Renal - Nephrotic Syndromes Flashcards

1
Q

Name the major (1) signs, (2) serum changes, and (3) urine changes associated with nephrotic syndrome.

A

(1) Edema

(2) Hypoalbuminemia and hyperlipidemia

(3) Proteinuria

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2
Q

Nephrotic syndromes are characterized by a proteinuria of ___ 3.5 g/day.

A

Nephrotic syndromes are characterized by a proteinuria of > 3.5 g/day.

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3
Q

__________ syndrome is characterized by the following:

(1) Hypoalbuminemia
(2) Hypogammaglobulinemia
(3) Hypercoagulable state
(4) Hyperlipidemia and hypercholesterolemia

A

Nephrotic syndrome is characterized by the following:

(1) Hypoalbuminemia
(2) Hypogammaglobulinemia
(3) Hypercoagulable state
(4) Hyperlipidemia and hypercholesterolemia

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4
Q

Nephrotic syndrome is characterized by the following:

(1) Hypo_________emia
(2) Hypo_________emia
(3) Hypercoagulable state
(4) Hyperlipidemia and hypercholesterolemia

A

Nephrotic syndrome is characterized by the following:

(1) Hypoalbuminemia
(2) Hypogammaglobulinemia
(3) Hypercoagulable state
(4) Hyperlipidemia and hypercholesterolemia

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5
Q

Nephrotic syndrome is characterized by the following:

(1) Hypoalbuminemia
(2) Hypogammaglobulinemia
(3) Hyper_________ state
(4) Hyper_________emia and hyper_________emia

A

Nephrotic syndrome is characterized by the following:

(1) Hypoalbuminemia
(2) Hypogammaglobulinemia
(3) Hypercoagulable state
(4) Hyperlipidemia and hypercholesterolemia

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6
Q

What causes the S/Sy associated with nephrotic syndrome?

(I.e. hypoalbuminemia, hypogammaglobulinemia, hypercoagulable state, hyperlipidemia, and hypercholesterolemia)

A

Proteinuria due to a variety of glomerular disorders

(> 3.5 mg/day)

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7
Q

The following diseases are six causes of _________ _________:

(1) Minimal change disease
(2) Focal segmental glomerulosclerosis
(3) Membranous nephropathy
(4) Membranoproliferative glomerulonephritis
(5) Diabetes mellitus
(6) Systemic amyloidosis

A

The following diseases are six causes of nephrotic syndrome:

(1) Minimal change disease
(2) Focal segmental glomerulosclerosis
(3) Membranous nephropathy
(4) Membranoproliferative glomerulonephritis
(5) Diabetes mellitus
(6) Systemic amyloidosis

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8
Q

Name six causes of nephrotic syndrome:

(1) _________ _________ disease
(2) Focal ___________ _____________
(3) Membranous nephropathy
(4) Membranoproliferative glomerulonephritis
(5) Diabetes mellitus
(6) Systemic amyloidosis

A

Name six causes of nephrotic syndrome:

(1) Minimal change disease
(2) Focal segmental glomerulosclerosis
(3) Membranous nephropathy
(4) Membranoproliferative glomerulonephritis
(5) Diabetes mellitus
(6) Systemic amyloidosis

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9
Q

Name six causes of nephrotic syndrome:

(1) Minimal change disease
(2) Focal segmental glomerulosclerosis
(3) ____________ _______pathy
(4) ____________ ____________
(5) Diabetes mellitus
(6) Systemic amyloidosis

A

Name six causes of nephrotic syndrome:

(1) Minimal change disease
(2) Focal segmental glomerulosclerosis
(3) Membranous nephropathy
(4) Membranoproliferative glomerulonephritis
(5) Diabetes mellitus
(6) Systemic amyloidosis

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10
Q

Name six causes of nephrotic syndrome:

(1) Minimal change disease
(2) Focal segmental glomerulosclerosis
(3) Membranous nephropathy
(4) Membranoproliferative glomerulonephritis
(5) _________ _________
(6) Systemic _________

A

Name six causes of nephrotic syndrome:

(1) Minimal change disease
(2) Focal segmental glomerulosclerosis
(3) Membranous nephropathy
(4) Membranoproliferative glomerulonephritis
(5) Diabetes mellitus
(6) Systemic amyloidosis

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11
Q

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

A

Minimal change disease

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12
Q

Minimal change disease is the most common cause of nephrotic syndrome in children. It is typically ___________ and is associated with __________ disease as well.

