Renal Disease 1

Question 1

what parts of the kidney can be affected by renal disease

Answer 1

glomeruli, tubules, interstitium and vasculature

Question 2

how small does a particle have to be to pass through the glomeruli?

Answer 2

less than 70 Kd

Question 3

Azotemia

Answer 3

elevated BUN (blood urea nitrogen) and creatinine (breakdown of skeletal mm.)  due to decreased glomerular filtration rate (GFR)
-problem with glomerular filtration unit

Question 4

Uremia

Answer 4

azotemia plus clinical symptoms ( gastroenteritis, peripheral neuropathy, dermatitis, acidosis, pericarditis and hyperkalemia)

Question 5

Acute nephritic syndromw

Answer 5

HEMATURIA (blood in urine)

• acute onset, maybe protenuria or hypertension
• INFLAMMATION that destroys the endothelium that leads to bleeding and destroys the glomeruli so reduced GFR

Question 6

Nephrotic syndrome

Answer 6

severe PROTEINURIA (high urine albumin > 3.5 gms per day) which will decrease the oncotic pressure, leading to edema (anasarca even in other body parts)
-hypoalbumenima, hyperlipidemia and lipiduria (urine)
FROTHY PEE

Question 7

acute renal failure

Answer 7

Oliguria/anuria with sudden onset of azotemia – small amounts of urine

Question 8

autosomal dominant (adult) polycystic kidney disease clinical presentation

Answer 8

1/500-1000 people
multiple expanding cysts in both kidneys, gradual onset of renal failure in adults, urinary tract hemorrhage (hematuria), pain, hypertension and urinary tract infection and flank pain around the 4th decade

Question 9

etiology of adult polycystic kidney disease

Answer 9

defective gene is PDK1 (in 90% of families) located on chromosome 16, gene encodes polycycstin-1

Question 10

AD polycystic extrarenal pathology

Answer 10

1/3 of patients have cysts in liver, “berry” aneurysms may develop in the circle of willis (intracranial) - 30%

Question 11

pathology of AD polycystic kid dis

Answer 11

very large kidneys with numerous cysts that arise in every part of the tubular system
~ will not develop the disease until around 20 years old, but need tx

Question 12

AR polycystic kidney disease clinical presentation

Answer 12

1/20,000 births (less common than AD)

• develops from infancy to several years of age is rare
• defective protein is PKHD1 - fibrocystin (not same at AD)

Question 13

AR Polcyc kid dis extrarenal pathology

Answer 13

almost all have liver cysts and progressive liver fibrosis

Question 14

AR polycys dis pathology

Answer 14

numerous small uniform size cysts from collecting tubules in the cortex and medulla
they are like sponges thats glomeruli are replaced with cysts

Question 15

mechanisms of glomerular disease

Answer 15

1. immune complex depost in the basement memnrace or mesangium (from circulating complexes, antibodies against the glomerulus or planted antigens)
2. epi and endothelial cell injury

Question 16

pathological evaluation of kidney biopsies

Answer 16

light microscopy is the standard but immunofluorecnse is used as well as electron microscopy
silver and PAS stain for basement membrane, trichrome for collagen)

Question 17

what does the appearance of each glomerular disease mechanism look like on electron microscopy

Answer 17

• circulating immune complexes are “dot” like around the glomeruli –> trapped and deposited in a non-linear fashion
• circulating antibodies directed against the glomerulus are smooth, linear Ab deposits –> ab directed against the basement membrane of glomerulus
• antibodies against non-glomerular antigens are a mix

Question 18

what are the 4 major types of nephrotic syndrome

Answer 18

minimal change, focal and segmental glomerulosclerosis. membrane nephropathy and nodular glomerulosclerosis

Question 19

Minimal change disease affects:

Answer 19

-children mainly(2/3 of casess)

Question 20

minimal change pathology

Answer 20

"minimal change.."
LM - normal
IF - no deposits
EM - foot process effacement (taken out)- kidney can't hold protein
** good response to corticosteroid tx

Question 21

focal segmental glomerulosclerosis affects:

Answer 21

mainly adults

• may be primary or secondary to other glomerular diseases, some are familial(genetic)
• affects some (focal) of the glomeruli and part (segmental) of the tuft

Question 22

pathology focal segmental glomerulosclerosis

Answer 22

LM - focal (some glomeruli) and segmental (part of involved glomerulus) sclerosis with obliteration of capillary loops
If and EM - no deposits in the idiopathic primary form
-poor response to corticosteroids (can control, but not stop the progression - 50% will have renal failure within 10 years) –> eventually all the glomeruli would be affected

Question 23

Diabetes Mellitus renal failure frequency

Answer 23

renal failure, 2nd to MI as a cause of death

Question 24

membranous nephopathy

Answer 24

most common in adults (30-50)

-primary disease or secondary to infection, malignancy, SLE (lupus) or drugs

Question 25

pathology memb nephropathy

Answer 25

LM: nearly normal (variable)
IF: deposits –> uniform electron microscopy and thickening of basement membrane
EM: deposits in subepi side of GBM
-poor to moderate response to corticosteroids (40% go to end stage renal failure in 2-20 years)

Question 26

diabetis glomerular pathology

Answer 26

LM - nodular glomeruloscerosis –> scarred down
IF - no complex deposits
EM - Thick GBM
other changes: hyaline ateriosclerosis, atherosclerosis and nephrosclerosis (scaring down the whole kidney, not just the glomeruli)

Question 27

Kimmelstiel - wilson lesion

Answer 27

Diabetes balls of pink material in the tuft –> daignostic

mesangial nodules that is replacing normal tissue

Question 28

2 major nephritic syndromes

Answer 28

acute postinfectious (poststreptococcal) and IgA nephropathy

Question 29

Acure postinfetious glomerulonephritis

Answer 29

-most common in children 1-4 wks after strep, but other infections can cause

Question 30

APG path

Answer 30

LM - prolif endothelial and mesangail cells, infiltration of neutrophils and monocytes - cellular cresents common
IF, EM - immune complexes in GBM and sometimes mesangium b/c cross-reactivity from infection
-progression to chronic renal disease is more common in adults
-spotty granular deposition from circulating deposits

Question 31

IgA nephropathy usually occurs in

Answer 31

children and young adults

Question 32

IgA nephropathy

Answer 32

dramatic hemoturia 1-2 days after upper resp tract infec

-will resolve and then come back

Question 33

Henoch-Schonlen

Answer 33

when pt. has IgA nephropathy (kidney disease) and clinical symptoms like skin rash, GI pain and arthritis

Question 34

pathology IgA nephropathy

Answer 34

LM- variable mesangel cell prolif
If and Em - immune (iGa) complexes within the mesangium - not as destructive becasue IgA doesnt complement well but destruciton from macrophages)

Question 35

crescentic or rapidly progressive glomerulonephritis

Answer 35

assoc with antibod directed against glomerular basement membrane antigen, deposition of immune complexes (50 % of cases) or lack of immune complex deposition (we call this pauci-immnue b/c we dont really know whats happening)

• clinical cluster of syndromes, not a specific form
• death in weeks to months is untreated
• little urine produced
• crescentic due to prolif epi cells with infiltration of histiocytes

Question 36

end stage chronic renal disease

Answer 36

due to untreated glomerular disease - loss of tubules and glomeruli leads to fibrosis
original renal disease cannot be found normally because by time diagnosed - so much scaring
- bell curve onset (middle age is sweet spot for developing)
-protenuria, hypertnsion and azotemia can help detect renal disease during routine exam
-corticosteroids slow down disease, but dialysis and transplant are needed for survival