Pathoma - Kidney and Urinary Tract Flashcards

1
Q

Horseshoe kidney is caused by fusion of the kidneys where?

A

The lower pole

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2
Q

How does fusion of the kidneys at the lower pole affect its development?

A

Kidneys will be abnormally located in the lower abdomen due to the horseshoe kidney getting caught on the inferior mesenteric artery

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3
Q

Bilateral renal agenesis will lead to what?

A

Oligohydramnios - leading to lung hypoplasia, flat face with low set ears (Potter’s sequence)

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4
Q

What are the characteristics of a dysplastic kidney?

A

Noninherited - congenital malformation in the renal parenchyma characterized by cysts - tends to be unilateral

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5
Q

What are the basic principles of PKD?

A

Inherited defect leading to bilateral enlarged kidneys with cysts in the renal cortex and medulla

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6
Q

Autosomal recessive PKD is characterized by?

A

Presenting in infants as worsening renal failure and hypertension - congenital hepatic fibrosis and hepatic cysts

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7
Q

ADPKD is characterized by?

A

Presenting in young adults as hypertension - HTN (increased renin), hematuria, and worsening renal failure ADPKD mutation

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8
Q

What is the hallmark associated with acute renal failure?

A

Azotemia - increased BUN and Creatinine often with oliguria

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9
Q

Prerenal azotemia is due to what?

A

Decreased blood flow to the kidneys

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10
Q

Decreased blood flow to the kidneys will do what to the GFR?

A

Decrease GFR leading to azotemia and oliguria

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11
Q

In prerenal kidney injury, what causes the elevation of the BUN:Cr ratio?

A

Decreased blood flow results in increased aldosterone

Aldosterone leads to increased H2O reabsorption

BUN is reabsorbed with H2O

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12
Q

How does prerenal injury affect the FENa and urine osmo?

A

Tubular function remains intact and urine osmo is within normal limits

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13
Q

What is postrenal kiney injury?

A

Decreased outflow results in decreased GFR, azotemia, and oliguria

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14
Q

During the early stages of post renal azotemia, what happens to BUN:Cr, FENa, and urine osmo?

A

Increased back pressure leads to increased BUN forced into reabsorption

Tubular function is intact leading to normal FENa and urine osmo

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15
Q

Long standing post renal obstruction will due what to the BUN:Cr?

A

With long standing obstruction there will be damage to the epithelium leading to a decrease in the amount of BUN that can be reabsorbed. Decrease BUN:Cr.

Damage to epithelium will also lead to a decrease in Na reabsorption and increase FENa and an inability to concentrate urine

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16
Q

What is acute tubular necrosis?

A

Injury and necrosis of tubular epithelial cells

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17
Q

What are the key urinary finding associated with acute tubular necrosis? Why do these occur?

A

Brown granular casts

Caused by epithelium “slouging off” and forming a cast within the tubulum

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18
Q

Dysfunctional tubular epithelium associated with acute tubular necrosis leads to what?

A

Decreased reabsorption of BUN due to damage to epithelium

Decreased reabsorption of Na leading to increased FENa and the inability to concentrate urine

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19
Q

How can ischemia lead to ATN?

A

Decreased blood supply leads to necrosis of the proximal tubule and think ascending limb

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20
Q

What are the clinical features of ATN?

A

Oliguria with brown granular casts

Elevated BUN and creatinine but ratio less than 15

Hyperkalemia due to decreased renal excretion

Metabolic acidosis

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21
Q

What are the characterisitcs associated with acute interstitial nephritis?

A

Drug induced hypersensitivity leading to intrarenal azotemia

Eosinophils may be found in the urine

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22
Q

What is this an image of?

A

Acute tubular necrosis

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23
Q

What is this an image of?

A

Foot process effacement associated with minimal change dz

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24
Q

What is the main characteristic of nephrotic syndrome?

A

Proteinuria > 3.5g/day

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25
Q

Why does can nephrotic syndrome lead to:

Pitting edema

Increased risk of infection

Fatty cast in urine

Increased risk of blood clots

A

Pitting edema - loss of albumin leading to decreased oncotic pressure, and fluid staying in the tissue

Risk of infection - loss of gammaglobulin

Fatty casts - Hyperlipidemia and cholesteremia from the liver

Clots - loss of AT3 in the urine

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26
Q

Who is minimal change disease most likely to affect?

A

This is the most common cause of nephrotic syndrome in children

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27
Q

How would minimal change dz appear on light micro?

A

Normal glomeruli with H&E staining

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28
Q

Minimal change disease will show what on electron microscopy?

A

Foot process effacement (flattening of the podocyte)

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29
Q

How will minimal change disease show up on IF?

A

No immune complex deposition leading to negative IF

30
Q

Who is most commonly affected by Focal Segemental Glomerulosclerosis?

A

Hispanics and African Americans

31
Q

FSGS can be associated with what?

A

HIV, heroin use and sickle cell disease

32
Q

How will FSGS show up on light micro?

A

Focal and segemental glomerulosclerosis

33
Q

How will FSGS show up on EM

A

Foot process effacement

34
Q

How will FSGS show up on IF?

A

No immune complex

35
Q

What are some associations of membranous nephropathy?

A

Caucasian adults, Hep B or C, SLE or drugs

36
Q

How will membranous nephropathy show up on light micro?

A

Thick basement membrane

37
Q

How will membranous nephropathy show up on EM and IF?

A

IF - granular deposits due to immune complex deposition

EM - spike and dome appearance due to subepithelial deposits

38
Q

Membranoproliferative glomerularnephritis will tend to have what appearance on H&E?

A

Thick glomerular basement membrane yielding tram track appearance

Due to immune complex deposition

39
Q

What are the two types of MPGN?

A

Type 1 - subendothelial associated with HBV and HCV

Type II - C3 autoantibody that stabilized C3 convertase leading to overactivation of complement, inflammation and decreased circulating C3

40
Q

What is the initial event in diabetes leading to nephrotic syndrome?

A

Elevated serum glucose leads to non-enzymatic glycosylation of vascular basement membrane and hyaline arteriosclerosis

41
Q

What is the most affected region of the kidney in diabetes?

A

Efferent arteriole leading to increased glomerular filtration pressure

42
Q

What causes the Kimmelstiel-Wilson nodules in DM?

A

Sclerosis of the mesangium

43
Q

What is the hallmark of amyloidosis?

A

Green-apple birefringence under congo red staining

44
Q

What are the basic principles of Nephritic Syndrome?

A

Glomerular inflammation and bleeding leading to RBC casts and dysmorphic RBCs in Urine

Periorbital edema

HTN

Oliguria and Azotemia

Biopsy will reveal hypercellular and inflamed glomeruli

45
Q

Why are there neutrophils in glomerulus during nephritic syndrome?

A

Immune complex deposition leads to activation of complement. C5a will attract neutrophils which mediate damage

46
Q

What are the important characteristics of Post-strep glomerulonephritis?

A

2-3 wks after infection

Cola colored urine (Hematuria)

47
Q

What is the IF and EM findings on PSGN?

A

Sub-epithelial humps on EM

Granular IF

48
Q

What are the characteristics of RPGN?

A

Crescents in the Bowman’s space that are comprised of fibrin and macrophages

49
Q

What 5 diseases can lead to RPGN?

A

Goodpastures

PSGN

Diffuse proliferative

c-ANCA

p-ANCA

50
Q

How will Goodpasture’s present on IF?

A

Linear deposits on the GBM

51
Q

What causes the linear deposition associated with Goodpastures?

A

auto-Ab against collagen in the BM of the glomeruli

52
Q

What are two key clinical symptoms of goodpasture’s?

A

Hemoptysis and hematuria

53
Q

What is the cause of diffuse proliferative GN presentation?

A

Due to diffuse antigen-antibody complex deposition - usually sub-endothelial and most commonly associated with SLE presenting as Nephritic syndrome

Granular IF pattern due to subendothelial immune complex deposition

54
Q

A negative IF in a patient with RPGN will make a physician think?

A

ANCA associated RPGN

55
Q

How can you distinguish Wegener’s from Goodpasture’s?

A

c-ANCA - Wegener’s - will present with nasopharyngeal, lung and kidney problems

Goodpastures - Lung and kidney

56
Q

How can you distinguish the two p-ANCA diseases?

A

Churg-Strauss - Allergic reaction, granulomatous inflammation

Microscopic polyangiitis - Does have that

57
Q

IgA nephropathy (Berger Disease) will present as what?

A

IgA complex deposition in mesangium leading to mesangial proliferation

58
Q

Why will IgA nephropathy present the way it does?

A

IgA immune complex deposition will lead to hematuria

IgA deposition will occur post mucosal infection

59
Q

What are the characteristics of Alport’s Syndrome?

A

Inherited defect in type IV collagen (X-linked)

Thinning of the BM

60
Q

What are the classic symptoms of Alport Syndrome?

A

Isolated hematuria, sensory hearing loss, and ocular disturbances

61
Q

What is the main result of chronic pyleonephritis?

A

Interstitial fibrosis and atrophy of tubules due to multiple bouts of acute pyelonephritis

62
Q

What is a major morphological change associated with Chronic pyleonephritis?

A

Thyroidization of the Kidney

63
Q

What is the most common cause of clacium oxalate/calcium phosphate kidney stones?

A

Idiopathic hypercalcuria

64
Q

What is the cause of ammonium magnesium phosphate kidney stones?

A

Urease-positive organisms - leading to alkaline urine and formation of a stone

65
Q

Angiomyolipoma is comprised of what?

A

Blood vessels, smooth muscle, and adipose tissue

66
Q

RCC can present with involvement of what?

A

The left renal vein which can lead to blockage of the left spermatic vein

67
Q

What is the pathogenesis of RCC?

A

Loss of VHL gene leading to increased IGF-1, HIF (increasing VEGF and PDGF)

68
Q

Wilms Tumor is most common in who and presents with what?

A

Most common malignant renal tumor in children, comprised of blastema (immature kidney mesenchyme)

Presents with Hematuria and hypertension due to renin secretion

69
Q

What is WAGR syndrome?

A

Common syndrome associated with WT1 mutation

W - Wilms tumor

A - Aniridia

G - Genital abnormalities

R - Mental and growth retardation

70
Q

What are the major risk factors for Urothelial (transitional cell) carcinoma

A

Smoking

71
Q

Papillary Urothelial cell carcinoma tend to be associated with?

A

p53 mutations

72
Q

Squamous cell carcinoma is most common associated with?

A

Bacterial infection with Schistosoma haematobium infection (Egyptian male)