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MHD > Nephrotic Syndromes > Flashcards

Nephrotic Syndromes Flashcards

1
Q

What is the typical appearance of urine in nephrotic syndrome?

A

Foamy urine due to proteinuria

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2
Q

What is the hallmark problem in nephrotic syndrome?

A

Proteinuria

Increased permeability of the glomerulus to plasma proteins

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3
Q

In normal physiology, how does the glomerulus prevent certain molecules and proteins from filtration?

A

Filters by size and charge. Particles that are too large will not get through.
Negatively charged proteins will not get through even if they are small enough (like albumin), due to two negatively charged layers on either side of the lamina densa

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4
Q

List some general symptoms of nephrotic syndrome

A

Massive proteinuria (>3.5 g/day)
Hypoalbuminemia
Generalized edema
Hyperlipidemia and lipiduria

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5
Q

What is the mechanism of hypoalbuminemia in nephrotic syndromes?

A

Loss of protein in the urine leads to hypoalbuminemia and a drop in plasma colloid osmotic pressure (causing edema)

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6
Q

What is the mechanism of hyperlipidemia in nephrotic syndromes?

A

Hypoalbuminemia triggers increased synthesis of lipoproteins in liver

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7
Q

What is the mechanism of lipiduria in nephrotic syndromes?

A

Due to the increased permeability of the glomerular basement membrane to lipoproteins

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8
Q

Membranous Nephropathy

Typical Clinical Presentation

A

Edema (ankle, periorbital)
Thrombosis (loss of antithrombin III)
Infections

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9
Q

Membranous Nephropathy

Etiology/Pathogenesis

A

In-situ immune complex formation
Could be due to antibodies against an “intrinsic” renal antigen or a “planted” antigen (that has lodged itself in the basement membrane)

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10
Q

Membranous Nephropathy

Where do the immune complexes have their reaction?

A

Basal surface of the podocytes (the distal zone of the capillary wall)

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11
Q

Membranous Nephropathy

Does it cause inflammation?

A

No!

Immune complex events in the proximal zone of the glomerular capillary wall are much more likely to cause inflammation, but Membranous Nephropathy affects the distal zone.

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12
Q

Membranous Nephropathy

Describe the appearance on silver stain and why.

A

Get a “spike and dome” or “holey” appearance

The podocytes are trying to form a new basement membrane, so they cause the spike and dome appearance

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13
Q

In older patients diagnosed with membranous nephropathy, what should you do in addition to treatment?

A

Rule out possible cancer, which could played a role in the immune complex deposition

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14
Q

What are the granular deposits of Membranous Nephropathy made of?

A

IgG + Complement

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15
Q

Membranous Nephropathy

Prognosis

A

33% spontaneous remission
33% will need dialysis
33% will have proteinuria without renal failure progression

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16
Q

Membranous Nephropathy

Treatment

A

Immunosuppressive drugs
Nonspecific antiproteinuric
Treat the underlying disease
Kidney transplant

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17
Q

Minimal Change Disease

What is the pathological origin?

A

Reversible podocyte injury

Derived from…
T cells, cytokines, immune depression, NSAIDs

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18
Q

Minimal Change Disease

Who is primarily affected?

A

Children 2-6yo

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19
Q

Minimal Change Disease

Clinical Presentation

A

Edema (periorbital, generalized)

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20
Q

Minimal Change Disease
What is seen on Light microscopy? Immunofluorescence?
Electron microscopy?

A

Light microscopy and IF look normal.

EM is most critical
See effacement (fusion) of foot processes
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21
Q

What is a big difference between Minimal Change Disease and Membranous nephropathy?

A

Minimal Change Disease has no immune complex deposition!

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22
Q

Minimal Change Disease

Prognosis

A

Relapsing episodes of nephrotic syndrome

Resolution at puberty

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23
Q

Minimal Change Disease

Treatment

A

Steroids

24
Q

Focal and Segmental Glomerular Sclerosis (FSGS)

Typical Clinical Presentation

A

Nephrotic Syndrome
Higher incidence of hematuria, reduced GFR, HTN
Non selective proteinuria is more common

25
Q

FSGS

What groups is it more common in?

A

African Americans and Hispanics

26
Q

FSGS

Pathogenesis

A

Irreversible injury to podocytes

Some glomeruli (not all) will show an increase in mesangial matrix-like substance, leading to obliteration of the capillaries and sclerosis

See effacement of epithelial foot processes on EM in all glomeruli

27
Q

FSGS

Which glomeruli tend to be involved in the initial stages? As it progresses?

