102914 path of kidney

Question 1

systemic diseases associated with nephrotic syndrome

Answer 1

diabetic nephropathy
amyloidosis
light chain deposition disease

Question 2

hereditary glomerular diseases

Answer 2

Alport syndrome

thin basement membrane disease

Question 3

hyperfiltration in diabetes occurs when

Answer 3

early in diabetes

Question 4

how does hyperfiltration in diabetes occur?

Answer 4

gluocse causes afferent arteriole dilation
angiotensin II causes efferent arteriole constriction

both lead to increased GFR and glomerular loss of proteins

Question 5

pathogenesis of diabetic nephropathy

Answer 5

hyperfiltration

hypertrophy

widening of GBM and podocyte changes leading to proteinuria

fibrosis

Question 6

hypertrophy in diabetic nephropathy

Answer 6

seen early
increase in number of mesangial cells and capillary loops, increasing filtration SA

size of kidney may increase

Question 7

hallmarks of diabetic nephropathy

Answer 7

mesangial expansion mediated by glucose and glucose derived AGEs

nodular diabetic glomerulosclerosis (acellular Kimmelstiel Wilson lesion)

Question 8

describe widening of GBM in diabetic nephropathy

Answer 8

accumulation of type IV collagen

net reduction in negatively charged heparin sulfate

Question 9

tubulointerstitial fibrosis is seen when in diabetic nephropathy?

Answer 9

later

Question 10

tubulointerstitial fibrosis

Answer 10

tubular cells change phenotype and become fibroblasts

Question 11

stages of diabetic nephropathy

Answer 11

pre-DN: increase in GFR, renal hypertrophy

stage 3: mesangial expansion, GBM thickens. HTN. GFR not as high as before. start seeing protein in urine.

stage 4: overt DN. mesangial nodules and tubulointerstitial fibrosis. proteinuria, nephrotic syndrome, decreased GFR.

Question 12

treatment for diabetic nephropathy

Answer 12

HTN therapy
glucose control
reduce proteinuria (with renin agiotensin aldosterone system blockade to decrease constric of efferent arteriole)
lipid lowering therapy
lifestyle change

Question 13

amyloidosis

Answer 13

deposition in extracellular spaces of proteinaceous material

Question 14

amyloidosis affecting the kidney can be described by

Answer 14

light chains secreted by single clone of B cells

Question 15

manifestations in amyloidosis affecting the kidney

Answer 15

often enlarged kidney and HTN is absent even when renal fxn is impaired

proteinuria in absence of microscopic hematuria

tubular defects from amyloid deposits (renal tubular acidosis, polyuria polydipsia)

Question 16

light microscopy of amyloidosis

Answer 16

light pink PAS as opposed to diabetes

Question 17

what is another stain that can be done for amyloidosis

Answer 17

Congo red stain-apple green birefringence

Question 18

light chain deposition disease

Answer 18

deposition of excess immunoglobulin light chains in kidney

usually kappa light chains
50% coexist with multiple myeloma

Question 19

light microscopy for light chain deposition disease

Answer 19

see nodular glomerulosclerosis, like for diabetes and amyloidosis

wouldn’t be able to tell difference btwn it and amyloidosis unless tested positive for congo red for amyloidosis

Question 20

extra renal manifestations of amyloidosis

Answer 20

AL amyloidosis can affect almost any organ other than brain

Question 21

what you see hematuria independent of proteinuria, think about

Answer 21

changes in glomerular membrane

Question 22

dysmorphic RBC in urine indicates

Answer 22

RBC had to have crossed the glomerular membrane

Question 23

Alport syndrome

Answer 23

usually X linked recessive

mutation of COL4A5 gene which encodes alpha5 chain of type IV collagen (causes defect in basement membrane)

Question 24

renal manifestations of Alport syndrome

Answer 24

hematuria
proteinuria (absent early but develops eventually)
HTN
ESRD in all affected males with XLAS

Question 25

extrarenal manifestations of Alport syndrome

Answer 25

cochlear defects
ocular defects
leiomyomatosis (less common)

Question 26

pathology of Alport syndrome

Answer 26

early in disease, glomeruli may be normal
later-global and segmental glomerulosclerosis, interstitial fibrosis

EM: variable thickening, thinning, basket weave, and lamellation of GBM

Question 27

thin basement membrane disease (benign familial hematuria)

Answer 27

usually autosomal dominant

continuous/intermittent microhematuria, w or w/o gross hematuria. generally no renal insufficiency

extra renal symptoms are rare
small risk of progression to ESRD

Question 28

pathology of thin basement membrane disease

Answer 28

LM: normal glomeruli
IF: negative
EM: thin GBM