Nichols - Nephrotic Syndrome 1 Flashcards

1
Q

Features of nephrotic syndrome

A
  1. Edema
  2. Proteinuria
  3. Hypoalbuminemia
  4. Hyperlipidemia
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2
Q

Nephrotic syndrome is a non-inflammatory condition with _____ urinary sediment and ______ or _______ creatinine.

A

inactive; normal or mildly elevated

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3
Q

What is the key cell in nephrotic syndrome?

A

Visceral epithelial Cell(podocyte)

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4
Q

Features of nephritic syndrome

A
  1. “active” urinary sediment(dysmorphic RBCs and red cell casts)
  2. Inflammation
  3. Hypertension
  4. Elevated creatinine
  5. Cresents in biopsy
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5
Q

What is the key cell in nephritic syndrome?

A

Endothelial Cell

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6
Q

What are some other clinical presentations of glomerular disease, other than nephrotic and nephritic syndrome?

A
  1. Asymptomatic disease
  2. Macroscopic hematuria
  3. rapidly progressive glomerulonephritis
  4. Chronic glomerulonephritis
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7
Q

When will glomerular disease be without symptoms?

A
  1. Proteinuria between 150 mg to 3 g/day
  2. Mild hematuria
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8
Q

Describe macroscopic hematurea:

A

Brown, red, or red/brown urine with no clots

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9
Q

Typical presentation of patients with chronic glomeruolnephritis?

A
  1. Hypertension
  2. Renal insufficiency
  3. Proteinuria
  4. Shrunken kidney disease
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10
Q

What are casts?

A

Cylindrical formations of cells or proeinaceous material

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11
Q

What are the most common casts?

A

Hyaline casts

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12
Q

What are these?

A

Dysmorphic RBCs

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13
Q

Describe hyaline casts

A

colorless, homogenous, and transparent

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14
Q

What do the arrows point to?

A

Hyaline Casts

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15
Q

What forms hyaline casts?

A

The secretion of Tamm-Horsfall mucoprotein

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16
Q

Tamm-Horsfall mucoprotein is secreted by? Can sometimes contain what in conditions of proteinurea?

A

Renal Tubule Cells; Albumin

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17
Q

The second most common type of cast?

A

Granular Casts

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18
Q

What do granular casts result from?

A

Aggregates of plasma protein, breakdown of cellular casts, or the breakdown of cellular casts

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19
Q

What color are granular casts composed of fine granules? Coarse granules?

A

Grey or pale yellow;

Darker - often black

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20
Q

Where are granular casts formed?

A

Renal Tubule

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21
Q

What do granular cast almost always indicate?

A

Significal renal disease

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22
Q

What type of casts are seen with acute tubular necrosis?

A

“Muddy brown” granular casts

23
Q

What is this?

A

Granular Cast

24
Q

What are fatty casts?

A

Yellow-tan hyaline casts with fat globulin inclusions (oval fat bodies)

25
What forms fatty casts?
Breakdown of lipid-rich epithelial cells
26
What is the maltese cross pattern seen under polarized light associated with? Indicate what?
cholesterol or cholesterol esters are present. Lipiduria in Nephrotic Syndrome
27
What does the mechanism of disease in nephrotic syndrome depend on?
Whether there is inflammation as part of the disease process
28
Mechanisms of nephrotic syndrome without glomerular inflammation?
1. **Podocyte injury** in minimal change disease and focal segmental glomerulosclerosis 2. **Subepithelial immune complex formation** and **complement activation** in membranous nephropathy 3. **Glomerular capillary wall deposition** in diabetic nephropathy, amyloidosis, and light chain deposition disease
29
Mechanism of nephrotic syndrome with glomerular inflammation
1. **Mesiangial or capillary wall immune complex formation and complement activation** in IgA nephropathy and acute post-streptococcal glomerulonephritis 2. **Antibodies directed against GBM** in anti-GBM disease 3. **Necrotizing injury and inflammation of the glomerular capillaries** in disease associated with antineutrophil cytophil autoantibodies (ANCA)
30
Why are immune complexes formed and trapped in the glomeruli?
1. The high plasma flow rate through them 2. High intraglomerular pressure 3. high glomerular hydraulic conductivity
31
What does the manifestation of disease depend on?
The nature of the antigen involved and the site of immune complex deposition.
32
What diseases are subepithelial deposits seen in?
post-infectious glomerulonephritides and membranous nephropathy
33
What do subepithelial deposits tend to cause?
A nephrotic syndrome picture
34
Where are subendothelial and mesangial deposits formed?
Either locally or from passive entrapment of circulating immune complexes
35
What do subendothelial and mesangial deposits tend to cause?
Nephritic syndrome picture - focal or diffuse proliferative lupus nephritis and IgA nephropathy
36
Clincal features of nephrotic syndrome:
1. Urine protein excretion - \> 50mg/kg/day or \> 3.5 g/day in adults and \> 40mg/hr/M2 in children 2. Hypoalbumineamia - Serum level \< 3.5 g/dL 3. Hypercholesterolemia 4. Lipiduria 5. Can have xanthelasma(subQ lipid deposits)
37
3 purposes of kidney biopsy?
1. Make a dx 2. Determine px 3. Guide therapy
38
What 3 types of microscopy do renal biopsies need?
1. Light microscopy 2. Immunofluorescence 3. Electron Microscopy
39
Absolute contraindications of renal biopsy:
bleeding diathesis, uncontrolled hypertension
40
Relative controindications of renal biopsies:
single kidney, high pressure hydronephrosis, adult polyscystic kidney disease
41
What are the main sites of size hindrance for larger molecules in the glomerular filtration barrier?
Lamina densa of the GBM and the slit diaphragm
42
What is the estimated pore radius for spherical molecules?
42 Angstrom
43
What are the main sites of charge hindrance in the glomerular filtration barrier?
Anionic charge lamina rara interna and fenestrated capillary endothelium
44
How much protein is actually excreted in the urine? Why?
Only a tiny amount. Most of the filtered protein is reabsorbed by the renal tubules.
45
How are proteins taken up in the tubules?
Epithelial cells take them up through endocytosis.
46
What are the receptors that play a role in the endocytosis of protein?
megalin and cubulin
47
In glomerulonephritis what is the dominant protein in urine?
Albumin
48
Tubular disease is associatied with what type of proteinuria?
Low molecular weight proteins
49
What protein does a urine dipstick measure?
Albumin
50
How much albumin may health kidneys excrete each day?
30 mg
51
What is the rate of excretion for Tamm0Horsfall mucoprotein?
30-50 mg/day
52
What is microaluminuria?
Albumin levels between 30 and 300 mg/day it is the earliest clinical manisfestation of diabetic nephropathy.
53
What are some methods of measuring protein urine?
Dipstick, 24-hour urine collection, and spot urine protein/creatinine ratio.
54
Advantages of dipstick?
Cheap, quick, and semi-quantitative