Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Renal - Gupta > Nephrotic Syndrome II > Flashcards

Nephrotic Syndrome II Flashcards

1
Q 
What are some KEY findings in the following categories that help you to know you're dealing with someone with MINIMAL CHANGE DISEASE? 
• Hx
• Physical Exam
• Blood Tests 
• Urinalysis
A

Hx:
• Recent onset Facial and Lower Extremeity Edema

Physical Exam:
• PITTING EDEMA (3+)

Blood Tests:
• LOW serum albumin
• Normal BUN and Creatinine
• Negative Antibody Tests

Urinalysis:
• 4+ Proteinuria
• Urine Protein Creatinine ratio = 18 (very high)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Minimal Change Disease, describe the following:
• Age Distribution
• Blood Pressure

A

Bimodal Age Distribution
• Very Young and Very Old ppl. get it

***MOST COMMON cause of nephrotic syndrome in children

Blood Pressure:
• Normotensive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are the Primary and Secondary causes of Minimal Change Disease?

A

Primary:
• Ideopathic

Secondary:
• Malignancy: Hodkin’s Lymphoma

• Drugs: NSAIDs, interferon alpha

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Why would you not get a biopsy with minimal change disease?

A

If a child has nephrotic syndrome you can almost just bank on it being this

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

How is Minimal Change Disease Treated?

• two general tactics.

A

Supportive Measures:
• ACE I’s/ARB
• Treat Hyperlipidemia

Disease Modifiers:
• Oral Glucocorticoid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Why would you give people with Minimal Change Disease an ACE I or ARB despite the fact that they do not have HTN?

A

Lowering the Blood Pressure even in the absence of HTN is useful because it decreases proteinuria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is the Disease Modifying drug used in Minimal Change Disease?
• Response between two age groups that typically are Dx with MCD?
• chance of recurrence?

A

Children:
• More than 90% have an Excellent Response to Steroids

Adults:
•Respond much more slowly to steroids

Recurrence Rate is HIGH in minimal change disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What should you start thinking if you have a kid with minimal change disease that doesn’t respond to steroids?

A

• Consider FOCAL SEGEMENTAL GLOMERULOSCLEROSIS

Kids are expected to respond well to steroids if they have Minimal change disease (should respond within a few weeks)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

T or F: Focal Segmental Glomerulosclerosis is more associated with childhood disease

A

False, FSGS is more associated with Adulthood Disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q 
Focal Segmental Glomerulosclerosis, key features in:
• Hx
• Physical Examination
• Blood Tests
• Urine Analysis
A

Hx:
• Lower Extremity Edema

Physical Exam:
• HYPERTENSIVE
• Pitting edema

Blood Tests:
• HIGH creatinine
• HIGH albumin

Urine Analysis
• 4+ proteinuria
• HIGH urine protein creatinine ratio

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is THE MOST IMPORTANT THING TO KNOW about Focal Segmental Glomerulosclerosis?

A

• 50% of pts. with FSGS will progress to end-stage kidney disease within 10 years of Dx.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

FSGS (focal segmental glomerulosclerosis)
• Proteinuria?
• HTN?
• Renal Function?

A

Proteinuria:
• NOT selective in contrast to MCD (MCD proteinuria is albumin only)

HTN:
• Often Seen with FSGS

Renal Function:
• Compromised (aka you will have a high serum creatinine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is suPAR?

• what is is?

A

suPAR = antibody that binds INTEGRIN so that PODOCYTE can’t binds to the GBM

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are the primary and secondary causes of Focal Segmental Glomerulosclerosis?
• 4 secondary causes

A

Primary:
• Idiopathic

Secondary: 
Mutations in genes of the Glomerular Basement Membrane
• Alpha-actin-4
• Podocin
• TRCP6
• APOLIPOPROTEIN L1 gene

Infection
• HIV
• Parvo Virus

Drugs:
• Pamidronate
• Heroin
• Lithium

Loss of part of the kidney (causing more strain on the remaining glomeruli)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

T or F: mutations in Podocin and Nephrin are mild and typically do not become apparent until much later in life.

A

FALSE, mutations in Podocin and Nephrin usually are apparent at birth

Most other mutations cause FSGS in an adult

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is the only protein involved in Barrier formation of podocytes that will not cause a recurrence of FSGS in transplantation?

A

Alpha-Actin 4, most other mutations will recur

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What protein is responsible for the increased risk of FSGS and renal failure in individuals of African Descent?
• what chromosome is it found on?
• what is the point of this gene mutation?

A

Apolipoprotein L1 gene

  • Chromosome 22
  • APOL1 mutation prevents resistance by SRA protein of Tryponosoma T brucie rhodesience (sleeping sickness)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is the pattern of glomerular involvement in Focal Segemental Glomerulosclerosis?
• Key Features to look for on H and E?