A

Minimal change disease is the most common cause of nephrotic syndrome in children. It is typically idiopathic and is associated with Hodgkin’s disease as well.

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13
Q

How do glomeruli on H&E appear in minimal change disease?

A

Normal

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14
Q

In minimal change disease, lipids can be found in what part(s) of the kidney?

A

The proximal tubule cells

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15
Q

What is noticable on electron microscopy in cases of minimal change disease?

A

Effacement of foot processes

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16
Q

How do the glomeruli appear on immunoflorescence in patients with minimal change disease?

A

No change

(no immune complex deposits)

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17
Q

True/False.

Minimal change disease presents with both albuminuria and hypogammaglobulinemia.

A

False.

Minimal change disease presents with albuminuria but no hypogammaglobulinemia (selective proteinuria).

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18
Q

Minimal change disease shows what changes on imaging?

A

Lipids in proximal tubule cells (light microscopy);

podocyte process effacement (electron microscopy)

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19
Q

How is minimal change disease treated?

How does it typically respond to treatment?

A

Steroids;

excellent response

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20
Q

The renal damage seen in minimal change disease is mediated by what cell type?

A

T cells

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21
Q

What is the most common cause of nephrotic syndrome in Hispanics and African-Americans?

A

Focal segmental glomerulosclerosis

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22
Q

Focal segmental glomerulosclerosis is usually ___________ but may be asssociated with HIV, heroin use, and sickle cell disease.

A

Focal segmental glomerulosclerosis is usually idiopathic but may be asssociated with HIV, heroin use, and sickle cell disease.

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23
Q

Focal segmental glomerulosclerosis is usually idiopathic but may be asssociated with _____, ________ use, and _________ ______ disease.

A

Focal segmental glomerulosclerosis is usually idiopathic but may be asssociated with HIV, heroin use, and sickle cell disease.

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24
Q

How does focal segmental glomerulosclerosis appear on microscopic exam with H&E stain?

A

Focal (some glomeruli) and segmental (involving only part of the glomerulus) sclerosis

(shocking)

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25
Q

What is noticeable on electron microscopy in cases of focal segmental glomerulonephritis?

A

Effacement of foot processes

26
Q

How do the glomeruli appear on immunoflorescence in patients with focal segmental glomerulonephritis?

A

No change

(no immune complex deposits)

27
Q

How is focal segmental glomerulosclerosis disease treated?

A

Steroids

28
Q

How does the renal damage in focal segmental glomerulosclerosis respond to steroid treatment?

A

Poorly

(progresses to chronic renal failure)

29
Q

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

A

Membranous nephropathy

30
Q

Membranous nephropathy is usually ___________ but may be asssociated with hepatitis B, hepatitis C, solid tumors, SLE and drugs (e.g. NSAIDs, penicillamine).

A

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

31
Q

Membranous nephropathy is usually idiopathic but may be asssociated with hepatitis ___, hepatitis ___, solid ______, SLE and drugs (e.g. ______, ______).

A

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

32
Q

How does membranous nephropathy appear on microscopic exam with H&E stain?

A

Thick glomerular basement membrane

33
Q

How do the glomeruli appear on immunoflorescence in patients with membranous nephropathy?

A

Subepithelial deposits

(‘spike and dome’ appearance)

34
Q

How is membranous nephropathy treated?

A

Steroids

35
Q

How does the renal damage in membranous nephropathy respond to steroid treatment?

A

Poorly

(progresses to chronic renal failure)

36
Q

Which causes of nephrotic syndrome are associated with immune complex deposits and will immunofluoresce on microscopy?

A

Membranous nephropathy;

membranoproliferative glomerulonephritis

37
Q

Name the respective diseases that are the most common causes of nephrotic syndrome associated with each of the following demographics:

Children

African-Americans

Caucasians

Hispanics

A

Minimal change disease

Focal segmental glomerulonephritis

Membranous nephropathy

Focal segmental glomerulonephritis

38
Q

How do glomeruli appear on microscopy with H&E in patients with membranoproliferative glomerulonephritis?

