Renal Pathology- Warren Flashcards Preview

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Flashcards in Renal Pathology- Warren Deck (147):
1

What nephrotoxic cause of ATN will you see oxalate crystals?

Ethylene Glycol

2

What are 2 types of ATN?

Ischemic
Nephrotoxic

3

What are causes of nephrotoxic ATN?

Crush injury (myoglobin)
Urate (from tumor lysis syndrome)
Aminoglycosides
Heavy metals
ethylene glycol
Radiocontrast dye

4

Normal BUN: Cr ratio?

15:1

5

What kind of casts do you see in ATN?

Brown, granular casts

6

What kind of nephritis do NSAIDs, penicillin, and diuretics cause?

Acute Interstitial Nephritis (connective tissue between tubules)

7

What is found in the urine in Acute Interstitial Nephritis?

Eosinophils! High yield

8

Name one pharmacological cause of renal papillary necrosis.

Chronic analgesic abuse

9

What major protein is lost in nephrotic syndrome?

Albumin

10

Why do patients with nephrotic syndrome develop a hypercoaguable state?

They lose Antithrombin III

11

Why do patients with nephrotic syndrome get hyperlipidemia and hypercholesteremia?

Liver reacts by throwing excess fat into the blood because the blood gets "thin"

12

What is the most common cause of nephrotic syndrome in kids?

Minimal change disease

13

What can Minimal change disease be associated with?

Hodgkin Lymphoma (massive overproduction of cytokines from Reed Sternberg cells)

14

What do you lose in MCD?
Why?

Foot processes flatten/efface because of cytokine production

15

What histology hallmark is present in MCD?

NORMAL Glomeruli on H&E stain
EFFACEMENT of foot processes on EM

16

What is the only protein lost in MCD?

Albumin
(don't lose immunoglobulin)

17

What holds together lobules of the glomerulus?

Mesangial cells!

18

What is the most common cause of Nephrotic syndrome in Hispanics and African Americans?

FSGS: Focal Segmental Glomerular Sclerosis

19

What can FSGS be associated with?

Heroin Use
HIV
Sicke Cell Disease
(or Idiopathic)

20

What does "segmental" mean in FSGS?

Only a single part of the glomerulus will be involved

21

What does "focal" mean in FSGS?

Only some of the glomeruli in the kidney will be involved

22

FSGS is the progression of what disease if it doesn't respond to steroids?

Minimal Change Disease

23

Does FSGS respond to steroids?

No...progreses to Chronic renal failure

24

Most common cause of nephrotic syndrome in caucasian adults?

Membranous Nephropathy

25

Causes of Membranous Nephropathy?

Usually Idiopathic
Hepatitis B/C
SLE-->most common cause of death is renal failure!
Solid Tumors
Drugs

26

Usually patients with SLE don't present with nephrotic syndrome, but if they do, what is it?

Membranous Nephropathy

27

What is happening in Membranous Nephropathy histologically?

Glomerular basement membrane becomes really thick!

28

Why does the membrane become thick in Membranous Nephropathy?

Immune complex deposition

29

Where is the immune complex deposition in Membranous Nephropathy?

Right under the podocytes (epithelial cells)

30

How do podocytes respond to immune complex deposition?

Try to lay down more basement membrane!

31

What is the EM description of membranous nephropathy?

Spike and dome subepithelial deposits

32

What do you see in Membranoproliferative Glomerulonephritis?

Thick capillary membranes on H and E with "tram track appearance" due to Immune complex deposition

33

What are the 2 types of Membranoproliferative glomerulonephritis based upon?

Location of deposits

34

What are the 2 types of Membranoproliferative glomerulonephritis?

I: subendothelial
II: Intramembranous

35

Why are there neutrophils in nephritic syndrome?

Inflammation is driven by immune complexes which activate complement. A byproduct of this is C5a--these attract neutrophils which mediate damage.

36

What are RBC casts a sign of?

glomerular bleeding

37

What virulence factor of some Group A Beta hemolytic streps increase the changes of PSGN?

M protein! This defines nephritogenic strains

38

When does PSGN usually occur?

2-3 weeks post infection

39

Is PSGN nephritic or nephrotic?

Nephritic

40

What do you see on EM in PSGN?

subepithelial humps

41

What is RPGN histology?

Crescents! Made of Fibrin and macrophages --Inflammatory Debris

42

What disease have positive p-ANCA?

Microscopic polyangiitis
Churg Strauss

43

Most common nephropathy worldwide?

IgA nephropathy

44

Where do IgA complexes like to deposit?

Mesangium

45

What is Alport Syndrome?

