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Renal - Pathoma Flashcards

1
Q

Pathoma Renal

Unilateral renal agenesis leads to __________ of the existing kidney.

Bilateral renal agenesis leads to __________ __________.

A

Unilateral renal agenesis leads to hypertrophy of the existing kidney.

Bilateral renal agenesis leads to Potter Sequence.

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2
Q

Pathoma Renal

Are there any long-term sequelae of unilateral renal agenesis in the extant kidney?

A

Yes: increased risk of renal failure later in life due to hyperfiltration

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3
Q

Pathoma Renal

What condition is a non-inherited congenital malformation of the kidneys resulting in cysts and abnormal tissue in the renal parenchyma?

A

Dysplastic kidney

(usually unilateral, but must be distinguished from PKD if bilateral)

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4
Q

Pathoma Renal

Which form of PKD is associated with hepatic fibrosis and portal hypertension?

Which form is associated with hepatic cysts?

A

ARPKD

Both ADPKD and ARPKD

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5
Q

Pathoma Renal

A patient presents with shrunken kidneys, parenchymal fibrosis, and worsening renal failure.

You note cysts specifically in the medullary collecting ducts. What disease is this?

A

Medullary cystic kidney disease

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6
Q

Pathoma Renal

What is the expected BUN:Cr ratio, FENa, and OsmolarityUrine for prerenal azotemia?

A

> 15

< 1%

> 500 mOsm/kg

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7
Q

Pathoma Renal

Explain the BUN:Cr ratio (> 15), FENa (< 1%), and OsmolarityUrine (> 500 mOsm/Kg) seen in prerenal azotemia.

A

BUN:Cr ratio — The kidneys increase fluid and BUN reabsorption

FENa — Tubular function remains intact

OsmolarityUrine — Tubular function remains intact

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8
Q

Pathoma Renal

What is the expected BUN:Cr ratio, FENa, and OsmolarityUrine for postrenal azotemia?

A

> 15

< 1%

> 500 mOsm/kg

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9
Q

Pathoma Renal

Explain the BUN:Cr ratio (> 15), FENa (< 1%), and OsmolarityUrine (> 500 mOsm/Kg) seen in prerenal azotemia.

A

BUN:Cr ratio — Increased tubular pressure forces BUN back into serum

FENa — Tubular function remains intact

OsmolarityUrine — Tubular function remains intact

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10
Q

Pathoma Renal

What is the expected BUN:Cr ratio, FENa, and OsmolarityUrine for intrarenal azotemia?

A

< 15

> 2%

< 500 mOsm/kg

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11
Q

Pathoma Renal

Explain the BUN:Cr ratio (< 15), FENa (> 2%), and OsmolarityUrine (

A

BUN:Cr ratio — Tubular function decreases, resulting in decreased BUN reabsorption

FENa — Tubular function is impaired

OsmolarityUrine — Tubular function is impaired

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12
Q

Pathoma Renal

In what condition might post-renal azotemia lead to a low BUN:Cr ratio (< 15) and an elevated FENa (> 2%)?

A

Long-standing post-renal azotemia

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13
Q

Pathoma Renal

What are the two major etiologies of acute tubular necrosis?

A

(1) Ischemia (i.e. secondary to prerenal azotemia)
(2) Nephrotoxicity

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14
Q

Pathoma Renal

Name some of the nephrotoxic substances associated with acute tubular necrosis:

A___________

H___________

M___________

U___________

R___________

E___________

A

Name some of the nephrotoxic substances associated with acute tubular necrosis:

Aminoglycosides**

Heavy metals

Myoglobinuria

Urate

Radiocontrast dye

Ethylene glycol

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15
Q

Pathoma Renal

How can urate-induced acute tubular necrosis be avoided in patients undergoing chemotherapy?

A

Hydration + allopurinol

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16
Q

Pathoma Renal

What are the serum changes associated with acute tubular necrosis [think ion change(s) and pH]?

A

Hyperkalemia

+

metabolic alkalosis

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17
Q

Pathoma Renal

True/False.

Patients with acute tubular necrosis may require dialysis to treat their electrolyte imbalances, but they typically recover completely within a few weeks.

A

True.

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18
Q

Pathoma Renal

What cause of intrarenal azotemia is associated with drugs that act like haptens?

A

Acute interstitial nephritis

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19
Q

Pathoma Renal

Name some of the drugs that act like haptens and are associated with acute interstitial nephritis.

