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crrab ii > Part 3 (Warren) - W4 > Flashcards

Part 3 (Warren) - W4 Flashcards

1
Q

What are 2 hereditary diseases associated w/glomerular injury?

A
  1. alport syndrome
  2. thin membrane disease
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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
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3
Q

What is seen on EM of Alport syndrome?

A
  • irregular thick and thin GBM
    • splitting of the lamina densa
    • changes are widespread
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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
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5
Q

What does thin membrane disease present?

A
  • presents w/hematuria
  • EM: shows diffuse thinning of the GBM
  • has an excellent prognosis
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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.
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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
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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
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9
Q

What 3 things do you see with diabetic nephropathy?

A
  1. capillary basement membrane thickening
  2. nodular glomerulosclerosis
  3. diffuse mesangial sclerosis
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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

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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
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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
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13
Q

What is the major cause of tubulointerstitial nephritis?

A
  • inflammatory reactions
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14
Q

What do you see with acute and chronic tubulointerstitial nephritis?

A
  • acute - neutrophils
  • chronic - fibrosis and tubular atrophy
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15
Q

What can cause tubulointersitital nephritis?

A
  • pyelonephritis (infections)
  • drugs, heavy metals
  • urate, oxalate disease
  • MM
  • chronic UT obstruction
  • radiation
  • transplant rejection
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16
Q

What are 3 complications of pyelonephritis?

A
  • papillary necrosis
  • pyonephrosis
  • perinephric abscess
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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
Q

Typical (Classic) HUS

thrombotic microangiopathy

A
  • 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
Q

Atypical HUS associations

A
  • associatios with…
    • Antiphospholipid syndrome
    • Pregnancy
    • Vascular Renal Disease
    • Drug related - chemo drugs
27
Q

Familial HUS

A
  • inheritied deficiency of complement reg protein Factor H
  • get uncontrolled complement activation
  • high mortality rate
28
Q

Idiopathic TTP

A
  • 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
Q

What do you see grossly and micrscopically with renal infarcts?

A
  • gross:
    • ​acute WHITE infarcts
    • wedge shaped
    • depressed gray-white scars
  • Micro
    • ​red is dead - coagulative necrosis
30
Q

What does obstructive uropathy lead to?

A
  • hydronephrosis and renal atrophy
  • increased sucesptibiliy to infection and stone formation
  • kidneys could end up looking like a balloon
31
Q

What are the causes of obstructive uropathy?

A
  • congenital
  • stones
  • BIG PROSTATE
  • tumors in bladder, ureter, kidney, prostate, uterus
  • sloughed papillae
  • normal pregnancy
  • uterine prolapse
  • neurogenic disorders
32
Q

Who and when is urolithiasis most common?

What are the main types?

A
  • men > women
  • age of onset is 20-30 years.
  • calcium containing - oxalate and phosphate
  • struvite stones
  • uric acid stones
  • cystine stones
33
Q

What is an angiomyolipoma associated with? What is it made up of?

Bening or not?

A
  • 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
Q

What does an oncocytomas look like?

Microscopically?

Where does it arise from?

benign?

A
  • mahogany brown w/sclerotic scar
  • large eosinophilic cells w/abundant mitochondria in cells
  • arises from collecting ducts
  • considered a BENIGN TUMOR
35
Q

who is renal carcinoma common in?

3 presenting signs

A
  • male dominant
  • more common in ELDERLY
  • triad
    • hematuria
    • palpable mass
    • flank pain
36
Q

What does renal cell carcionma tend to invade?

What neoplastic syndromes can it present with?

A
  • 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
Q

What do cases of renal cell carcinoma invovle genetically?

A

Loss of VHL –> leads to IGF-1 and HIF tx factor

38
Q

Von Hippel Lindau Syndrome

Inheritance?

Presentation

Defect

A
  • 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
Q

what is the most common type of renal cell carcinoma?

A
  • clear cell carcinoma
    • see BRIGHT YELLOW TUMOR
  • loss of chromosome 3p in region of VHL
40
Q

Papillary carcinoma

What do you see?

Associations?

A
  • cuboidal cells w/papillary growth pattern & foam cells in papillary cores
  • trisomy 7, 17 and loss of Y

NOT associated w/3p loss.

41
Q

causes 5% of RCC that has an excellent prognosis

A
  • chromophobe carcinoma

crrab ii (26 decks)

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