A

Juxtamedullary glomeruli only in initial stages

Global sclerosis, tubular atrophy, and interstitial fibrosis in later stages

28
Q

FSGS

Prognosis

A

Progression to renal failure

29
Q

FSGS

Treatment

A

Initially, may be responsive to steroids though it will become resistant over time.
In kidney transplants, FSGS may recur

30
Q

FSGS

List some of the many etiologies

A

HIV-associated
Parvavirus B19
Heroin
Sickle Cell disease

Steroid use
HTN
Low birth weight
Obesity

31
Q

FSGS

What genetic mutations are commonly involved?

A

Mutations in genes encoding slit diaphragm proteins.

NPHS1 or NPHS2 – play a role in maintaining normal glomerular filtration barrier

32
Q

Which gene is associated with higher risk in adult African American onset of FSGS?

A

Apolipoprotein L1

33
Q

HIV-associated FSGS

What is seen on H&E stain of the glomerulus?

A

“Collapsing FSGS”
No obliteration and solidification of the capillaries
Instead, the glomerulus looks collapsed

34
Q 
Membranoproliferative Glomerulonephritis (MPGN)
Typical Clinical Presentation
A

Nephrotic syndrome na dhematuria

35
Q 
Membranoproliferative Glomerulonephritis (MPGN)
Etiology/Pathogenesis
A

Primary immune complex formation with classic complement activation

Could be secondary to chronic autoimmune disorders, hepatitis, endocarditis, bacterial infections

36
Q 
Membranoproliferative Glomerulonephritis (MPGN)
What is the classic appearance on EM?
A

Glomerular capillary wall has two visible lines
“Tram track” appearance

There are dense irregular dots that are immune complex (IgG+C3) deposits in the subendothelial space

37
Q 
Membranoproliferative Glomerulonephritis (MPGN)
Lab Tests
A

Low complement levels

38
Q 
Membranoproliferative Glomerulonephritis (MPGN)
Prognosis
A

Progress to renal failure

39
Q 
Membranoproliferative Glomerulonephritis (MPGN)
Treatment
A

Treat the underlying disease

40
Q

Dense Deposit Disease (DDD)

Typical Clinical Presentation

A

Nephrotic syndrome with hematuria

41
Q

Dense Deposit Disease (DDD)

Who most commonly gets it?

A

Older children

42
Q

Dense Deposit Disease (DDD)

Pathogenesis

A

Sustained activation of complement via alternative pathway (non-Ab mediated)

Sustained activation of complement is due to stabilized C3 convertase, often due to C3 nephritic factor

43
Q

Dense Deposit Disease (DDD)

What is the appearance on EM?

A

Ribbon-like dense appearance of the lamina densa

44
Q

Dense Deposit Disease (DDD)

What is most commonly the factor that causes sustained complement activation?

A

C3 nephritic factor

45
Q

Dense Deposit Disease (DDD)

Prognosis

A

Progress to renal failure

Recurrence in transplants

46
Q

Diabetes

What is the hallmark lesion/pathology of diabetic nephropathy?

A

Thick glomerular basement membrane due to non-enzymatic glycosylation of the basement membrane.

Therefore, the wall of the membrane is thicker and the kidney lumen is narrower

47
Q

Diabetes

In what vessel does hyaline/protein material tend to accumulate?

A

Efferent arterioles in the kidney

48
Q

Amyloidosis

What is the basic pathological issue?

A

Abnormally folded protein (Beta plated sheets) accumulating outside cells

49
Q

Amyloidosis

What stain should be used for diagnosis?

A

Congo Red stain

50
Q

What kind of malignancy is amyloidosis commonly involved with?

A

Plasma cell malignancy

51
Q

How do you diagnose/detect amyloidosis?

A

Tissue screening (such as a fat biopsy)

Deposits of amyloid may be present in both the kidney and adipose tissue

52
Q

Symptoms of Amyloidosis

A 
Nephrotic syndrome (proteinuria)
Heart involvement (could cause restrictive cardiomyopathy)
Submandibular swelling
Raccoon eyes
Macroglossia
53
Q

SLE

Who is most commonly affected?

A

Young women

54
Q

SLE

What is the basic pathological problem?

A

Chronic formation of circulating immune complexes in blood

55
Q

SLE

What is a kidney biopsy used for in SLE?

A

Staging of SLE

If the kidney is badly affected with diffuse proliferative necrosis, then treat that patient more aggressively

MHD (57 decks)

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