NPD

A

NSAIDs,

penicillins,

diuretics

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20
Q

Pathoma Renal

In addition to a case of severe acute interstitial nephritis resulting from hapten-like drugs (e.g. NSAIDs, penicillins, diuretics, etc.), name a few potential etiologies of renal papillary necrosis:

C________________

D________________

S________________

S________________

A

In addition to a case of severe acute interstitial nephritis resulting from hapten-like drugs (e.g. NSAIDs, penicillins, diuretics, etc.), name a few potential etiologies of renal papillary necrosis:

Chronic analgesic abuse

Diabetes mellitus

Sickle cell trait or disease

Severe acute pyelonephritis

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21
Q

Pathoma Renal

A patient presents with puffy eyes, foamy urine, hypoalbuminema, and hyperlipidemia. What is the likely generic diagnosis requiring further work-up?

A

Nephrotic syndrome

(may also present with hypertension, infection, clotting, etc.)

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22
Q

Pathoma Renal

Nephrotic syndrome is characterized by a proteinuria of what amount?

A

> 3.5 g / day

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23
Q

Pathoma Renal

What are the four serum characteristics of nephrotic syndrome?

(Two hypos- and two hypers-)

A

Hypoalbuminemia

Hypogammaglobulinemia

Hypercoagulable states

Hyperlipidemia and Hypercholesterolemia

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24
Q

Pathoma Renal

Why does nephrotic syndrome result in a hypercoagulable state?

A

Loss of antithrombin III

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25
Q

Pathoma Renal

What result may be seen in the urine due to the hyperlipidemia and hypercholesterolemia seen in nephrotic syndrome?

A

Fatty casts

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26
Q

Pathoma Renal

Name the major causes of nephrotic syndrome:

M______________

F______________

M______________

M______________

D______________

S______________

A

Name the major causes of nephrotic syndrome:

Minimal change disease

Focal segmental glomerulosclerosis

Membranous nephropathy

Membranoproliferative glomerulonephritis

Diabetes mellitus

Systemic amyloidosis

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27
Q

Pathoma Renal

What is the most common cause of nephrotic syndrome in children?

And in Caucasians?

And in Hispanics?

And in African-Americans?

A

Minimal change disease

Membranous nephropathy

Focal segmental glomerulosclerosis

Focal segmental glomerulosclerosis

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28
Q

Pathoma Renal

Although minimal change disease is idiopathic, it may be associated with what disease?

A

Hodgkin lymphoma

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29
Q

Pathoma Renal

How does minimal change disease appear on H&E?

A

Normal glomeruli;

maybe some lipids in proximal tubule cells

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30
Q

Pathoma Renal

How does minimal change disease appear on electron microscopy?

A

Foot process effacement

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31
Q

Pathoma Renal

True/False.

Minimal change disease is associated with loss of albumin and gammaglobulin in the urine.

A

False.

Minimal change disease is associated with loss of albumin only (selective proteinuria).

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32
Q

Pathoma Renal

Good response to steroids is seen in which etiology(ies) of nephrotic syndrome?

A

Minimal change disease only

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33
Q

Pathoma Renal

The damage in minimal change disease is mediated by what?

A

T cell cytokines

(hence the excellent response to steroids)

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34
Q

Pathoma Renal

Which major causes of nephrotic syndrome are immunofluorescence-negative?

A

Minimal change disease

Focal segmental glomerulosclerosis

(also DM and amyloidosis)

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35
Q

Pathoma Renal

True/False.

Focal segmental glomerulosclerosis is the most common cause of nephrotic syndrome in Hispanics and African-Americans. It is IF-negative and typically idiopathic (although also associated with HIV, heroin use, and sickle cell disease).

A

True.

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36
Q

Pathoma Renal

How does focal segmental glomerulosclerosis appear on H&E?

A

Exactly what the name says:

Some glomeruli (focal) and only some parts of those glomeruli (segmental) are sclerosed

I.e. focal segmental glomerulosclerosis

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37
Q

Pathoma Renal

How does focal segmental glomerulosclerosis appear on electron microscopy?

A

Foot process effacement

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38
Q

Pathoma Renal

True/False.

Membranous nephropathy is usually idiopathic, but it may be associated with HIV, heroin use, and sickle cell disease.

A

False (The previous description matched focal segmental glomerulosclerosis.).

Membranous nephropathy is usually idiopathic, but it may be associated with hepatitis B and C, solid tumors, SLE, and some drugs (e.g. NSAIDS, penicillamine).

