17 - Renal Pathology III Flashcards Preview

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Flashcards in 17 - Renal Pathology III Deck (53):
1

What diseases cause asymptomatic isolated hematuria?

- IgA nephropathy(Berger Disease)
- Alport syndrome
- Thin GBM disease

2

Describe IgA nephropathy

- Most common type of primary glomerulonephritis worldwide
- Affects children and young adults
- Quite common in young adults, but NOT in African-Americans
- Causes recurrent hematuria

3

What will you see in a urinalysis of IgA nephropathy?

Mild hematuria
Mild proteinuria

4

What will you see in an LM, IF and EM of IgA nephropathy?

NEED TO KNOW ****

- LM: Mesangial proliferation
- IF: Mesangial IgA
- EM: Mesangial deposits

***

5

Describe mesengial proliferation

Mesangial hypercellularity of more than 3 cells per mesangial region

6

Describe alport syndrome

- Alport syndrome is a defect of the glomerular basement membrane due to mutation in the gene encoding for the alpha-5 chain of collagen type IV

7

What are the clinical manifestations of alport syndrome?

- Sensorineural deafness
- X-linked dominant inheritance
- Affects males, females are carriers
- Presents with microscopic hematuria
- Progresses to renal failure
- Proteinuria is a sign of progression

8

What are the key lesions of alport syndrome?

*******

- GBM thickening, splitting and lamination
- "Basket-weave" pattern

***KNOW BASKET WEAVE***

AB --> Alport = Basket

9

Describe thin GBM disease

- Benign familial (hereditary) hematuria
- About HALF of the GBM is as uniformly thin as it should be

10

What will you see on the LM, IF and EM or thin GBM disease?

LM = normal
IF = negative
EM = thin GBM

11

What are the secondary causes of glomerulonephropathies?

- SLE (lupus)
- Diabetes
- Amyloidosis

12

Describe lupus nephritis (SLE)

- Kidney is a major target of disease
- Granular immune complexes deposit in the glomerulus

13

What are the types of immune complexes that deposit in lupus nephritis?

*********

Granular immune complex deposition of IgG, IgA, IgM, C3, C4

This is the ONLY glomerular disease with ALL immunoglobin deposition *****

14

What does diabetes cause in the kindey?

Nodular glomerulosclerosis

AKA diabetic nephropathy

15

How does diabetic nephropathy present?

Can present as proteinuria, nephrotic syndrome or chronic renal failure

16

Describe amyloidosis with kindney involvement

Amyloid gets "trapped" in:
- glomeruli
- blood vessels
- tubules

17

What does amyloid deposition cause?

***

- Usually the glomerular basement membrane becomes too "leaky" to proteins and the patient gets "nephrotic syndrome"
- Kidneys appear as a big pale "waxy" kidney

NEPHROTIC ***

18

What will amyloidosis appear like under polarized light?

NEED TO KNOW *****

"Apple green birefringence"

Know this word by word ***

This apple green birefrigence appearance will be visible when stained with CONGO RED and seen under polarized light *****

19

Describe acute tubular necrosis (ANT)?

- Destruction of renal TUBULAR epithelium
- Loss of renal function
- 50% of ACUTE renal failure

This is NOT in the glomerulus, in the TUBULAR epithelium ***

20

What are the two types of acute tubular necrosis?

- Ischemia
- Nephrotoxic

Either due to lack of blood flow or toxins

21

What types of nephrotoxic substances cause acute tubular necrosis (ANT)?

- Drugs (aminoglycosides, amphotericin B, gentamycin)
- Heavy metals (mercury)
- Organic solvents (carbon tetrachloride from dry cleaners)
- Radiocontrast dyes (contrast)
- Myoglobin (rhabdomyolysis in heat stroke)

22

What are the pathologic changes seen in acute tubular necrosis (ANT)?

- Dilated tubules with flattened epithelium
- Necrotic debris in tubules

23

Describe acute interstitial nephritis

A drug induced hypersensitivity reaction

24

What can cause acute interstitial nephritis?

- Synthetic penicillins
- Rifampin
- Ibuprofen
- Thiazide diuretics

25

What happens 2 weeks after taking the drug?

***

- Fever
- Eosinophilia***
- Rash
- Acute renal failure (oligouria + increased Cr)

***

26

What are the different types of pyelonephritis?

- Ascending = MOST COMMON*** (reflux or obstruction)
- Hematogenous is also possible (from blood)

Ascending means urethra --> bladder --> ureters --> kidney --> pelvis

27

What is the most common pathogen of pyelonephritis?

E coli ***

This is only for when it comes from ascending, not blood

28

What will you see in an acute case pylonephritis?

Neutrophils

29

What will you see in chronic pyelonephritis?

Lymphocytes and scars (fibrosis)

30

What is the most common pathogen of pyelonephritis from the blood?