A

Thickened glomerular basement membrane

(‘tram-track’ appearance)

39
Q

Type I membranoproliferative glomerulonephritis results in what form of immune deposits?

A

Subendothelial

(associated with hepatitis B and hepatitis C)

40
Q

Type II membranoproliferative glomerulonephritis results in what form of immune deposits?

A

Intramembranous

(associated with C3 nephritic factor — C3 overactivation and depletion)

41
Q

Type ___ membranoproliferative glomerulonephritis results in subendothelial immune deposits and is associated with hepatitis B and hepatitis C.

A

Type I membranoproliferative glomerulonephritis results in subendothelial immune deposits and is associated with hepatitis B and hepatitis C.

42
Q

Type ___ membranoproliferative glomerulonephritis results in intramembranous immune deposits (associated with C3 nephritic factor — C3 overactivation and depletion).

A

Type II membranoproliferative glomerulonephritis results in intramembranous immune deposits (associated with C3 nephritic factor — C3 overactivation and depletion).

43
Q

How is membranoproliferative glomerulonephritis treated?

A

Steroids

44
Q

How does the renal damage in membranoproliferative glomerulonephritis respond to steroid treatment?

A

Poorly

(progresses to chronic renal failure)

45
Q

Which etiologies of nephrotic syndrome respond well to steroid treatment?

A

Minimal change disease only

46
Q

Which etiologies of nephrotic syndrome typically do not respond well to steroid treatment and eventually progress to chronic renal failure?

A

Focal segmental glomerulosclerosis;

membranous nephropathy;

membranoproliferative glomerulonephritis

47
Q

Nephrotic syndrome is associated with what two systemic disorders?

A

Diabetes mellitus;

amyloidosis

48
Q

Diabetes mellitus is associated with nephrotic syndrome.

High serum glucose levels lead to _______ _______ of the vascular basement membrane, resulting in _______ arteriolosclerosis.

A

Diabetes mellitus is associated with nephrotic syndrome.

High serum glucose levels lead to nonenzymatic glycosylation of the vascular basement membrane, resulting in hyaline arteriolosclerosis.

49
Q

Which portion of the glomerular vasculature is most affected by nonenzymatic glycosylation in patients with diabetes mellitus?

A

Efferent arteriole

(> afferent arteriole; resulting in an increased GFR)

50
Q

What is the characteristic urine finding of diabetes mellitus which results from hyperfiltration (following nonenzymatic glycosylation of the vascular basement membrane)?

A

Microalbuminuria

51
Q

The kidney damage associated with diabetes mellitus can eventually progress to _________ syndrome.

A

The kidney damage associated with diabetes mellitus can eventually progress to nephrotic syndrome.

52
Q

How does the kidney appear on microscopy in diabetic patients that have progressed to nephrotic syndrome?

A

(1) Mesangial sclerosis
(2) Formation of Kimmelstiel-Wilson nodules

53
Q

What medication type can slow the progression of hyperfiltration-induced renal damage in diabetic patients?

A

ACE inhibitors

54
Q

Which organ is the most commonly affected in patients with systemic amyloidosis?

A

The kidneys

55
Q

Systemic amyloidosis leads to amyloid deposits in the _________ of the kidneys, leading to nephrotic syndrome.

A

Systemic amyloidosis leads to amyloid deposits in the mesangium of the kidneys, leading to nephrotic syndrome.

56
Q

How do congo-red-stained amyloid deposits in the kidney appear under polarized light in patients with systemic amyloidosis (progressing to nephrotic syndrome)?

A

Apple-green birefringence

57
Q

In patients with systemic amyloidosis, renal amyloid deposits in the mesangium show apple-green birefringence under ________ light after staining with ______ ______.

A

In patients with systemic amyloidosis, renal amyloid deposits in the mesangium show apple-green birefringence under polarized light after staining with congo red.

58
Q

Anti-phospholipase A2 receptor antibodies are associated with what renal disorder?

A

Membranous nephropathy

59
Q

Cresecent formation in glomeruli is characteristic of what category of renal disease?

A

Rapidly progressive glomerulonephritis

60
Q

What glomerular disease is associated with hepatitis B, hepatitis C, and some solid tumors?

A

Membranous nephropathy