spikes seen with silver stain,

Question 63

mcg

Answer 63

minimal change disease. benign, uniform and diffuse effacement of foot processes, detectable only by electron microscopy. most frequent cause of nephortic syndrome in children. between 2 to 6. increased incidence with atopic disorders, hodgkins lymphoma. fusion of foot processes. normal glomeruli + effaced foot processes =mcg. lipoid nephrosis. highly selective massive proteinuria, mostly albumin. most patients respond to corticosteroids

Question 64

fsgs

Answer 64

most common cause of nephrotic syndrome in adults in the US. hypertension, microscopic hematuria, azotemia

Question 65

what is the hallmark of fsgs

Answer 65

effacement of foot processes

Question 66

mpgn

Answer 66

pattern of immune mediated injury rather than a disease. type 1: deposition of immune complexes containing igg and complement. type 2: dense deposit disease. c3 glomerulopathy