Staph

Hematogenous is also called "descending"

Can cause the formation of an abscess on the kidney (more commonly in hematogenous than ascending)

31

Describe acute pyelonephritis

- Caused by infections of the pelvicalyceal system and renal parenchyma
- May present with pain in the costo-vertebral angle plus other signs of infecitons
- Diagnosis - pus (WBC) casts, urine culture

MUST have costo-vertebral angle pain and fever

32

What are the key lesions in acute pyelonephritis?

***

Neutrophilic infiltrates and neutrophil casts in tubules are characteristic

***

33

Describe chronic pyelonephritis

- May have insidious onset or a history of recurrent acute pyelonephritis
- Gradual loss of renal function
- Results in scarred kidney with chronic inflammation

34

What can cause chronic pyelonephritis?

- Obstruction (congenital or acquired)
- Instrumentation (catheter)
- Vesicoureteral reflux
- Pregnancy
- More common in females
- Previous lesions
- Immunosuppression or immunodeficiency

35

What is the hallmark of chronic pyelonephritis grossly?

********

Pitting geographic "scars"

36

What is the microscopic hallmark of chronic pyelonephritis

**** Commonly Tested ****

- Scarred renal cortex with destruction of architecture
- The atrophic tubules look like the thyroid gland architecture, so it is sometimes called "thyroidization"
- Fibrosis occurs due to chronic inflammatory infiltrate

***KNOW THYROIDIZATION***

37

What are the KEY lesions of chronic pyelonephritis?


***

- Scarred kidneys, chronic inflammation
- Severe tubular atrophy, thyroidization of the kidney

38

What are the types of benign renal tumors?

- Adenoma (papillary)
- Fibroma
- Angiomyolipoma
- Fibroma

39

What are the types of malignant renal tumors?

Renal Cell Carcinoma
- Clear cell
- Papillary
- Chromophobe

Urothelial Carcinoma

Wilms

Medullary Carcinoma

40

Describe a papillary adenoma

- Always within the cortex
- AKA “cortical adenoma”
- Small
- Usually

41

Describe an angiomyolipoma

- Association with tuberous sclerosis
- Mental retardation
- Multisystem hamartomas
- Consists of smooth muscle (myo), fat (lipo) and thick walled vessels (angio)

42

What are the risk factors for renal cell carcinoma?

- Tobacco***
- Chronic renal failure and acquired cystic renal disease ***
- Obesity
- HTN
- Unopposed estrogen therapy
- Asbestos
- Heavy metals

43

What are the symptoms of renal cell carcinoma?

- Hematuria (50-60%) ***
- Abdominal mass (25-40%) ***
- Flank pain (35-40%)
- Hypertension (20-40%)
- Weight loss (30-35%)
- Fever (5-15%)

44

What does renal cell carcinoma strongly tend to invade?

***KNOW THIS***

STRONGLY tend to invade the renal VEIN early

***KNOW THIS***

Highly emphasized

45

What are the three histotypes of renal cell carcinoma?

- Clear cell
- Papillary
- Chromophobe RRC

46

Describe the clear cell histotype of renal cell carcinoma

Clear cell RCC
- 80%
- Loss of tumor suppressor gene VHL
- Gross appearance is yellow/orange

47

Describe the papillary (chromophil) histotype of renal cell carcinoma

Papillary (chromophil) RCC
- 15%
- Activation of pro-oncongene gene MET

48

Describe the chromophobe histotype of renal cell carcinoma

Chromophobe RCC
- 5%
- Loss of multiple chromosomes resulting in hypodiploidy
- Prognosis is the best among 3 histotypes

49

Describe the microscopic image of papillary (chromophil) RRC

Papillary structures many of which enclose clusters of foamy macrophages


Tumor cells have abundant eosinophilic cytoplasm and mildly atypical nuclei

50

Describe the difference between clear cell and papillary cell RRC microscopically

Clear cell
- abundant clear cytoplasm
- abundant capillaries

Papillary
- eosinophilic (pink) cytoplasm
- sparse capillaries

51

Describe the renal morbidities of dialysis patients

- Approximately 35% of patients on chronic dialysis develop acquired cystic disease in their native kidneys.
- Renal cell carcinoma develops in approximately 6% of these patients.

52

Describe the associated syndrome of VHL

Von Hippel-Lindau syndrome (VHL)
- Cavernous hemangiomas in cerebellum, brain stem & eye
- These patients have a remarkably high incidence of RCC
- Associated with germline mutations of the VHL tumor suppressor gene on chromosome 3

53

Describe the associated syndrome of urothelial (transitional cell) carcinoma of the renal pelvis

- In renal pelvis
- 1/10 as common as renal cell carcinomas
- EXACTLY the same appearance as lower urinary tract carcinomas
- ***MUCH more likely to obstruct the kidney than renal cell carcinomas
- Associated with ureter and bladder carcinomas

You will see a mass that occupies almost all of the pelvis