Inherited Defect in Type IV collagen that results in thinning and splitting of GBM

46

What is the genetics of Alport Syndrome?

X-linked

47

What does Alport syndrome present as?

Isolated hematuria
Sensory hearing loss
Ocular disturbances

48

How is polycystic kidney disease inherited?

Infant from - autosomal recessive

Adult form - autosomal dominant

49

What are two things associated with the autosomal recessive form of polycystic kidney disease?

Congenital hepatic fibrosis
Hepatic cysts

50

What three things are associated with the autosomal DOMINANT form of polycystic kidney disease? (adult form)

BERRY ANEURYSM
hepatic cysts
mitral valve prolapse

51

What genes are mutated in adult PKD? what do they code for?

**autosomal dominant
APKD1 = encodes polycystin 1 = integral membrane protein

APKD2 = encodes polycystin 2 = Ca+ channel

52

What is medullary cystic kidney disease? (aka medullary sponge kidney)

Autosomal dominant
Cysts in the MEDULLARY COLLECTING DUCTS

53

What is a gross feature that would distinguish PKD from medullary cystic kidney disease?

In PKD kidneys appear enlarged

In medullary the kidneys appear shrunken

54

What are the hallmarks of acute renal failure?

Severe decrease in renal function with in days
AZOTEMIA (increased BUN and creatinine)
Oliguira

55

How do you distinguish pre-renal azotemia and intrarenal azotemia?

Pre-renal will have BUN:Cr>15 (more BUN is getting reabsorbed)

Intra-renal will have BUN:Cr

56

What will you see in acute tubular necrosis?

BROWN granular casts
BUN:Cr 2%
Inability to concentrate urine (urine osm

57

What cancer can minimal change disease be associated with?

Hodgkin Lymphoma

58

Normal glomeruli
Effacement of foot processes on EM
Selective loss of Albumin
Associated w/ Hodgkin's lymphoma

Minimal Change Disease

59

Large hypercellular glomeruli
Sub epithelial "humps"
Smokey Urine
Periorbital edema
Normal in children

Poststreptococcal glomerulonephritis

60

Describe Type 1 RPGN

LINEAR
Anti-bodies to the basement membrane show a LINE on IF

Good pasture syndrome

Presents with hematuria, hemoptysis, in young adult males

61

Describe Type 2 RPGN

GRANULAR
Immune complex depsoition

Usually PSGN or diffuse proliferative glomerulnephritis

62

Describe Type 3 RPGN

Negative IF (pauci-immune)
p-ANCA

Wegener granulomatosis, microscopic polyangitiis, Churg-Strauss syndrome

63

What is the antigen in Anti-GBM glomerulonephritis?

NC1 - a component of the noncollagenous domain of Type IV collagen

64

What is the antigen in Membranous glomerulonephritis?

M-type phospholipase A2 receptor

65

What is a "planted antigen"?

Antigen is non-glomerular in origin, but is localized in the kidney

66

What do you see histologically in Acute Poststreptococcal GLomerulonephritis?

Hypercellular glomeruli!

67

What does the EM of Acute Poststreptococcal Glomerulonephritis look like?

Subepithelial humps like a camel

68

Is Acute poststreptococcal glomerulonephritis nephritic or nephrotic?

Nephritic

69

How would you describe the urine in Poststreptococcal Glomerulonephritis?

Smoky urine

70

When you look at a child with poststreptococcal glomerulonephritis, what is one striking thing you can see instantly?

Periorbital edema

71

What do you see histologically in Rapidly progressive GN?

Crescents!

72

How many of the glomeruli have crescents in Rapidly progressive GN?

>50%

73

Describe the immunofluorescence of Type I RPGN.

Linear deposits (anti-GBM)

74

Describe the Immunofluorescence of Type II RPGN.

Lumpy-bumpy pattern (immune complex mediated)

75

What is present in Type III RPGN?

P or C-ANCA

76

Is RPGN nephritic or nephrotic?

Nephritic!

77

What is the most common nephrotic syndrome in kids?

Minimal Change Disease

78

In what disease do you see uniform, diffuse thickening of capillary walls and on silver stain you see "spikes"?

Membranous Glomerulonephropathy

79

on IF, what are the granular deposits along the GBM in Membranous Glomerulonephropathy composed of?

IgG and C3

80

What disease has an increased incidence with Hodgkin's Lymphoma?

Minimal Change Disease

81

What can you use to tx Minimal Change Disease?

Corticosteroids!

82

What do you see on EM in Minimal Change Disease?

Diffuse effacement of foot processes of the visceral epithelial cells (podocytes)

83

FSGS results from genetic abnormalities to what?

Nephrin and Podocin proteins

84

What type of FSGS has the worst prognosis?

