Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Renal - Gupta > Nephrotic Syndrome II > Flashcards

Nephrotic Syndrome II Flashcards

(49 cards)

Start Studying
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?

Study These Flashcards
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?

Study These Flashcards
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?

Study These Flashcards
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.

Study These Flashcards
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.

Study These Flashcards
A
  1. Supportive Measures
    • Control BP - ACE Is/ARB
    • Treat Hyperlipidemia
  2. Disease Modifiers
    • Corticosteroids
    • Calcineurin Inhibitors (Cyclosporin or Tacrolimus)
25
How do the treatment measures for FSGS differ from minimal change disease?
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
Apply the following terms to Minimal Change Disease and Focal Segmental Glomerular Sclerosis: • Good/Bad Px. • (non)/Steroid Sensitive NS
Minimal Change Disease: • Good Px. • Steroid Sensitive Focal Segemental Glomerular Sclerosis: • Bad Px. • Steroid Resistant
27
T or F: MCD may just be an early form of FSGS.
True, these disease are probably part of the same disease spectrum as seen in looking at children who progress to FSGS from MCD
28
What two type of Nephrotic Syndrome are associated with Subepithelial Immune complex deposits?
1. Membranous Nephropathy | 2. Post-infectious Glomerulonephrtitis
29
Membranous Nephropathy: • Who is this mostly seen in? • Prognosis?
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
What should you do if you have a pt. that is 60 or 70 and is found to have a membranous nephropathy?
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
What are the categories of Primary and Secondary Membranous Nephropathies?
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
Compare the two theories of immune complex deposition in Membranous Nephropathy and the processes/disease that are thought to follow these patterns.
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
What are frequently the Targets of autoanibodies generated in Membranous Nephropathy? • are these associated with congenital or primary MN?
1. M-type phospholipase A2 receptor - -> PRIMARY MN 2. Neutral Endopeptidase - -> Congenital MN
34
What two types of membranous nephropathy are associated with the autoimmunity model?
Targets of Autoimmunity: • M-type Phospholipase A2 receptor (primary MN) • Neutral Endopeptidase (congenital)
35
Describe the 5 steps in In-Situ Immune Complex formation in Membranous Nephropathy.
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
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?
* PLA2R autoantibody is present in 70% of pts. with M type phospholipase A2 * Expressed in PODOCYTES in normal human glomeruli * IgG4 = autoantibody type
37
Do you ever see PLA2R in patients with secondary membranous nephropathy? • where is the damage done in membranous nephropathies?
PLA2R: • NO - this is found in patients with PRIMARY IDEOPATHIC Membranous Nephropathy • Damage in membranous Nephropathy = Subendothelial
38
What are 6 risk factors for loss of renal function in membranous Nephropathy?
1. Male Gender 2. Over 10g/day of proteinuria 3. HTN 4. Azotemia 5. Tubulointerstitial Fibrosis 6. Glomerulosclerosis
39
What are some key features to look for on LM, IF, and EM in membranous Nephropathy?
LM: • SPIKES on silver STAIN IF: • Granular along GBM EM: • SPIKE and DOME appearance of Subepithelial Deposits along GBM
40
T or F: in membranous nephropathy the capillary loops are thickened and prominent and the cellularity is increased
FALSE, cellularity is NOT increased in the membranous nephropathy
41
What is the typically treatment for membranous nephropathy? • what happens if this treatment fails? • Why do we treat is this way?
****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
``` KEY features of Post-Infectious Glomerulonephritis? • Hx • Physical Exam. • Blood Tests • Urine Analysis ```
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
How will a patient with Post-Infectious Glomerulonephritis typically present?
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
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?
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
What is the typical appearance of Glomeruli in Post-streptococcal GN on H and E? • what physiologic problem is caused by these changes?
* Glomeruli are Globally and diffusely enlarged and HYPERCELLULAR due to NEUTROPHILS, MACROPHAGES, MESANGIAL and ENDOTHELIAL proliferation * INFLAMMATION causes obstruction of Capillary Lumen
46
What will you see on immunofluorescence of someone who has a Nehpritis following clearance of a recent infection? • what causes this appearance?
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
What do you expect to see on EM for patients that have hematuria, Low C3 and normal C4 following a recent URI?
Post-streptococcal GN • DOME-SHAPED SUBEPITHELIAL HUMPS
48
What treatment measures are taken for PIGN? | • Prognosis?
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
What lab values should you track after someone has been Dx with PIGN to ensure that they're improving?
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.
© 2025 Bold Learning Solutions. Terms and Conditions