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AU2017 Gen Path Exam 2 > Lecture 8- Renal Disease 1 > Flashcards

Lecture 8- Renal Disease 1 Flashcards

1
Q

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

A

Azotemia

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2
Q

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

A

Uremia

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3
Q

3 major clinical renal syndromes

A

acute nephritic syndrome

nephrotic syndrome

acute renal failure

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4
Q

Most significant symptom of acute nephritic syndrome

A

hematuria

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5
Q

Most significant symptom of nephrotic syndrome

A

severe proteinuria

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6
Q

Most significant symptom of acute renal failure

A

oliguria/anuria

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7
Q

2 congenital cystic renal diseases

A

autosomal dominant (adult) polycystic kidney disease

autosomal recessive (childhood) polycystic kidney disease

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8
Q

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

A

500-1000

multiple expanding cysts in both kidneys

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9
Q

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

A

PKD1 located on chromosome 16

polycystin-1

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10
Q

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

A

1/3 of patients have cysts in liver

saccular “berry” anueryms in the circle of willis

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11
Q

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

A

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

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12
Q

Clinical presentation of AD adult polycystic kidney disease

A

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

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13
Q

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

A

20,000

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14
Q

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

A

PKHD1

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15
Q

Defective protein on AR childhood polycystic kidney disease?

A

fibrocystin

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16
Q

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

A

shortly after birth to several years

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17
Q

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

A

uniform size cysts

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18
Q

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

A

liver cysts and progressive liver fibrosis

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19
Q

what is the most obvious clinical sign of nephrotic syndrome

A

severe edema (because of the heavy proteinuria)

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20
Q

4 different types of nephrotic syndrome

A

minimal change disease

focal segmental glomerulosclerosis

membranous neuropathy

nodular glomerulosclerosis (DM)

21
Q

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

A

minimal change disease

22
Q

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

A

epithelial foot process effacement

23
Q

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

A

nothing- normal appearing glomeruli

24
Q

What can you see in immunofluroesence in minimal change disease?

A

nothing- no immune complexes

25
Q

Treatment for minimal change disease?

A

corticosteroid - good response

26
Q

Common cause of nephrotic syndrome in adults?

A

focal and segmental glomerulosclerosis

27
Q

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

A

can be any of the 3

28
Q

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

A

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

29
Q

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

A

no immune complex deposits in idiopathic form

30
Q

What is the response to treatment in focal segmental glomerulosclerosis?

A

poor response to corticosteroid treatment

31
Q

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

A

50%

32
Q

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

A

membranous neuropathy

33
Q

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

A

idney

infection, malignancy, SLE, or drugs

34
Q

Pathology of membranous neuropathy:

LM:
IF:
EM:

A

LM: nearly normal

IF: immune complex deposits

EM: depostis in subepithelial side of the GBM

35
Q

WHat is the response to treatment in membranous neuropathy?

A

poor response to corticosteroid tx

36
Q

Pathology of glomerular disease in diabetes mellitus:

LM:
IF:
EM:

A

LM: nodular glomerulosclerosis (know this)

IF: no immune complex deposits

EM: thick GBM

37
Q

3 other changes you see in glomerular disease in diabetics

A

hyaline arteriolosclerosis
atherosclerosis
nephrosclerosis

38
Q

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

A

2nd

39
Q

what disease do you see a kimmelstiel-wilson lesion?

A

glomerulo disease in diabetes

40
Q

Nephritic syndrome is charactarized by

A
  1. Hematuria (know this)
  2. oliguria and azotemia
  3. HTN
41
Q

2 major forms of nephritic syndrome

A

acute postinfectious (poststreptococcal) glomerulonephritis

IgA neuropathy

42
Q

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

A

children

streptococcal pharyngitis

43
Q

Pathology of acute postinfect glom.nephr.

LM:
IF:
EM:

A

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

44
Q

… is one of the most common causes of renal disease

A

IgA neuropathy

45
Q

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

A

IgA neuropathy

46
Q

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

A

Henoch-Schonlein pupura

47
Q

Pathology of IgA neuropathy

LM:

IF and EM:

A

LM: variable mesangial proliferation

IF and EM: immune (IgA) complexes within the mesangium

48
Q

What is the characteristic finding of rapidly progressive glomerulonephritis?

A

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

49
Q

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

A

crescentic or rapidly progressing glomerulonephritis

AU2017 Gen Path Exam 2 (9 decks)

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