HIV nephropathy

85

What pathological condition is a mix between nephrotic and nephritic syndromes?

Membranoproliferative GN

86

What appearance of Membranoproliferative GN do you see on silver stain?

"Tram-track" or double contour due to mesangial cell interposition into the GBM!

87

What is the pathogenesis of Type I MPGN?

Immune complex deposition with activation of classical and alternative complement pathways

88

What do you see on EM in Type I MPGN?

Subendothelial deposits

89

What is another name for Type II MPGN?
Why?

Dense Deposit Disease
Lamina densa of the GBM is ribbon-like and extremely electron dense due to deposits of unknown material

90

When do patients usually get Berger Disease?

following an upper respiratory, GI, or urinary tract infection

91

What is the triad in Alport Syndrome?

Nephritis
Sensorineural hearing loss
Eye disorders

92

What is the genetics behind Alport Syndrome?

Autosomal Recessive
X-linked Dominant Inheritance

93

What is the mutation in Alport Syndrome?

Alpha 5 chain of Type 4 collagen

94

What GN is most likely to give you chronic GN?

Rapidly Progressive GN

95

How would you describe the IF staining for SLE?

"Full house" - stains with everything! So lots of immune components present

96

List the Nephritic syndromes we need to know!

"MIA DAR" Kinda dumb i know...but since there is an "i" we know they are nephritic

Membranoproliferative GN
IgA nephropathy
Acute Poststreptococcal GN
Diffuse Proliferative GN
Alport Syndrome
Rapidly Progressive GN

97

List the 3 main nephrotic syndromes we need to know!

Minimal Change Disease
FSGS
Membranous Nephropathy

98

What are the other 2 general nephrotic syndromes?

Amyloidosis
Diabetic Glomerulonephropathy

99

What is the diagnostic lesion on LM for Diabetic GN?

Kimmelstiel-Wilson Lesions

100

What do you see under LM for amyloidosis?
What stain?

Apple-green birefringence
Congo Red Stain

101

Most common cause of death in SLE patients?

Diffuse Proliferative Glomerulonephritis
(Lupus Nephritis)

102

Describe the pathogenesis of diabetic nephropathy

-high serum glucose leads to noneznymatic glycosylation of the vascular basement membranes

-Results in arteriolosclerosis, thickened GBM and increased mesangial matrix

103

When would you see Kimmelstiel Wilson nodules?

Diabetic Nephropathy

They are hyaline masses at the periphery of the glomerulus

104

What is the most common causes of acute renal failure? (and presents as acute loss of renal function)

Acute Tubular Necrosis

105

Describe the pathology of Acute Tubular Necrosis

-Tubular epithelial necrosis (due to ischemia or toxins)

-Sloughing of tubular cells into the lumen (obstruction leads to increased intratubular pressure and DECREASED GFR)

-Hyaline and granular casts
Interstitial edema and increased lymphocytes

-Toxic ATN may show specific changes (like calcium oxalate crystals in ethylene glycol ingestion)

106

How would you distinguish an acute from chronic nephritis?

Acute - many neutrophils and edema

Chronic- fibrosis and atrophy

107

What are you at increased risk for if you have vesicoureteral reflux?

pyleonephritis!!!

108

When are the only two times you will really see any damage to the calyces?

CHRONIC PYELONEPHRITIS
and anlagesic nephropathy

109

Name 5 risk factors for acute pyelonephritis?

1. indwelling urinary catheter
2. urinary tract obstruction
3. Vesicourecteral reflux
4. Diabetes mellitus
5. Pregnancy

110

When is onset of acute drug-induced interstitial nephritis after exposure to drug?

approx. 15 days

111

Acute Drug-induced interstitial nephritis is a mixture of what two types of hypersensitivities?

Type 1 (IgE mediated) and
Type 4 (delayed or T-cell mediated)

112

Why do you common see interstitial nephritis in multiple myeloma patients?

BENCE JONES proteinuria and cast nephropathy

Directly toxic to tubules!!!

Can combine with Tamm-Horsfall to cause obstructions

113

What is nephrosclerosis? Describe its pathogenesis.

The renal pathology associated with the sclerosis of renal arterioles and small arteries

-medial and intimal vessel thickening secondary to variety of factors
-Hyaline deposition in arterioles due to endothelial injury with extravasation of plasma proteins and increased basement membrane matrix

114

What is malignant nephrosclerosis?

Renal disease associated with malignant hypertension

*pathogenesis unclear

115

What would a "flea bitten" appearance on the gross pathology of the kidney indicate?