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39
Q

Pathoma Renal

How does membranous nephropathy appear on immunofluorescence?

A

Subepithelial ‘spike and dome’ deposits

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40
Q

Pathoma Renal

How does membranous nephropathy appear on H&E?

A

Thick glomerular basement membrane

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41
Q

Pathoma Renal

How does membranoproliferative glomerulonephritis appear on H&E?

A

Thick glomerular basement membrane;

‘tram-track’ appearance

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42
Q

Pathoma Renal

Both membranous nephropathy and membranoproliferative glomerulonephritis present with thickened glomerular basement membranes, but a ‘tram-track’ appearance is only associated with which?

A

Membranoproliferative glomerulonephritis

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43
Q

Pathoma Renal

What antibody is associated with membranous nephropathy?

A

Anti-phospholipase A2 receptor antibodies

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44
Q

Pathoma Renal

Granular, subepithelial, ‘spike and dome’ IF on EM is characteristic of which etiology of nephrotic syndrome?

A

Membranous nephropathy

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45
Q

Pathoma Renal

Granular, subendothelial IF on EM is characteristic of which etiology of nephrotic syndrome?

A

Type I membranoproliferative glomerulonephritis

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46
Q

Pathoma Renal

Granular, intramembranous IF on EM is characteristic of which etiology of nephrotic syndrome?

A

Type II membranoproliferative glomerulonephritis

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47
Q

Pathoma Renal

Type I membranoproliferative glomerulonephritis is associated with ___________ immune complex deposits.

Type II membranoproliferative glomerulonephritis is associated with ___________ immune complex deposits.

A

Type I membranoproliferative glomerulonephritis is associated with subendothelial immune complex deposits.

Type II membranoproliferative glomerulonephritis is associated with intramembranous immune complex deposits (dense deposit disease).

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48
Q

Pathoma Renal

Which form of membranoproliferative glomerulonephritis is associated with HBV and HCV?

A

Type I

(subendothelial deposits)

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49
Q

Pathoma Renal

Which form of membranoproliferative glomerulonephritis is associated with C3 nephritic complement (an autoantibody that stabilizes C3 convertase)?

A

Type II

(intramembranous deposits - dense deposit disease)

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50
Q

Pathoma Renal

Type II (intramembranous) membranoproliferative glomerulonephritis is associated with ___________ complement, an autoantibody that stabilizes ___________.

A

Type II (intramembranous) membranoproliferative glomerulonephritis is associated with C3 nephritic complement, an autoantibody that stabilizes C3 convertase.

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51
Q

Pathoma Renal

Which etiologies of nephrotic syndrome typically respond very poorly to steroids and progress to chronic renal failure?

A

Focal segmental glomerulosclerosis

Membranous nephropathy

Membranoproliferative glomerulonephritis

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52
Q

Pathoma Renal

The nonenzymatic glycosylation of vascular basement membranes seen in DM leads to what form of sclerosis?

Which arteriole is most affected?

A

Hyaline arteriolosclerosis;

efferent

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53
Q

Pathoma Renal

How do the glomeruli appear in patients with DM?

A

Mesangial sclerosis;

Kimmelstiel-Wilson nodules

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54
Q

Pathoma Renal

How can the progress of hyperfiltration-induced damage associated with diabetes mellitus be slowed?

A

ACE inhibitors

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55
Q

Pathoma Renal

True/False.

After the lungs, the kidneys are the most commonly involved organs in systemic amyloidosis.

A

False.

The kidneys are the most commonly involved organs in systemic amyloidosis.

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56
Q

Pathoma Renal

Amyoidosis-induced nephrotic syndrome is characterized by amyloid deposits in what location(s)?

A

The mesangium

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57
Q

Pathoma Renal

After staining with _________, amyloidosis can be seen under polarized light as ___________ birefringence.

A

After staining with congo red, amyloidosis can be seen under polarized light as apple-green birefringence.

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58
Q

Pathoma Renal

Casts in the urine indicate a pathology of which organ(s)?

A

The kidneys only

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59
Q

Pathoma Renal

What is the main sign that a renal disorder is likely a nephritic syndrome (rather than nephrotic)?

A

RBC casts and dysmorphic RBCs in urine

(signs of glomerular inflammation)

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60
Q

Pathoma Renal

Nephritic syndromes are characterized by the presence of urinary ________ casts, _______uria, _______ proteinuria, _______tension, and _______ retention.

