Lecture 8- Renal Disease 1

Question 1

… is defined as elevated BUN and creatinine levels, due to decreased GFR

Answer 1

Azotemia

Question 2

… is defined as azotemia (elevated BUN and creatinine) plus other symptoms (gastroenteritis, peripheral neuropathy, dermatitis, acidosis, pericarditis, hyperkalemia)

Answer 2

Uremia

Question 3

3 major clinical renal syndromes

Answer 3

acute nephritic syndrome

nephrotic syndrome

acute renal failure

Question 4

Most significant symptom of acute nephritic syndrome

Answer 4

hematuria

Question 5

Most significant symptom of nephrotic syndrome

Answer 5

severe proteinuria

Question 6

Most significant symptom of acute renal failure

Answer 6

oliguria/anuria

Question 7

2 congenital cystic renal diseases

Answer 7

autosomal dominant (adult) polycystic kidney disease

autosomal recessive (childhood) polycystic kidney disease

Question 8

AD adult polycystic kidney disease is seen in 1 out of every … people and is characterized by…

Answer 8

500-1000

multiple expanding cysts in both kidneys

Question 9

What is the deective gene in AD adult polycystic kidney disease? what does this gene encode for?

Answer 9

PKD1 located on chromosome 16

polycystin-1

Question 10

what are the extrarenal symptoms in AD adult polycystic kidney disease?

Answer 10

1/3 of patients have cysts in liver

saccular “berry” anueryms in the circle of willis

Question 11

How are the kidneys described in AD adult polycystic kidney disease?

Answer 11

very large (up to 4 kg) with numerous cytss that arise in every part of the tubular system

Question 12

Clinical presentation of AD adult polycystic kidney disease

Answer 12

flank pain around 4th decade, hematuria, HTN, uti, renal failure

Question 13

AR childhood polycystic kidney disease happens in 1 out of … live births

Answer 13

20,000

Question 14

AR childhood polycytic kidney disease is due to mutations in … gene

Answer 14

PKHD1

Question 15

Defective protein on AR childhood polycystic kidney disease?

Answer 15

fibrocystin

Question 16

when does renal failure occur in AR p.c. kidney disease

Answer 16

shortly after birth to several years

Question 17

AR p.c. kidney disease have numerous … that arise from the collecting tubules

Answer 17

uniform size cysts

Question 18

What is the extrarenal pathology of AR p.c. kidney disease?

Answer 18

liver cysts and progressive liver fibrosis

Question 19

what is the most obvious clinical sign of nephrotic syndrome

Answer 19

severe edema (because of the heavy proteinuria)

Question 20

4 different types of nephrotic syndrome

Answer 20

minimal change disease

focal segmental glomerulosclerosis

membranous neuropathy

nodular glomerulosclerosis (DM)

Question 21

Which nephrotic syndrome is most common in children (2/3 of all cases)

Answer 21

minimal change disease

Question 22

What can you see in electron microscopy of minimal change disease?

Answer 22

epithelial foot process effacement

Question 23

What can you see in light microscopy of minimal change disease?

Answer 23

nothing- normal appearing glomeruli

Question 24

What can you see in immunofluroesence in minimal change disease?

Answer 24

nothing- no immune complexes

Question 25

Treatment for minimal change disease?

Answer 25

corticosteroid - good response

Question 26

Common cause of nephrotic syndrome in adults?

Answer 26

focal and segmental glomerulosclerosis

Question 27

is focal segmental glomerulosclerosis primary (idiopathic), secondary, or genetic ?

Answer 27

can be any of the 3

Question 28

What can you see in light microscopy of focal segmental glomerulosclerosis?

Answer 28

focal (some gloreuli) and segmental (part of involved glomerulus) sclerosis with obliteration of capillary loops

Question 29

What can you see in EM and IF of focal segmental glomerulosclerosis?

Answer 29

no immune complex deposits in idiopathic form

Question 30

What is the response to treatment in focal segmental glomerulosclerosis?

Answer 30

poor response to corticosteroid treatment

Question 31

What percent of patients progress to renal failure after 10 years with focal segmental glomerulosclerosis?

Answer 31

50%

Question 32

Which kind of nephrotic syndrome if most commone in adults age 30-50 but may be seen in children?

Answer 32

membranous neuropathy

Question 33

Membranous neuropathy may be primary (disease limited to the ....) or secondary to ...,...,... or ...

Answer 33

idney infection, malignancy, SLE, or drugs

Question 34

Pathology of membranous neuropathy: LM: IF: EM:

Answer 34

LM: nearly normal IF: immune complex deposits EM: depostis in subepithelial side of the GBM

Question 35

WHat is the response to treatment in membranous neuropathy?

Answer 35

poor response to corticosteroid tx

Question 36

Pathology of glomerular disease in diabetes mellitus: LM: IF: EM:

Answer 36

LM: nodular glomerulosclerosis (know this) IF: no immune complex deposits EM: thick GBM

Question 37

3 other changes you see in glomerular disease in diabetics

Answer 37

hyaline arteriolosclerosis atherosclerosis nephrosclerosis

Question 38

renal failure is ... to MI as cause of death in diabetics

Answer 38

2nd

Question 39

what disease do you see a kimmelstiel-wilson lesion?

Answer 39

glomerulo disease in diabetes

Question 40

Nephritic syndrome is charactarized by

Answer 40

1. Hematuria (know this) 2. oliguria and azotemia 3. HTN

Question 41

2 major forms of nephritic syndrome

Answer 41

acute postinfectious (poststreptococcal) glomerulonephritis IgA neuropathy

Question 42

acute postinfectious glomerulonephritis is most common in ... about 1-4 weeks after a bout of ....

Answer 42

children streptococcal pharyngitis

Question 43

Pathology of acute postinfect glom.nephr. LM: IF: EM:

Answer 43

LM: proliferation of endothelial and mesangial cells, inflamm cells; may develop into cellular crests IF: immune complex deposition, granular EM: immune complexes in GBM + or - mesangium

Question 44

... is one of the most common causes of renal disease

Answer 44

IgA neuropathy

Question 45

... affects children and young adults, heamaturia is noted 1-2 days post upper respiratory tract infection; resolves then recurs

Answer 45

IgA neuropathy

Question 46

When renal disease is associated with pupuric skin rash, GI pain, arthritis then its called

Answer 46

Henoch-Schonlein pupura

Question 47

Pathology of IgA neuropathy LM: IF and EM:

Answer 47

LM: variable mesangial proliferation IF and EM: immune (IgA) complexes within the mesangium

Question 48

What is the characteristic finding of rapidly progressive glomerulonephritis?

Answer 48

crescentic glomerulonephritis due to proliferation of epithelial cells with infiltration of histiocytes

Question 49

... is an acute clinical syndrome but not a specific form of glomerulonephritis

Answer 49

crescentic or rapidly progressing glomerulonephritis