Malignant nephrosclerosis

116

What would you think if you saw and "onion-skin" appearance of a blood vessel?

hyperplastic arteriolosclerosis which involves thickening of vessel wall by hyperplasia of smooth muscle

It is a consequence of...
Malignant hypertension!

117

What are two possible causes of hypertension that is not responsive to HTN medications?

Renal Artery stenosis
Fibromuscular Dysplasia (young women)

118

Describe the pathogenesis of HUS/TTP

-endothelial cell injury
-Denuded endothelium reveals thrombogenic tissues
-Reduced prostaglanding I2 and NO
-Platelet activation and aggregation

119

What causes 75% of Typical/Classical HUS?

Verocytotoxin producing E. Coli 0157:H7

120

What four things could cause atypical HUS?

1. Antiphospholipid syndrome
2. Pregnancy
3. Vascular renal diseases
4. Drug related

121

What is deficient in familial HUS?

Inhertited deficiency of the complement regulatory protein FACTOR H

-Factor H typically protects cells from uncontrolled complement activation

122

What is the defect in idiopathic TTP?

Due to an acquired or genetic defect in the protesase that cleaves large von Willebrand multimers

ADAMTS-13

123

What is hydronephrosis? what is it usually caused by?

Distention/dilation of renal pelvis and calyces

Usually caused by urinary tract obstruction

Leads to compression and possible atrophy of renal cortex and medulla

124

Which gender is more likely to get urolithiasis? What is the peak ago of onset?

Men > women

20-30 years old

125

What are the four main types of stones?

Calcium
Ammonium magnesium phosphate (struvite)
Uric Acid
Cystine

126

What causes struvite (ammonium magnesium phosphate) stones?

Caused by infection with urease + bugs that hydrolyze urea to ammonia (resulting in alkalinization of urine)

Commonly form staghorn calcuil

127

Which 3 organisms are urease + that can cause struvite stones?

Proteus mirabilis
Staph Saprophyticus
Klebsiella

128

What is the most common type of stone?

Calcium (80%)

129

Which two types of stones appear radiopaque on x-ray?

calcium
Struvite (ammonium magnesium phosphate)

130

Which two types of stones appear radioucent on x-ray and precipitate at low pH?

Uric Acid
Cystine

131

Which stones have the coffin lid appearance?

Struvite (Ammonium magnesium phosphate)

132

Cystine stones are rare, what usually causes them?

Autosomal recessive condition in which cystine-reabsorbing PCT transporter loses function

Causes cystinuria

Cystine is poorly soluble- thus stones form in urine

common in kids

133

What is an angiomyolipoma?

Bengin renal tumor composed of vessels, smooth muslce, and fat

Associated with Tuberus Sclerosis

134

What is an oncocytoma?

Benign renal epithelial cell tumor

Presents with painless hematuria, flank pain, and abdominal mass

135

What color is an oncocytoma on gross pathology?

Mahogany Brown

136

What is most common primary renal malignancy?

Renal cell carcinoma

137

What is the most important risk factor for renal cell carcinoma?

Tobacco use (2x more incidence in smokers)

and Obestity

138

What is the classic triad for renal cell carcinoma?

Hematuria
Palpable mass
Flank pain

(patients rarely present with all 3)

May also see fever, weight loss, or paraneoplastic sydnrome

139

What gene is deleted in renal cell carcinoma?

loss of VHL gene on chromosome 3
(von Hippel-Lindau)

Acts as a tumor suppressor gene --> encodes a protein that is part of the ubiquitin ligase complex (which targets proteins for degradation)

Thus specific proteins that increase transcription and promote angiogenesis are not degraded

140

What is Von Hippel Lindau syndrome associated with?

Renal cell carcinoma

Autosomal dominant disorder
Associated with inactivation of the VHL gene leading to increased risk of renal cell carcinoma

141

What are the three subtypes of renal cell carcinoma?

Clear cell carcinoma (most common)
Papillary carcinoma
Chromophobe carcinoma

142

Where does renal cell carcinoma like to metastasizes to?

Lung or bone

"silent" cancer because commonly presents as a metastatic neoplasm!

143

Why do you sometimes see a left sided varicocele in renal cell carcinoma?

Invades the renal vein, and can block drainage of testicular vein

Then goes to the IVC and spreads hematogenously

144

Which renal neoplasm commonly causes paraneoplastic syndromes?

Renal cell carcinoma

145

What is the most common tumor of the urinary tract system?

Transitional cell carcinoma (urothelial carcinoma)

Usually arises in bladder

146

What is the number one risk factor for urothelial carcinoma?

Cigarette smoke!

147

Between HUS and TTP, one is more "renal" and one is more "neurological"...which ones?

TTP is more Neurological
HUS has more renal involvement