A

Nephritic syndromes are characterized by the presence of urinary RBC casts, oliguria, limited proteinuria, hypertension, and salt retention.

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61
Q

Pathoma Renal

Which may be characterized by puffy eyes (due to salt retention) and hypertension, nephrotic syndrome or nephritic syndrome or both?

A

Both.

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62
Q

Pathoma Renal

Which is usually characterized by oliguria, nephrotic syndrome or nephritic syndrome or both?

A

Nephritic syndrome

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63
Q

Pathoma Renal

Describe the proteinuria associated with nephritic syndrome.

A

Limited;

< 3.5 g / day

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64
Q

Pathoma Renal

What would renal biopsy show in a patient with a nephritic syndrome?

A

Hypercellular, inflammed glomeruli

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65
Q

Pathoma Renal

What is the main etiology for all the forms of nephritic syndrome?

A

Immune-complex deposition

(and C5a activation attracting neutrophils)

66
Q

Pathoma Renal

What is the most common nephropathy worldwide?

A

IgA nephropathy

(Berger disease - a form of nephritic syndrome)

67
Q

Pathoma Renal

Describe the classic presentation for IgA nephropathy (Berger disease).

A

Hematuria (gross or microscopic) with RBC casts following some form of mucosal infection (e.g. a gastroenteritis)

(usually in an individual between their teens and early 30s)

68
Q

Pathoma Renal

Why are mucosal infections associated with IgA nephropathy (Berger disease)?

A

Mucosal infections ramp up IgA production

69
Q

Pathoma Renal

Does IgA nephropathy (Berger disease) cause any immunofluorescence findings?

A

Yes;

mesangial immune-complex deposition

70
Q

Pathoma Renal

True/False,

IgA nephropathy is known to sometimes slowly progress to renal failure.

A

True.

71
Q

Pathoma Renal

Name the nephritic syndrome caused by inherited defects in type IV collagen.

Name the nephritic syndrome caused by acquired autoantibodies to type IV collagen.

A

Alport syndrome

Goodpasture syndrome

72
Q

Pathoma Renal

What, if any, effect does Alport syndrome have on the glomerular basement membrane?

A

Thinning and splitting

73
Q

Pathoma Renal

Describe the major presentation of Alport syndrome.

A

Isolated hematuria;

sensory hearing loss;

ocular disturbances

(‘can’t see, bloody pee, can’t hear a bee’)

74
Q

Pathoma Renal

Name a nephritic syndrome that typically follows infection and that progresses to rapidly progressive glomerulonephritis in some cases.

A

Poststreptococcal glomerulonephritis

75
Q

Pathoma Renal

1% of pediatric cases of poststreptococcal glomerulonephritis progress to what?

25% of adult cases of poststreptococcal glomerulonephritis progress to what?

A

Renal failure;

rapidly progressive glomerulonephritis

76
Q

Pathoma Renal

True/False.

25% of pediatric cases of poststreptococcal glomerulonephritis progress to full-blown renal failure.

1% of pediatric cases of poststreptococcal glomerulonephritis develop into a rapidly progressive glomerulonephritis.

A

False.

1 % of pediatric cases of poststreptococcal glomerulonephritis progress to full-blown renal failure.

25 % of adult cases of poststreptococcal glomerulonephritis develop into a rapidly progressive glomerulonephritis.

77
Q

Pathoma Renal

Poststreptococcal glomerulonephritis typically results from ______genic strains of S. pyogenes but may also result from non___________ organisms as well.

A

Poststreptococcal glomerulonephritis typically results from nephritogenic strains of S. pyogenes but may also result from nonstreptococcal organisms as well.

78
Q

Pathoma Renal

Poststreptococcal glomerulonephritis is typically seen in _________ (children/adults) but may also be seen in _________ (children/adults).

A

Poststreptococcal glomerulonephritis is typically seen in children but may also be seen in adults.

79
Q

Pathoma Renal

How long after a pharyngeal or cutaneous infection with group A streptococcus will development of poststreptococcal glomerulonephritis usually arise?

A

2 - 3 weeks

80
Q

Pathoma Renal

Describe the appearance on EM, if any change from normal, of the immune-complex deposits seen in poststreptococcal glomerulonephritis.

A

Granular immunofluorescence in subepithelial‘humps’

81
Q

Pathoma Renal

What is the treatment for poststreptococcal glomerulonephritis?

A

Supportive

82
Q

Pathoma Renal

A patient presents with fibrin/macrophage crescent structures in their Bowman capsules.

