Part 3 (Warren) - W4 Flashcards
1
Q
What are 2 hereditary diseases associated w/glomerular injury?
A
- alport syndrome
- thin membrane disease
2
Q
What do you see with alport syndrome?
Who does it commonly affect?
When does it present?
A
- nephritis
- nerve deafness
- eye disorders (lens, cataracts, corneal dystrophy)
- males more
- x-linked dominant inheritance
- 5 - 20 years
- renal failure by age 20-50
3
Q
What is seen on EM of Alport syndrome?
A
- irregular thick and thin GBM
- splitting of the lamina densa
- changes are widespread
4
Q
What is the pathogenesis of alport syndrome?
A
- defective GBM synthesis - x-linked form has mutation encoding alpha 5 chain of type IV collagen
5
Q
What does thin membrane disease present?
A
- presents w/hematuria
- EM: shows diffuse thinning of the GBM
- has an excellent prognosis
6
Q
What do you see with chronic glomerulonephritis?
A
- small diffuse granular kidneys
- Forms of rapidly progressive GN more likely to progress to this.
7
Q
How does systemic lupus erythematosus present?
What do you see on stains?
A
- failure of self tolerance of immune system
- affects skin, joints, kidney, serosal membranes
- Lupus nephritis
- 60-70% of kidney invovlement
- see everything stained on IF
- wire loop lesions - thickening of capillary wall w/subendothelial deposits
8
Q
How does Hemoch-Schonlein Purpura present?
Who is it common in?
What is depostited where?
A
- PRESENTS: purpuric skin lesions on arms, legs, butt
- abdominal pain, vomiting, bleeding
- arthralgia
- renal issues: hematuria, proteinuria, crescentic GN possible
- children 3-8 years after URI
- IgA deposited in mesangium
9
Q
What 3 things do you see with diabetic nephropathy?
A
- capillary basement membrane thickening
- nodular glomerulosclerosis
- diffuse mesangial sclerosis
10
Q
What is the most common cause of acute renal failure - presents with destruction of tubular epithelial cells
A
Acute Tubular Necrosis/Acute Tubular Injury
11
Q
What are the 2 patterns of ATN/ATI
A
- ischemic
- period of inadequate blood flow to kidneys usually related to hypotension and shock
- nephrotoxic
- drugs, heavy metals, lead, organic solvents, contrast dye
12
Q
What is the clinical course of ATN?
A
- initiation - slight decline in urine output and increased BUN
- maintenance: true oliguria, acid base issues, may need dialysis
- recovery - steady increase in urine volume
- prognosis - 95% recover if patient survives initial event
13
Q
What is the major cause of tubulointerstitial nephritis?
A
- inflammatory reactions
14
Q
What do you see with acute and chronic tubulointerstitial nephritis?
A
- acute - neutrophils
- chronic - fibrosis and tubular atrophy
15
Q
What can cause tubulointersitital nephritis?
A
- pyelonephritis (infections)
- drugs, heavy metals
- urate, oxalate disease
- MM
- chronic UT obstruction
- radiation
- transplant rejection
16
Q
What are 3 complications of pyelonephritis?
A
- papillary necrosis
- pyonephrosis
- perinephric abscess
17
Q
What is seen on morphology with chronic pyelonephritis?
A
- damage to pelvocalyceal
- irregularly scarred surface
- blunted deformed calyx
18
Q
How is multiple myeloma damaging to the kidneys?
A
- Has Bence Jones proteinuria that has Ig light chains that are directly toxic to tubules
- combines with Tamm-Horsfall protein to form large tubular casts that cause obstruction and peritubular inflammation
19
Q
What is shown in the image?
A
-
Nephrosclerosis
- ischemic atrophy w/tubular atrophy and intersitital fibrosis
- due to thickening and hyalinization of walls of arterioles and small arteries that narrow the vessel lumen
20
Q
Malignant nephrosclerosis
What is it associated with?
Who is it more common in?
Pathogenesis?
A
- occurs w/hypertension (malignant)
- men, blacks, younger patents
- initial vascular injury followed by leaky vessels, fibrinoid necrosis
- kidney is ischemic and RAAS activated.
21
Q
What is seen on micrscopy with malignant nephrosclerosis?
A
- fibrinoid necrosis of arterioles
- onion skinning of vessels/hyperplastic arteriolitis
22
Q
Renal Artery Stenosis
Cause?
