Nephrotic Syndrome I Flashcards

1
Q

What are the features of nephrotic syndrome?

A
  1. Edema
  2. Proteinuria
  3. Hypoalbuminemia
  4. Hyperlipidemia
  5. Non-inflammatory
  6. Inactive urinary sediment (dysmorphic rbcs and rbc casts)
  7. Normal or mildly elevated creatinine
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2
Q

What is the key cell involved in nephrotic syndrome?

A

Visceral epithelial cell (podocyte)

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3
Q

What are some of the characteristics present in patients who present with chronic Glomerulonephritis?

A
  1. Hypertension
  2. Renal insufficiency
  3. Proteinuria (often over 3g/24hr)
  4. Smooth shrunken kidneys on ultrasound
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4
Q

What do patients with renal failure due glomerulonephritis referred to as rapidly progressive generally have in their urine?

A
  1. Red blood cell casts due to large numbers of red cells clumping in the tubules.
  2. Proteinuria but usually less than 3g/24hr
  3. BP is usually normal
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5
Q

What is one of the major causes of Rapidly progressive glomerulonephritis?

A

Autoimmune Vasculitis and patients will have features in other organs especially the skin

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6
Q

What are casts?

A

Cylindrical formations of cells or proteinaceous material.

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7
Q

What are the most common casts?

A

Hyaline casts

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8
Q

How are hyaline casts formed?

A

Form via precipitation of Tamm-Horsfall mucoprotein which is secreted by the renal tubule cells and sometimes contain albumin in conditions of proteinuria.
*A few Hyaline casts are normal

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9
Q

What are the second most common types of casts?

A

Granular type

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10
Q

What are granular casts?

A

Formed from aggregates of plasma proteins (albumin or immunoglobulin light chains) or the breakdown of cellular casts

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11
Q

How can granular casts differ in appearance?

A

Granular casts composed of fine granules are usually grey or pale yellow, granular casts composed of coarse grnules are darker often black

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12
Q

Where are granular casts believed to be formed?

A

In the renal tubule

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13
Q

How are “Fatty casts believed to be formed?

A

By the breakdown of lipid-rich epithelial cells

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14
Q

What are “Fatty cast”?

A

Tan-yellow hyaline casts with fat globule inclusions (oval fat bodies)

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15
Q

What is formed with “Fatty casts” in cholesterol esters are present?

A

Maltese cross pattern seen underpolarized light.

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16
Q

What are “Fatty casts” indicative of?

A

Lipiduria of nephrotic syndrome

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17
Q

What are red blood cell casts indicative of?

A

Glomerular damage

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18
Q

What are white cell casts indicative of?

A

Inflammation due to pyelonephritis, interstitial nephritis, acute post-streptococcal glomerulonephritis or nephrotic syndrome if it is associated with an inflammatory condition

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19
Q

What are the Nephrotic syndrome related diseases without glomerular inflammation?

A
  1. Minimal Change Disease
  2. FSGS
  3. Membranous nephropathy
  4. Diabetic Nephropathy
  5. Amyloidosis
  6. Light Chain deposition disease
20
Q

What are the Nephrotic syndrome diseases with glomerular Inflammatory involvement?

A
  1. IgA Nephropathy
  2. Acute post-streptococcal glomerulonephritis
  3. Anti-GBM disease
21
Q

In early course of post-infectious glomerulonephritis in general and in post-streptococcal glomerulonephritis in particular where do the inmmune complexes tend to be deposited?

A

the immune complexes tend to be deposited in a subendothelial location early on

22
Q

In the later course of post-infectious
glomerulonephritis in general and in post-streptococcal glomerulonephritis in particular where do the inmmune complexes tend to be deposited?

A

They tend to be deposited in subepithelial location later on.

23
Q

Where are the immune complex deposits classically in lupus nephritis?

A

Subendothelial

24
Q

What are the factors that predispose the Glomeruli to trapping immune complexes?

A
  1. High plasma flow rate through them
  2. High intraglomerular pressure
  3. High glomerular hydraulic conductivity
25
What syndrome do subeepithelial deposits tend to cause?
Nephrotic.
26
What syndrome do subendothelial and messangial deposits tend to cause?
Nephritic
27
What are the clinical features of nephrotic syndrome?
1. 50mg/kg/day of 3.5g/day 2. 40mg/hour/M2 in children 3. Hypoalbuminemia 4. Hypercholesterolemia 5. Lipiduria 6. Possible Xanthelasma (sub-Q lipid deposits)
28
What are the absolute contraindications for renal biopsies?
1. Bleeding Diathesis | 2. Uncontrolled hypertension
29
What are some relative contraindications for renal biopsies?
1. Single Kidney 2. High pressure hydronephrosis 3. Adult polycystic kidney disease
30
What can cause possible death because of a kidney biopsy?
Unrecognized retroperitoneal bleeding.
31
What is the main site of size hindrance for larger molecules?
Lamina Densa and slit disphragm
32
What is the main site for charge hinderance?
The anionic charged Lamina rara interna and fenestrated capillary endothelium
33
What is the major hinderance to albumin being filtered into the urinary space?
Charge!!!!
34
What happens to the proteins that make it into the urinary space?
Majority are reabsorbed in the proximal tubule.
35
How do epithelial cells of the proximal tubule reabsorb proteins?
By endocytosis involving the MEGALIN and CUBULIN.
36
What kinds of protein aare seen in the urine in Tubular disease?
LMW proteins
37
What protein does urine dipstick measure?
Albumin.
38
What is the low detection limit for a urine dipstick?
300-500mg/day
39
What is the definition of microalbuminemia?
30-300mg perday
40
What are 3 of the primary management aspects of nephrotic syndrome management?
1. Control of hypertension/Low salt 2. Ace inhibitor ARB 3. Corticosteroids 4. Immunosuppresion
41
What disease is associated with Foot process effacement?
MCD
42
What disease is associated with "Spike and Dome" appearance?
Membranous Nephropathy
43
What disease is associated with subepithelial humps?
Post-infectious glomerulonephritis
44
What disease is associated with Basketweave appearance?
Alport syndrome
45
Wire loops =
Lupus Nephritis
46
Tram Tracks=
Membranoproliferative glomerulonephritis
47
What is the protein that covers the foot processes and endothelial cells of the glomerulus?
Sialoprotein