What form of renal pathology does this indicate?

A

Rapidly progressive glomerulonephritis

83
Q

Pathoma Renal

How long does it typically take for rapidly progressive glomerulonephritis to develop into renal failure?

A

Weeks to months

84
Q

Pathoma Renal

A patient presents with fibrin/macrophage crescent structures in their Bowman capsules, indicating rapidly progressive glomerulonephritis.

What can be used to differentiate the many different etiologies?

A

Immunofluorescence

85
Q

Pathoma Renal

A patient presents with fibrin/macrophage crescent structures in their Bowman capsules, indicating rapidly progressive glomerulonephritis.

You note linear immunofluorescence. Name the most likely​ potential etiology(ies).

A

Goodpasture syndrome

86
Q

Pathoma Renal

A patient presents with fibrin/macrophage crescent structures in their Bowman capsules, indicating rapidly progressive glomerulonephritis.

You note granular immunofluorescence. Name the most likely potential etiology(ies).

A

(1) Poststreptococcal glomerulonephritis (most common)
(2) Diffuse proliferative glomerulonephritis (seen in SLE)

87
Q

Pathoma Renal

A patient presents with fibrin/macrophage crescent structures in their Bowman capsules, indicating rapidly progressive glomerulonephritis.

You note pauci-immune immunofluorescence. Name the most likely potential etiology(ies).

A

(1) Granulomatosis with polyangiitis
(2) Microscopic polyangiitis
(3) Churg-Strauss

88
Q

Pathoma Renal

What does it mean that the renal immunofluorescence associated with granulomatosis with polyangiitis, microscopic polyangiitis, and Churg-Strauss are all pauci-immune?

A

It means there is no immunofluorescence

89
Q

Pathoma Renal

What form of renal disease is most common in patients with SLE?

A

Diffuse proliferative glomerulonephritis

(a form of rapidly progressive glomerulonephritis)

90
Q

Pathoma Renal

Identify the immunofluorescence pattern for each of the following etiologies of rapidly progressive glomerulonephritis:

Microscopic polyangiitis

Goodpasture syndrome

Diffuse proliferative glomerulonephritis

A

Negative (pauci-immune)

Linear

Granular (usually sub-endothelial)

91
Q

Pathoma Renal

Identify the immunofluorescence pattern for each of the following etiologies of rapidly progressive glomerulonephritis:

Granulomatosis with polyangiitis

Poststreptococcal glomerulonephritis

Churg-Strauss

A

Negative (pauci-immune)

Granular

Negative (pauci-immune)

92
Q

Pathoma Renal

Identify each of the following renal pathologies as either nephrotic or nephritic syndromes:

Alport syndrome

Membranous nephropathy

Poststreptococcal glomerulonephritis

A

Nephritic

Nephrotic

Nephritic

93
Q

Pathoma Renal

Identify each of the following renal pathologies as either nephrotic or nephritic syndromes:

Minimal change disease

Membranoproliferative glomerulonephritis

IgA nephropathy

A

Nephrotic

Nephrotic

Nephritic

94
Q

Pathoma Renal

Identify each of the following renal pathologies as either nephrotic or nephritic syndromes:

Focal segmental glomerulosclerosis

Systemic amyloidosis

Rapidly progressive glomerulonephritis

A

Nephrotic

Nephrotic

Nephritic

95
Q

Pathoma Renal

Identify each of the following pathologies as being more associated with either nephrotic or nephritic syndromes:

SLE

HIV

Streptococcus pyogenes

A

Nephritic or nephrotic (usually diffuse proliferative glomerulonephritis; may also be membranous nephropathy)

Nephrotic (focal segmental glomerulosclerosis)

Nephritic (poststreptococcal glomerulonephritis)

96
Q

Pathoma Renal

Identify each of the following pathologies as being more associated with either nephrotic or nephritic syndromes:

Diabetes mellitus

Hepatitis B

Sickle cell disease

A

Nephrotic

Nephrotic (membranous nephropathy; membranoproliferative glomerulonephritis)

Nephrotic (focal segmental glomerulosclerosis)

97
Q

Pathoma Renal

Identify each of the following pathologies as being more associated with either nephrotic or nephritic syndromes:

Hepatitis C

Solid tumors

Mucosal infections

A

Nephrotic (membranous nephropathy; membranoproliferative glomerulonephritis)

Nephrotic (membranous nephropathy)

Nephritic (IgA nephropathy)

98
Q

Pathoma Renal

Identify each of the following pathologies as being more associated with either nephrotic or nephritic syndromes:

Goodpasture syndrome

Alport syndrome

Churg-Strauss

A

Nephritic (rapidly progressive glomerulonephritis)

Nephritic

Nephritic (rapidly progressive glomerulonephritis)

99
Q

Pathoma Renal

True/False.

A patient presenting with hematuria almost certainly has a nephritic syndrome.

A

False.

This could be a urinary tract tumor, kidney stone, polycystic kidney disease, BPH, renal cyst(s), sickle cell disease, hydronephrosis, pyelonephritis (if fever present), benign familial hematuria, idiopathic hematuria, etc.

100
Q

Pathoma Renal

In cases of cystitis, fever is usually _________.

A

In cases of cystitis, fever is usually absent.

101
Q

Pathoma Renal

Describe the gold standard lab findings for a typical UTI:

Culture —

A

Describe the lab findings in a typical UTI:

Culture — > 100,000 colony-forming units (gold standard)

102
Q

Pathoma Renal

Describe the lab findings in a typical UTI:

Urinalysis —

A

Describe the lab findings in a typical UTI:

Urinalysis — Cloudy urine + > 10 WBCS / hpf

103
Q

Pathoma Renal

Describe the lab findings in a typical UTI:

Dipstick —

A

Describe the lab findings in a typical UTI:

Dipstick — Positive leukocyte esterase + nitrites

104
Q

Pathoma Renal

What urine dipstick result indicates pyuria?

A

Leukocyte esterase

105
Q

Pathoma Renal

True/False.

Urinary dipstick in case of UTIs is will typically show the presence of nitrates.

A

False.

Urinary dipstick in case of UTIs is will typically show the presence of nitrites.

(Bacteria convert nitrates to nitrites.)

106
Q

Pathoma Renal

What urine dipstick result indicates presence of bacteria?

(Why?)

A

Nitrites

(Bacteria convert nitrates to nitrites.)

107
Q

Pathoma Renal

What are the two main etiologies for UTI development?

A

Escherichia coli (80%)

Staphylococcus saprophyticus

108
Q

Pathoma Renal

Name three culture-positive etiologies for UTI besides E. coli (80%) and S. saprophyticus.

A
  • Klebsiella pneumoniae*
  • Proteus mirabilis*
  • Enterococcus faecalis*
109
Q

Pathoma Renal

Name two culture-negative etiologies for UTI (i.e. which bacteria cause pyuria with a negative urine culture?).

A
  • Chlamydia trachromatis*
  • Neisseria gonnorrheae*

(Note: dysuria due to urethritis is a dominant presenting sign.)

110
Q

Pathoma Renal

Name the three most common etiologies of pyelonephritis.

A

E. coli (90%)

  • Enterococcus faecalis*
  • Klebsiella spp.*
111
Q

Pathoma Renal

What condition can lead to ‘thyroidization’ of the kidney(s)?

(I.e. tubules are atrophic and contain eosinophilic proteinaceous material.)

A

Chronic pyelonephritis

112
Q

Pathoma Renal

What is a major instigating factor for chronic pyelonephritis in children?

A

Vesicoureteral reflux

113
Q

Pathoma Renal

What is a major instigating factor for chronic pyelonephritis in adults?

A

Obstruction

(e.g. BPH or cervical carcinoma)

114
Q

Pathoma Renal

What renal scarring pattern is characterstic of the effects of vesicoureteral reflux?

A

Scarring at the upper and lower poles; blunted calyces

115
Q

Pathoma Renal

Risk factors for nephrolithiasis mainly center around a high _________ in the urinary filtrate and a low _________ in the urinary filtrate.

A

Risk factors for nephrolithiasis mainly center around a high [solute] in the urinary filtrate and a low volume in the urinary filtrate.

116
Q

Pathoma Renal

What are the main ways that the pain of nephrolithiasis is described?

A

Colicky,

unilateral,

flank

(in conjunction with hematuria)

117
Q

Pathoma Renal

What is the most common type of renal stone?

A

Calcium (oxalate or phosphate salts)

118
Q

Pathoma Renal

What is the most common cause of calcium oxalate (or phosphate) nephrolithiasis?

What is the treatment?

A

Idiopathic hypercalcuria;

hydrochlorothiazide

119
Q

Pathoma Renal

After calcium (phosphate or oxalate), what are the next two most common types of nephrolithiasis?

