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
What are the forms of nephritic syndrome and what are they caused by?
1. Acute proliferative glomerulonephritis- post Strep infections 2. Non-Strep acute glomerulonephritis- other infections 3. Rapidly progressive/crescentic glomerulonephritis- idiopathic or systemic
26
What is nephrotic syndrome?
Derangement in capillary walls causes increased plasma protein permeability
27
Loss of albumin in nephrotic syndrome causes what?
Systemic edema
28
What diseases cause nephrotic syndrome?
Membranous nephropathy (adults) Minimum change disease (children) Focal segmental glomerulosclerosis Idiopathic focal segmental glomerulosclerosis HIV associated neohropathy Membranoproliferative glomerulonephritis
29
What is the difference between membranous nephropathy and minimum change disease (besides age)?
MN- immune mediated MCD- not immune mediated
30
What is the most common cause of adult nephrotic syndrome?
Focal segmental glomerulosclerosis
31
What is HIV associated nephropathy a variant of?
Focal segmental glomerulosclerosis
32
What is the most common type of glomerulonephritis worldwide?
IgA nephropathy (Berger disease)
33
What is the pathology of IgA nephropathy?
IgA deposits cause gross hematuria following infection
34
What is the cause of hereditary nephritis?
X-linked mutation Defective type IV collagen
35
What is another name for hereditary nephritis?
Alport syndrome
36
What are the symptoms of hereditary nephritis?
Chronic renal failure Nerve deafness Eye disorders
37
What kind of mutation causes thin BM lesions?
Type IV collagen mutation
38
What is the morphology of chronic glomerulonephritis?
Kidneys are bilaterally/symmetrically contracted with granular surface and thinned cortex Completely effaced glomeruli (can’t ID primary lesion)
39
What are the symptoms of chronic glomerulonephritis?
``` Hypertension Pericarditis Gastroenteritis Secondary hyperparathyroidism Renal osteosystrophy ```
40
What are examples of glomerular lesions associated with systemic diseases?
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
41
What are the characteristics of Henoch-Schonlein purpura?
IgA deposition in children, vasculitis, abdominal symptoms, joint pain, glomerulonephritis
42
What are the major processes of tubular and intertitial disease?
1. Ischemic/toxic tubular injury (acute tubular injury) | 2. Inflammatory reactions of tubules and interstitium
43
What is the most common cause if ARF?
Ischemic/toxic tubular injury
44
What does ischemia and direct toxic injury to kidney tubules lead to?
Necrosis
45
What are the phases of ischemic/toxic tubular injury?
1. Initiation 2. Maintenance 3. Recovery
46
What can cause luminal destruction in tubular injury?
Injured cells detach from BM and cause obstruction
47
What is tubulointerstitial nephritis?
Absence of nephritic and nephrotic syndromes
48
What are examples of inflammatory reactions of tubules and interstitium?
Tubulointerstitial nephritis Pyelonephritis and UTI
49
What type of infections cause most UTIs?
Gram negative GI normal flora
50
What route of spread is most common in pyelonephritis and UTI?
Ascending route
51
What is the morphology of acute pyelonephritis?
Patchy suppurative inflammation Tubular necrosis Neutrophil cast Renal scars from necrosis, pyonephrosis, perinephric abscesses
52
What is the morphology of chronic pyelonephritis?
Tubulointerstitial inflammation Irregular renal scarring Dilated/deformed calyxes
53
What are the types of chronic pyelonephritis and which is most common?
Reflux nephropathy- most common Chronic obstructive pyelonephritis
54
What is the second most common cause of acute tubular injury?
Tubulointerstitial nephritis induced by drugs and toxins
55
What kind of response does acute, drug-induced interstitial nephritis cause?
IgE and T cell response
56
What is the morphology of acute urate nephropathy?
Uric avid crystals ppt in tubules and CDs
57
What causes chronic urate nephropathy?
Prolonged hyperuricemia (gout)
58
What is nephrolithiasis?
Uric acid stones
59
What is hypercalcemia and nephrocalcinosis?
Renal calcium deposition and stone formation
60
What is the other name for light chain cast nephropathy?
Myeloma kidney
61
What condition is light chain cast nephropathy associated with?
Multiple myeloma
62
What occurs in bile cast nephropathy?
Increased serum bilirubin causes bile cast formation
63
What is hepatorenal syndrome?
Impairment of renal function in patients with liver failure
64
What is nephrosclerosis?
Renal arteriolar sclerosis Wall thickening and hyalinization
65
What is the morphology of nephrosclerosis?
Vascular lesions cause diffuse, ischemic nephron atrophy Cortex shrinking Granular surface Small kidneys
66
What is malignant nephrosclerosis?
Nephrosclerosis superimposed on hypertensive chronic renal disease or scleroderma
67
What is the morphology of malignant nephrosclerosis?