Gross:
A
- normally occlusion w/athermatous plaque
- ischemic kidney is small
- non-ischemic kidney shows hyaline arteriosclerosis
23
Q
Group of disorders that has thrombosis in capillaries and arterioles throughout the body - inappropriate activation of clotting cascade.
What does this cause?
A
- Thrombotic microganiopathies
- renal failure
- thrombocytopenia
- MAHA
24
Q
What is the pathogenesis of thrombotic microganiopathies?
A
- many trigers –> endothelial cell injury –> leads to clots
- reduced prostaglandin I2 and NO
25
**Typical (Classic)** HUS
thrombotic microangiopathy
* most caused by **E. coli**
* produces **verocytotoxin** that causes **endothelial lysis, increased endothelin, decreased NO**
* sudden onset bleeding, oliguria, hematuria, MAHA, neuro changes
* can recover in weeks (with dialysis)
26
**Atypical HUS** associations
* associatios with...
* Antiphospholipid syndrome
* Pregnancy
* Vascular Renal Disease
* Drug related - chemo drugs
27
**Familial HUS**
* inheritied deficiency of **complement reg protein Factor H**
* get uncontrolled complement activation
* high mortality rate
28
**Idiopathic TTP**
* more common in women under 40
* due to defect in process that **cleaves large von-wF muliverse (ADAMTS-13)**
* has nueor features, **50% renal invovlement**
* high mortality
* **treat w/plasma exchange & corticosteroids**
29
What do you see grossly and micrscopically with renal infarcts?
* **gross:**
* **acute WHITE infarcts**
* **wedge shaped**
* **depressed gray-white scars**
* **Micro**
* **red is dead - coagulative necrosis**
30
What does obstructive uropathy lead to?
* hydronephrosis and renal atrophy
* increased sucesptibiliy to infection and stone formation
* kidneys could end up looking like a balloon
31
What are the **causes** of **obstructive uropathy?**
* congenital
* stones
* BIG PROSTATE
* tumors in bladder, ureter, kidney, prostate, uterus
* sloughed papillae
* normal pregnancy
* uterine prolapse
* neurogenic disorders
32
Who and when is **urolithiasis** most common?
What are the main types?
* men \> women
* age of onset is 20-30 years.
* calcium containing - oxalate and phosphate
* struvite stones
* uric acid stones
* cystine stones
33
What is an **angiomyolipoma** associated with? What is it made up of?
Bening or not?
* composed of **vessels, smooth muscle & fat**
* associated w/**tuberus sclerosis**
* autosomal dominant
* CNS hamartomas, retinal hamaratomas, pulmonary & cardiac myomas, cutaneous lesions, renal angiomylolipoma "potato tubers in the brain"
* **BENIGN**
34
What does an **oncocytomas** look like?
Microscopically?
Where does it arise from?
benign?
* **mahogany brown** w/sclerotic scar
* **large eosinophilic cells** w/abundant mitochondria in cells
* arises from **collecting ducts**
* considered a BENIGN TUMOR
35
who is **renal carcinoma** common in?
3 presenting signs
* **male dominant**
* **more common in ELDERLY**
* triad
* **hematuria**
* **palpable mass**
* **flank pain**
36
What does **renal cell carcionma** tend to invade?
What neoplastic syndromes can it present with?
* **invades RENAL VEIN -** may extend into inferior vena cava
* could cause **varicocele** if spermatic vein is blocked.
* metastasis to **bone and lung**
* paraneoplastic
* EPO - high hemoglobin
* Renin - hypertension
* PtH - hypercalcemia
* ACTH - cushing's syndrome
37
What do cases of **renal cell carcinoma** invovle genetically?
Loss of **VHL** --\> leads to **IGF-1 and HIF tx factor**
38
**Von Hippel Lindau Syndrome**
Inheritance?
Presentation
Defect
* **autosomal dominant** disorder
* presnts with **hemangioblastoma** on cerebellum and retina w/**renal cysts and carcinoma**
* **VHL gene on** chromosome **3p25.3**
* tumor supressor gene - normally targets proteins for destruction
39
what is the most common type of renal cell carcinoma?
* **clear cell carcinoma**
* see BRIGHT YELLOW TUMOR
* loss of chromosome 3p in region of VHL
40
**Papillary carcinoma**
What do you see?
Associations?
* cuboidal cells w/papillary growth pattern & foam cells in papillary cores
* **trisomy 7, 17 and loss of Y**
NOT associated w/3p loss.
41
causes 5% of RCC that has an excellent prognosis
* chromophobe carcinoma
