Chapter 20- The Kidney Flashcards

1
Q

What are the functions of the kidney?

A

Filters blood and produces urine

Waste excretion

Regulation of water concentration, salt, calcium, phosphorus, etc

Maintains plasma pH

Hormone production

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2
Q

What are the four compartments of the kidney?

A
  1. Glomeruli
  2. Tubules
  3. Interstitium
  4. Vessels
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3
Q

Chronic renal disease destroys what kidney compartments?

A

All of them

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4
Q

What is azotemia and what does it reflect?

A

Increased BUN/serum creatinine

Reflects reduced GFR

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5
Q

What is uremia?

A

Constellation of clinical signs and symptoms associated with azotemia

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6
Q

What type of collagen makes up the glomerulus BM?

A

Type IV

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7
Q

What cells support the glomerulus?

A

Mesangial cells

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8
Q

What type of cells are mesangial cells?

A

Contractile, phagocytic, secretive

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9
Q

What is glomerular filtration determined by?

A

Molecule size and charge

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10
Q

What type of iron is most permeable to the glomerulus?

A

Cations

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11
Q

What are the functions of the JGA?

A

BP and GFR

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12
Q

What structures are involved in the JGA?

A

DCT and afferent arteriole

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13
Q

What is the macula densa?

A

Specialized cells in the DCT

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14
Q

What is the function of the macula densa?

A

Detects sodium concentration (high levels contract the arteriole)

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15
Q

When is renin secreted?

A

When BP falls

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16
Q

What are the pathologic responses to glomerular injury?

A
  1. Hypercellularity
  2. GBM thickening
  3. Hylainosis
  4. Sclerosis
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17
Q

What form of hypercellularity is seen in acute glomerular injury?

A

Crescents

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18
Q

What mechanisms underlie most forms of glomerular injury?

A

Immune- deposition of complexes, Abs against Ags or GBM

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19
Q

What is the pathogenesis of Goodpasture syndrome?

A

Ab against Type IV collagen

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20
Q

Where do cations, anions and neutral molecules accumulate in the glomerulus?

A

Cations- cross BM, form subepithelial complexes

Anions- trapped subendothelially

Neutral- accumulate in mesangium

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21
Q

What is a common feature of glomerular disease and why?

A

Epithelial cell injury

Podocytes have limited regenerative capacity

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22
Q

What are the major features of progressive renal disease?

A
  1. Focal segmental glomeeulosclerosis

2. Tubulointerstitial fibrosis

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23
Q

What is nephritic syndrome?

A

Manifestation of glomerular inflammation

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24
Q

What is nephritic syndrome characterized by?

A

Hematuria

Oliguria

Azotemia

Proteinuria

Hypertension

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25
Q

What are the forms of nephritic syndrome and what are they caused by?

A
  1. Acute proliferative glomerulonephritis- post Strep infections
  2. Non-Strep acute glomerulonephritis- other infections
  3. Rapidly progressive/crescentic glomerulonephritis- idiopathic or systemic
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26
Q

What is nephrotic syndrome?

A

Derangement in capillary walls causes increased plasma protein permeability

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27
Q

Loss of albumin in nephrotic syndrome causes what?

A

Systemic edema

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28
Q

What diseases cause nephrotic syndrome?

A

Membranous nephropathy (adults)

Minimum change disease (children)

Focal segmental glomerulosclerosis

Idiopathic focal segmental glomerulosclerosis

HIV associated neohropathy

Membranoproliferative glomerulonephritis

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29
Q

What is the difference between membranous nephropathy and minimum change disease (besides age)?

A

MN- immune mediated

MCD- not immune mediated

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30
Q

What is the most common cause of adult nephrotic syndrome?

A

Focal segmental glomerulosclerosis

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31
Q

What is HIV associated nephropathy a variant of?

A

Focal segmental glomerulosclerosis

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32
Q

What is the most common type of glomerulonephritis worldwide?

A

IgA nephropathy (Berger disease)

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33
Q

What is the pathology of IgA nephropathy?

A

IgA deposits cause gross hematuria following infection

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34
Q

What is the cause of hereditary nephritis?

A

X-linked mutation

Defective type IV collagen

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35
Q

What is another name for hereditary nephritis?

A

Alport syndrome

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36
Q

What are the symptoms of hereditary nephritis?

A

Chronic renal failure

Nerve deafness

Eye disorders

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37
Q

What kind of mutation causes thin BM lesions?

A

Type IV collagen mutation

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38
Q

What is the morphology of chronic glomerulonephritis?

A

Kidneys are bilaterally/symmetrically contracted with granular surface and thinned cortex

Completely effaced glomeruli (can’t ID primary lesion)

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39
Q

What are the symptoms of chronic glomerulonephritis?

A
Hypertension
Pericarditis
Gastroenteritis
Secondary hyperparathyroidism
Renal osteosystrophy
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40
Q

What are examples of glomerular lesions associated with systemic diseases?