Fibrinoid necrosis Intravascular thrombosis Salt and water retention
68
What is the pathology of malignant nephrosclerosis?
Stimulation of RAS- aldosterone causes salt and water retention Drives an ever-increasing cycle of BP
69
What does renal artery stenosis induce?
Excessive renin secretion
70
What is most renal artery sclerosis due to?
Atheromatous plaque
71
What is the morphology of renal artery sclerosis?
Reduced kidney size Diffuse ischemic atrophy
72
What are thrombotic microangiopathies?
Spectrum of syndromes that cause excessive platelet activation Thromboses in kidney vessels cause hypertension
73
What are the two forms of thrombotic microangiopathies and there causes?
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
What other vascular disorders are associated with kidney disease?
Atheroembolic renal disease (cholesterol crystals embolize) Sickle cell nephropathy Diffuse cortical nephrosis (diffuse microthrombi) Renal infarcts
75
Why do renal infarcts occur?
Little collateral circulation
76
What congenital anomalies are associated with the kidneys?
Agenesis Hypoplasia (unilateral) Ectopic Horseshoe kidneys
77
What is the difference between bilateral and unilateral agenesis?
Bilateral- incompatible with life Unilateral- hypertrophy of remaining kidney
78
Is hypoplasia normally unilateral or bilateral?
Unilateral
79
What are the characteristics of true kidney hypoplasia?
No scars, less than 6 renal lobes
80
Where do ectopic kidneys normally lie?
Just above the pelvic brim or in the pelvis
81
Why is their an increased risk of bacterial infection in ectopic kidneys?
Ureter can be tortuous or kinked
82
What is the difference between simple and crossed ectopic kidneys?
Simple- kidneys are in an abnormal position on the proper side Crossed- both kidneys are on the same side
83
What type of pole fusion is most common in horseshoe kidneys?
Lower pole
84
What types of cystic diseases can occur in kidneys?
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
Which form of PKD is adult and which is childhood?
Adult- autosomal dominant PKD Childhood- autosomal recessive PKD
86
What are the characteristics of AD PKD?
High penetrance, universally bilateral PKD1 mutation in most cases Kidneys are massively enlarged and almost entirely cystic 40% also show liver cysts
87
What are the characteristics of AR PKD?
Mutations in PKHD1 Liver almost always has cysts and proliferating bile ducts Sponge-like appearance due to small cysts in the cortex Bilateral
88
What is the morphology of medullary sponge kidney?
Multiple cystic dilations of the collecting ducts in the medulla Pyramids are lost and filled with cysts
89
What is the morphology of nephronophthisis and adult onset medullary cystic disease?
Variable number of cysts in the medulla, concentrated at the corticomedullary junction Small, contracted kidneys Granular surface
90
What is the morphology of multicystic renal dysplasia?
Unilateral or bilateral Kidney is large, irregular and multicystic
91
What are most cases of multicystic renal dysplasia associated with?
Ureteropelvic obstruction Urethral agenesis Atresia
92
Why do prolonged dialysis patients develop cysts?
Obstruction from calculi and interstitial fibrosis
93
Where do simple cysts of the kidney commonly occur?
Cortex
94
What is the morphology of simple kidney cysts?
Smooth walls filled with clear, serous fluid
95
What are the characteristics of urinary tract obstruction?
Increased susceptibility to infection and stone formation Renal atrophy and expanded renal pelvis
96
What is hydronephrosis?
Dilation of renal pelvis and calicoes due to urine obstruction outflow
97
What is urolithiasis?
Increased concentrations of stone constituents
98
Where is urolithiasis most common in the kidney?
Calyces and pelvis
99
What benign neoplasms are associated with the kidney and what are their characteristics?
1. Renal papillary adenomatous- small, yellow, cortical 2. Angiomyolipoma- hamartomatous lesion (vessels, muscle, fat) 3. Oncocytoma- eosinophilic epithelial cells, packed with mitochondria
100
What malignant neoplasm is histologically identical to low grade papillary RCC?
Renal papillary adenoma
101
What is the cut off between benign and malignant renal papillary adenoma?
3cm
102
Why are angiomyolipomas significant?
Can cause hemorrhage
103
What malignant neoplasms are associated with the kidney?
Renal cell carcinoma (RCC) Clear cell carcinoma Papillary carcinomas Chromophobe renal carcinoma Urothelial carcinoma of the renal pelvis
104
What is the most common form of renal cancer in adults?
RCC
105
What are the three forms of RCC and their characteristics?
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
What is paraneoplastic syndrome attributable to?
Hormone production
107
Why do urothelial carcinomas of the renal pelvis manifest early?
Produce hematuria or obstruction
108
What concomitant tumour can occur with urothelial carcinomas of the renal pelvis?
Bladder