A

(1) Calcium (oxalate or phosphate)
(2) Ammonium magnesium phosphate
(3) Uric acid

120
Q

Pathoma Renal

A patient presents with colicky right flank pain and hematuria.

Urinalysis shows radiolucent crystals.

Radiolucence most likely indicates what type of stone?

A

Uric acid nephrolithiasis

121
Q

Pathoma Renal

Identify if each of the following nephrolithiasis is radiopaque or radiolucent:

Calcium (oxalate or phosphate)

Struvite (ammonium magnesium phosphate)

Uric acid

A

Identify if each of the following nephrolithiasis is radiopaque or radiolucent:

Calcium (oxalate or phosphate) - Radiopaque

Struvite (ammonium magnesium phosphate) - Radiopaque

Uric acid - Radiolucent

122
Q

Pathoma Renal

Which form of nephrolithiasis has the strongest association with infection (e.g. due to Proteus vulgaris or Klebsiella spp.)?

A

Struvite (ammonium magnesium phosphate)

123
Q

Pathoma Renal

What is the major predisposing factor for uric acid nephrolithiasis?

A

Hyperuricemia

(e. g. due to gout, leukemia, myeloproliferative disorders)
* (Other factors include hot and arid conditions, low urine volume, and low pH.)*

124
Q

Pathoma Renal

Name the mainstay treatment for each of the following forms of nephrolithiasis:

Calcium (oxalate or phosphate)

Struvite (ammonium magnesium phosphate)

Uric acid

A

Calcium - Hydrochlorothiazide

Struvite - Surgery + antibiotics

Uric acid - Hydration + urine alkalinization (+ allopurinol for gout)

125
Q

Pathoma Renal

Name a rare form of nephrolithiasis most commonly seen in children with a certain genetic defect.

A

Cystine stones

126
Q

Pathoma Renal

Treatment is extremely similar for which two forms of nephrolithiasis?

A

Uric acid stones

Cystine stones

(hydration + urine alkalinization)

127
Q

Pathoma Renal

Describe the microscopic appearance of the following types of nephrolithiasis:

Calcium (phosphate or oxalate)

Struvite (ammonium magnesium phosphate)

A
128
Q

Pathoma Renal

Describe the microscopic appearance of the following types of nephrolithiasis:

Uric acid

Cystine

A
129
Q

Pathoma Renal

Name the three most common causes of end-stage renal disease (i.e. chronic renal failure).

A
  1. Diabetes mellitus
  2. Hypertension
  3. Glomerular disease
130
Q

Pathoma Renal

ESRD treatment includes dialysis and renal transplant. The dialysis often leads to ________ development, increasing risk of subsequent ________ carcinoma.

A

ESRD treatment includes dialysis and renal transplant. The dialysis often leads to cyst development, increasing risk of subsequent renal cell carcinoma.

131
Q

Pathoma Renal

List the major effects of chronic renal failure:

_______emia

_______tension

_______kalemia

Metabolic _____osis

_______calcemia and renal osteo____________

Anemia due to decreased _____________

A

List the major effects of chronic renal failure:

Azotemia

Hypertension

Hyperkalemia

Metabolic acidosis

Hypocalcemia and renal osteodystrophy

Anemia due to decreased erythropoietin

132
Q

Pathoma Renal

List some of the major effects of the azotemia (~uremia) associated with chronic renal failure (ESRD).

(E.g. appetite, inflammatory, hematogenous, and motor effects)

Mnemonic: ESRD makes you NAP-PE.

A

Nausea

Anorexia

Pericarditis

-

Platelet dysfunction

Encephalopathy (and asterixis)

133
Q

Pathoma Renal

Which substance is the main controlling factor over the diameter of the afferent arteriole lumen?

A

PGE2

(Hence, why NSAIDs restrict blood flow to the glomeruli.)

134
Q

Pathoma Renal

Which substance is the main controlling factor over the diameter of the efferent arteriole lumen?

A

Angiotensin II

(Hence, why ACE inhibitors dilate the efferent arteriole.)

135
Q

Pathoma Renal

Angiomyolipomas are ______omas more commonly seen in patients with ____________.

A

Angiomyolipomas are hamartomas more commonly seen in patients with tuberous sclerosis.

136
Q

Pathoma Renal

Renal cell carcinomas arise from what tissue(s)?

A

Renal tubule epithelium

137
Q

Pathoma Renal

What are the three classic S/Sy of which some mix is typically seen in renal cell carcinoma?