A

Focal - only some glomeruli involved

Segmental - only some lobules within each glomerulus are involved

Key Features:
•Increased Mesangial Matrix

  • Obliterated Capillary Lumina
  • Deposition of hyaline masses and lipid droplets
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What is the problem with diagnosing Focal Segmental Glomerulosclerosis off of a biopsy?

A

A person may have FSGS BUT you can miss it because of the FOCAL nature of the disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What will you see on immunofluorescence and EM with FSGS?

• which is more important in diagnosis?

A

IF:
• IgM and C3 may stain positive and get trapped in the GBM but this is NOT PATHOGENIC and not that imp. in dx.

EM
• **SEVERE PODOCYTE FOOT PROCESS EFFACEMENT
• occlusion of capillaries and endocapillaries
• Hyaline Deposits

21
Q

What two subtypes of FSGS involve heavy proteinuria?
• which has the worst prognosis?
• Which as the best prognosis?

A

Collapsing - (11% of pts) WORST RENAL SURVIVAL

Tip - (17% of pts.) more likely to obtain remission

Ironic b/c these are the only two types that involve proteinuria*

22
Q

What does the Collapsing Type of FSGS look like on H and E?

A

• Crushed Glomerulus similar to Crescentric but in collapsing the cellular expansion extends between glomerular lobules

23
Q

Compare and Contrast Immunofluorescence properties of Minimal Change Disease and FSGS.

A

BOTH:
• Stain Negative on IF

FSGS - may have some non-specific staining with IgM that deposits in the scar tissue

24
Q

What are the treatment methods for FSGS?

• two aspects from which the disease is treated.

A
  1. Supportive Measures
    • Control BP - ACE Is/ARB
    • Treat Hyperlipidemia
  2. Disease Modifiers
    • Corticosteroids
    • Calcineurin Inhibitors (Cyclosporin or Tacrolimus)
25
Q

How do the treatment measures for FSGS differ from minimal change disease?

A

Calcineurin inhibitors like CYCLOSPORIN and TACROLIMUS are implemented in the treatment of FSGS but these chemo. drugs are NOT used in MCD (minimal change disease)

Note: the response to cyclosporin etc. is still not that great

26
Q

Apply the following terms to Minimal Change Disease and Focal Segmental Glomerular Sclerosis:
• Good/Bad Px.
• (non)/Steroid Sensitive NS

A

Minimal Change Disease:
• Good Px.
• Steroid Sensitive

Focal Segemental Glomerular Sclerosis:
• Bad Px.
• Steroid Resistant

27
Q

T or F: MCD may just be an early form of FSGS.

A

True, these disease are probably part of the same disease spectrum as seen in looking at children who progress to FSGS from MCD

28
Q

What two type of Nephrotic Syndrome are associated with Subepithelial Immune complex deposits?

A
  1. Membranous Nephropathy

2. Post-infectious Glomerulonephrtitis

29
Q

Membranous Nephropathy:
• Who is this mostly seen in?
• Prognosis?

A

Membranous Nephropathy Seen in:
• WHITE CAUCASION MALES between 40 and 60 y/o

Px:
• 30% of cases spontaneously resolve

• 40% progress to renal failure

30
Q

What should you do if you have a pt. that is 60 or 70 and is found to have a membranous nephropathy?

A

Send them for common cancer screenings such as LUNG, COLON Cancer, and Melanoma

this is because cancer is often a secondary cause of Membranous Nephropathy

31
Q

What are the categories of Primary and Secondary Membranous Nephropathies?

A

Primary: always idiopathic

Secondary:
• Infection: Hep B, Syphilis, Malaria
• Autoimmune: SLE
• Drugs: Penicillamine, NSAIDs, Gold
• Malignancy: Lung cancer, Colon cancer, Melanoma

*also associated with sickle cell

32
Q

Compare the two theories of immune complex deposition in Membranous Nephropathy and the processes/disease that are thought to follow these patterns.

A

Filtered Antigen Theory:
• Circulating Ab. it deposited in the Subepithelial Space (after passing through the endothelium)
• POST-STREPTOCOCCAL GLOMERULONEPHRITIS is believed to work this way

AUTOIMMUNITY model:
• Auto antibody forms to a locally generated antigen

*Important concept: this is IN SITU formation of immune complexes AFTER they make it through the GBM, otherwise they are too large to pass through

33
Q

What are frequently the Targets of autoanibodies generated in Membranous Nephropathy?
• are these associated with congenital or primary MN?

A
  1. M-type phospholipase A2 receptor
    - -> PRIMARY MN
  2. Neutral Endopeptidase
    - -> Congenital MN
34
Q

What two types of membranous nephropathy are associated with the autoimmunity model?

A

Targets of Autoimmunity:
• M-type Phospholipase A2 receptor (primary MN)

• Neutral Endopeptidase (congenital)

35
Q

Describe the 5 steps in In-Situ Immune Complex formation in Membranous Nephropathy.