A
  1. Henoch-Schonlein purpura

Lupus nephritis

GN associated with bacterial endocarditis and other infections

Diabetic nephropathy

Fibrillary glomerulonephritis

Goodpasture syndrome

Microscopic polyangitis

Wegner granulomatosis

Essential mixed cryoglobulinemia

Plasma cell dyscrasias

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41
Q

What are the characteristics of Henoch-Schonlein purpura?

A

IgA deposition in children, vasculitis, abdominal symptoms, joint pain, glomerulonephritis

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42
Q

What are the major processes of tubular and intertitial disease?

A
  1. Ischemic/toxic tubular injury (acute tubular injury)

2. Inflammatory reactions of tubules and interstitium

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43
Q

What is the most common cause if ARF?

A

Ischemic/toxic tubular injury

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44
Q

What does ischemia and direct toxic injury to kidney tubules lead to?

A

Necrosis

45
Q

What are the phases of ischemic/toxic tubular injury?

A
  1. Initiation
  2. Maintenance
  3. Recovery
46
Q

What can cause luminal destruction in tubular injury?

A

Injured cells detach from BM and cause obstruction

47
Q

What is tubulointerstitial nephritis?

A

Absence of nephritic and nephrotic syndromes

48
Q

What are examples of inflammatory reactions of tubules and interstitium?

A

Tubulointerstitial nephritis

Pyelonephritis and UTI

49
Q

What type of infections cause most UTIs?

A

Gram negative GI normal flora

50
Q

What route of spread is most common in pyelonephritis and UTI?

A

Ascending route

51
Q

What is the morphology of acute pyelonephritis?

A

Patchy suppurative inflammation

Tubular necrosis

Neutrophil cast

Renal scars from necrosis, pyonephrosis, perinephric abscesses

52
Q

What is the morphology of chronic pyelonephritis?

A

Tubulointerstitial inflammation

Irregular renal scarring

Dilated/deformed calyxes

53
Q

What are the types of chronic pyelonephritis and which is most common?

A

Reflux nephropathy- most common

Chronic obstructive pyelonephritis

54
Q

What is the second most common cause of acute tubular injury?

A

Tubulointerstitial nephritis induced by drugs and toxins

55
Q

What kind of response does acute, drug-induced interstitial nephritis cause?

A

IgE and T cell response

56
Q

What is the morphology of acute urate nephropathy?

A

Uric avid crystals ppt in tubules and CDs

57
Q

What causes chronic urate nephropathy?

A

Prolonged hyperuricemia (gout)

58
Q

What is nephrolithiasis?

A

Uric acid stones

59
Q

What is hypercalcemia and nephrocalcinosis?

A

Renal calcium deposition and stone formation

60
Q

What is the other name for light chain cast nephropathy?

A

Myeloma kidney

61
Q

What condition is light chain cast nephropathy associated with?

A

Multiple myeloma

62
Q

What occurs in bile cast nephropathy?

A

Increased serum bilirubin causes bile cast formation

63
Q

What is hepatorenal syndrome?

A

Impairment of renal function in patients with liver failure

64
Q

What is nephrosclerosis?

A

Renal arteriolar sclerosis

Wall thickening and hyalinization

65
Q

What is the morphology of nephrosclerosis?

A

Vascular lesions cause diffuse, ischemic nephron atrophy

Cortex shrinking

Granular surface

Small kidneys

66
Q

What is malignant nephrosclerosis?

A

Nephrosclerosis superimposed on hypertensive chronic renal disease or scleroderma

67
Q

What is the morphology of malignant nephrosclerosis?

A

Fibrinoid necrosis

Intravascular thrombosis

Salt and water retention

68
Q

What is the pathology of malignant nephrosclerosis?

A

Stimulation of RAS- aldosterone causes salt and water retention

Drives an ever-increasing cycle of BP

69
Q

What does renal artery stenosis induce?

A

Excessive renin secretion

70
Q

What is most renal artery sclerosis due to?

A

Atheromatous plaque

71
Q

What is the morphology of renal artery sclerosis?

A

Reduced kidney size

Diffuse ischemic atrophy

72
Q

What are thrombotic microangiopathies?

A

Spectrum of syndromes that cause excessive platelet activation

Thromboses in kidney vessels cause hypertension

73
Q

What are the two forms of thrombotic microangiopathies and there causes?

A
  1. Thrombotic thrombocytopenia purpura (TTP)- ADAMSTS13 deficiencies (vWF regulator)
  2. Hemolytic uremic syndrome (HUS)- shiga-toxin producing bacteria (typical) or mutations in complement regulatory proteins (atypical)
74
Q

What other vascular disorders are associated with kidney disease?

A

Atheroembolic renal disease (cholesterol crystals embolize)

Sickle cell nephropathy

Diffuse cortical nephrosis (diffuse microthrombi)

Renal infarcts

75
Q

Why do renal infarcts occur?

A

Little collateral circulation

76
Q

What congenital anomalies are associated with the kidneys?

A

Agenesis

Hypoplasia (unilateral)

Ectopic

Horseshoe kidneys

77
Q

What is the difference between bilateral and unilateral agenesis?

A

Bilateral- incompatible with life

Unilateral- hypertrophy of remaining kidney

78
Q

Is hypoplasia normally unilateral or bilateral?

A

Unilateral

79
Q

What are the characteristics of true kidney hypoplasia?

A

No scars, less than 6 renal lobes

80
Q

Where do ectopic kidneys normally lie?

A

Just above the pelvic brim or in the pelvis

81
Q

Why is their an increased risk of bacterial infection in ectopic kidneys?

A

Ureter can be tortuous or kinked

82
Q

What is the difference between simple and crossed ectopic kidneys?

A

Simple- kidneys are in an abnormal position on the proper side

Crossed- both kidneys are on the same side

83
Q

What type of pole fusion is most common in horseshoe kidneys?

A

Lower pole

84
Q

What types of cystic diseases can occur in kidneys?

A

Autosomal dominant polycystic kidney disease

Autosomal recessive PKD

Medullary sponge kidney

Nephronophthisis and adult onset medullary cystic disease

Multicystic renal dysplasia

Acquired (dialysis associated) cystic disease

Simple cysts

85
Q

Which form of PKD is adult and which is childhood?

A

Adult- autosomal dominant PKD

Childhood- autosomal recessive PKD

86
Q

What are the characteristics of AD PKD?

A

High penetrance, universally bilateral

PKD1 mutation in most cases

Kidneys are massively enlarged and almost entirely cystic

40% also show liver cysts

87
Q

What are the characteristics of AR PKD?

A

Mutations in PKHD1

Liver almost always has cysts and proliferating bile ducts

Sponge-like appearance due to small cysts in the cortex

Bilateral

88
Q

What is the morphology of medullary sponge kidney?

A

Multiple cystic dilations of the collecting ducts in the medulla

Pyramids are lost and filled with cysts

89
Q

What is the morphology of nephronophthisis and adult onset medullary cystic disease?

A

Variable number of cysts in the medulla, concentrated at the corticomedullary junction

Small, contracted kidneys

Granular surface

90
Q

What is the morphology of multicystic renal dysplasia?

A

Unilateral or bilateral

Kidney is large, irregular and multicystic

91
Q

What are most cases of multicystic renal dysplasia associated with?

A

Ureteropelvic obstruction

Urethral agenesis

Atresia

92
Q

Why do prolonged dialysis patients develop cysts?

A

Obstruction from calculi and interstitial fibrosis

93
Q

Where do simple cysts of the kidney commonly occur?

A

Cortex

94
Q

What is the morphology of simple kidney cysts?

A

Smooth walls filled with clear, serous fluid

95
Q

What are the characteristics of urinary tract obstruction?

A

Increased susceptibility to infection and stone formation

Renal atrophy and expanded renal pelvis

96
Q

What is hydronephrosis?

A

Dilation of renal pelvis and calicoes due to urine obstruction outflow

97
Q

What is urolithiasis?

A

Increased concentrations of stone constituents

98
Q

Where is urolithiasis most common in the kidney?

A

Calyces and pelvis

99
Q

What benign neoplasms are associated with the kidney and what are their characteristics?

A
  1. Renal papillary adenomatous- small, yellow, cortical
  2. Angiomyolipoma- hamartomatous lesion (vessels, muscle, fat)
  3. Oncocytoma- eosinophilic epithelial cells, packed with mitochondria
100
Q

What malignant neoplasm is histologically identical to low grade papillary RCC?

A

Renal papillary adenoma

101
Q

What is the cut off between benign and malignant renal papillary adenoma?

A

3cm

102
Q

Why are angiomyolipomas significant?

A

Can cause hemorrhage

103
Q

What malignant neoplasms are associated with the kidney?

A

Renal cell carcinoma (RCC)

Clear cell carcinoma

Papillary carcinomas

Chromophobe renal carcinoma

Urothelial carcinoma of the renal pelvis

104
Q

What is the most common form of renal cancer in adults?

A

RCC

105
Q

What are the three forms of RCC and their characteristics?

A
  1. Clear cell- solitary, spherical masses, invade the renal vein
  2. Papillary- multifocal and bilateral, hemorrhagic and cystic, papillary formations (microscopic)
  3. Chromophobe- eosinophilic cells with perinuclear halos
106
Q

What is paraneoplastic syndrome attributable to?

A

Hormone production

107
Q

Why do urothelial carcinomas of the renal pelvis manifest early?

A

Produce hematuria or obstruction

108
Q

What concomitant tumour can occur with urothelial carcinomas of the renal pelvis?

A

Bladder