A

Hematuria

Palpable mass

Flank pain

138
Q

Pathoma Renal

Name some of the paraneoplastic syndromes associated with renal cell carcinoma.

A

EPO-, renin-, PTHrP-, and ACTH-secreting tumors

139
Q

Pathoma Renal

Renal cell carcinomas are rarely associated with left-sided _________.

A

Renal cell carcinomas are rarely associated with left-sided varicoceles.

140
Q

Pathoma Renal

What are the most common gross and microscopic findings in renal cell carcinomas?

A

Gross - yellow mass

Microscopic - clear cells

141
Q

Pathoma Renal

Loss of VHL leads to increases in what factors?

A

IGF-1

HIF transcription factor (increasing VEGF and PDGF)

142
Q

Pathoma Renal

What is the classic anatomical location and demographic associated with sporadic renal cell carcinoma?

A

Upper renal pole;

60-year-old male

143
Q

Pathoma Renal

What is the major risk factor for sporadic renal cell carcinoma?

A

Cigarette smoke

144
Q

Pathoma Renal

Although renal cell carcinomas are especially at-risk for hematogenous spread (via the renal vein), what lymph nodes are most likely to be involved?

A

The retroperitoneal nodes

145
Q

Pathoma Renal

What tumor arises from renal blastema and is characterized by primitive glomeruli, tubules, and stromal cells?

A

Wilms tumor

146
Q

Pathoma Renal

Wilms tumors are _________ (benign/malignant).

A

Wilms tumors are malignant.

147
Q

Pathoma Renal

True/False.

Renal cell carcinoma is the most common renal malignancy in children.

A

False.

Wilms tumor is the most common renal malignancy in children.

148
Q

Pathoma Renal

What is the classic presentation for Wilms tumor?

A

Unilateral flank mass, hematuria, and hypertension

149
Q

Pathoma Renal

Why do Wilms tumors present with hypertension?

A

Tumor renin secretion

150
Q

Pathoma Renal

Name the three syndromes associated with Wilms tumor.

A

WAGR

Denys-Drash

Beckwith-Wiedmann

(‘What could go wrong dining and dashing at BWs?’)

151
Q

Pathoma Renal

Besides the presence of a nephroblastoma (Wilms tumor), name the main aspects of WAGR syndrome.

A

Aniridia, Genital abnormalities, mental/motor Retardation

152
Q

Pathoma Renal

Besides the presence of a nephroblastoma (Wilms tumor), name the main aspects of Denys-Drash syndrome.

A

Progressive glomerular disease,

male pseudohermaphroditism

(‘double Ds get the PP’)

153
Q

Pathoma Renal

Besides the presence of a nephroblastoma (Wilms tumor), name the main aspects of Beckwith-Wiedmann syndrome.

A

Neonatal hypoglycemia,

muscular hemihypertrophy,

organomegaly

154
Q

Pathoma Renal

Name the respective mutation associated with each of the following syndromes:

WAGR -

Denys-Drash -

Beckwith-Wiedmann -

A

Name the respective mutation associated with each of the following syndromes:

WAGR - WT1 (deletions)

Denys-Drash - WT1 (mutations)

Beckwith-Wiedmann - WT2

155
Q

Pathoma Renal

What is the major risk factor for urothelial (transitional cell) carcinomas?

Name some others.

A

Cigarette smoke;

naphthylamine, azo dyes, long-term cyclophosphamide or phenacetin use

156
Q

Pathoma Renal

Describe the flat form of urothelial (transitional cell) carcinoma.

A

Early p53 mutations:

high-grade, flat tumor with early invasion

157
Q

Pathoma Renal

Describe the papillary form of urothelial (transitional cell) carcinoma.

A

Low-grade papillary tumor that progresses to high-grade and then invades

158
Q

Pathoma Renal

True/False.

Urothelial (transitional cell) carcinomas are often multifocal and recurrent.

A

True.

159
Q

Pathoma Renal

What must occur before squamous cell carcinomas can arise in the bladder or other lower urinary tract locations?

A

Squamous metaplasia

(from transitional epithelia)

160
Q

Pathoma Renal

Name three very distinct risk factors for squamous cell carcinoma of the bladder.

A

Chronic cystitis (older woman)

Schistosoma haematobium (Egyptian male)

Long-standing nephrolithiasis

161
Q

Pathoma Renal

Name three structural risk factors for adenocarcinoma of the bladder.

A

Urachal remnant

Cystitis glandularis

Extrophy of the bladder

S1 - Selected Review (14 decks)

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