A
  1. Free IgG binds to Clatherine coated parts of foot processes
  2. IgG induces MAC formation
  3. (a) Antigen-antibody compexes are shed to form subepithelial deposits (b) C5b-9 are sent to the urinary space
  4. Podocytes (gomerular epithelium) responds to C5b-9 by releasing oxidants and proteases in
  5. Damage to Podocytes allows for increased protein passage through slits and Effacement of podocytes
36
Q

If you were to test someone with IDEOPATHIC MEMBRANOUS NEPHROPATHY for an autoantibody, which would you choose?
• where is the target expressed?
• type of antibody?

A
  • PLA2R autoantibody is present in 70% of pts. with M type phospholipase A2
  • Expressed in PODOCYTES in normal human glomeruli
  • IgG4 = autoantibody type
37
Q

Do you ever see PLA2R in patients with secondary membranous nephropathy?
• where is the damage done in membranous nephropathies?

A

PLA2R:
• NO - this is found in patients with PRIMARY IDEOPATHIC Membranous Nephropathy

• Damage in membranous Nephropathy = Subendothelial

38
Q

What are 6 risk factors for loss of renal function in membranous Nephropathy?

A
  1. Male Gender
  2. Over 10g/day of proteinuria
  3. HTN
  4. Azotemia
  5. Tubulointerstitial Fibrosis
  6. Glomerulosclerosis
39
Q

What are some key features to look for on LM, IF, and EM in membranous Nephropathy?

A

LM:
• SPIKES on silver STAIN

IF:
• Granular along GBM

EM:
• SPIKE and DOME appearance of Subepithelial Deposits along GBM

40
Q

T or F: in membranous nephropathy the capillary loops are thickened and prominent and the cellularity is increased

A

FALSE, cellularity is NOT increased in the membranous nephropathy

41
Q

What is the typically treatment for membranous nephropathy?
• what happens if this treatment fails?
• Why do we treat is this way?

A

Treat this way because 30% of cases Regress Spontaneously

IF proteinuria is LESS than 4gm/day:
• give ACEI or ARB to get BP below 125/75

IF proteinuria is GREATER than 4gm/day:
• give ACEI or ARB to get BP below 125/75

IF on ACEI or ARB and Proteinuria remains above 4 gm/day:
• USE CYTOTOXIC agents - Steroids or Calcineurin inhibitors

42
Q 
KEY features of Post-Infectious Glomerulonephritis? 
• Hx
• Physical Exam.
• Blood Tests
• Urine Analysis
A

Hx:
• Dark Colored Urine
• Wt. Gain
• Decreased Urine output

Physical:
• HYPERTENSION
• pitting edema

Blood Tests:
•HIGH creatinine
• LOW albumin

Urine Analysis:
• 3+ proteinuria
• Urine Protein Creatinine ratio = 3.6 (high but not in nephrotic range)

43
Q

How will a patient with Post-Infectious Glomerulonephritis typically present?

A

Pt. who has recently cleared an UPPER RESPIRATORY TRACT infection that presents with HYPERTENSION and COLA Colored URINE. They will show ASCITES, Peripheral Edema and signs of fluid retention

44
Q

What key immunological studies to lood for in POST-INFECTIOUS GLOMERULONEPHRITIS?
• what do these indicate?
• How will you select the test based on the preceding infection?

A

LOW C3 and Normal C4 (normal C4 indicates that ALTERNATIVE complement cascade is activated)

THROAT INFECTION:
• anti-streptolysin O (ASO)

SKIN INFECTION:
• Anti-DNAse B titers

SEPSIS:
• positive blood culture

45
Q

What is the typical appearance of Glomeruli in Post-streptococcal GN on H and E?
• what physiologic problem is caused by these changes?

A
  • Glomeruli are Globally and diffusely enlarged and HYPERCELLULAR due to NEUTROPHILS, MACROPHAGES, MESANGIAL and ENDOTHELIAL proliferation
  • INFLAMMATION causes obstruction of Capillary Lumen
46
Q

What will you see on immunofluorescence of someone who has a Nehpritis following clearance of a recent infection?
• what causes this appearance?

A

this is POST-STREPTOCOCCAL GLOMERULONEPHRITIS

Appearance:
• Diffuse granular deposits in MESANGIUM and CAPILLARY walls

Stain will be positive for C3 and IgG Granular Appearance b/c this is a SUBENDOTHELIAL disease

47
Q

What do you expect to see on EM for patients that have hematuria, Low C3 and normal C4 following a recent URI?

A

Post-streptococcal GN

• DOME-SHAPED SUBEPITHELIAL HUMPS

48
Q

What treatment measures are taken for PIGN?

• Prognosis?

A

Post-Infectious GN

SUPPORTIVE measures taken (disease should clear itself)
• Control HTN (ACE/ARB)
• Dialysis

PROGNOSIS = good, disease should resolve over a few weeks

49
Q

What lab values should you track after someone has been Dx with PIGN to ensure that they’re improving?

A

C3 Levels:
• these should normalize over the period of about 6 wks

Hematuria:
• should resolve withing 12 months

Renal - Gupta (17 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions