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
Q

What syndrome do subeepithelial deposits tend to cause?

A

Nephrotic.

26
Q

What syndrome do subendothelial and messangial deposits tend to cause?

A

Nephritic

27
Q

What are the clinical features of nephrotic syndrome?

A
  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
Q

What are the absolute contraindications for renal biopsies?

A
  1. Bleeding Diathesis

2. Uncontrolled hypertension

29
Q

What are some relative contraindications for renal biopsies?

A
  1. Single Kidney
  2. High pressure hydronephrosis
  3. Adult polycystic kidney disease
30
Q

What can cause possible death because of a kidney biopsy?

A

Unrecognized retroperitoneal bleeding.

31
Q

What is the main site of size hindrance for larger molecules?

A

Lamina Densa and slit disphragm

32
Q

What is the main site for charge hinderance?

A

The anionic charged Lamina rara interna and fenestrated capillary endothelium

33
Q

What is the major hinderance to albumin being filtered into the urinary space?

A

Charge!!!!

34
Q

What happens to the proteins that make it into the urinary space?

A

Majority are reabsorbed in the proximal tubule.

35
Q

How do epithelial cells of the proximal tubule reabsorb proteins?

A

By endocytosis involving the MEGALIN and CUBULIN.

36
Q

What kinds of protein aare seen in the urine in Tubular disease?

A

LMW proteins

37
Q

What protein does urine dipstick measure?

A

Albumin.

38
Q

What is the low detection limit for a urine dipstick?

A

300-500mg/day

39
Q

What is the definition of microalbuminemia?

A

30-300mg perday

40
Q

What are 3 of the primary management aspects of nephrotic syndrome management?

A
  1. Control of hypertension/Low salt
  2. Ace inhibitor ARB
  3. Corticosteroids
  4. Immunosuppresion
41
Q

What disease is associated with Foot process effacement?

A

MCD

42
Q

What disease is associated with “Spike and Dome” appearance?

A

Membranous Nephropathy

43
Q

What disease is associated with subepithelial humps?

A

Post-infectious glomerulonephritis

44
Q

What disease is associated with Basketweave appearance?

A

Alport syndrome

45
Q

Wire loops =

A

Lupus Nephritis

46
Q

Tram Tracks=

A

Membranoproliferative glomerulonephritis

47
Q

What is the protein that covers the foot processes and endothelial cells of the glomerulus?

A

Sialoprotein