Nephrotic and Nephritic Syndrome Flashcards

1
Q

What is the mainstay of nephritic syndrome? What about nephrotic syndrome?

A
  • Nephritic syndrome usually presents with blood in urine and sometimes hypertension
  • Nephrotic syndrome usually presents with proteinuria and edema.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What are the proteins secreted by the kidneys

A

(PRE) Erythropoeitin, Prostaglandins, and Renin

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

what is the normal anatomy of the kidney?

A

The normal kidney has a well delineated cortex and medulla. In the fetal kidney you may see fetal lobulations. The surface of the kidney is smooth but may present with an occasional cyst and may have adipose tissue. IT IS NOT LUMPY (granularity) as you would see it in hypertension.

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

histologically on a kidney biopsy, what do you see between the glomerular and the bowman’s capsule?

A

From Glomerular–> to the Bowmans Capsule

  1. Fenestrated endothelium of the capillaries.
  2. Glomerular capillary basement membrane.
  3. Glomerular Capillary Loops with the epithelial cells with podocytes (foot processes)
  4. Mesangium (muscle cells that support the capillaries)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What are the glomerular diseases?

A

NAACA

  1. Acute nephritic syndrome
  2. Nephrotic Syndrome
  3. Asymptomatic hematuria or proteinuria
  4. Acute renal failure
  5. Chronic renal failure
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Azotemia is what

A

is the laboratory manifestations of renal problems and it means increased BUN and creatinine.

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

what are the three causes of azotemia?

A
  1. Pre-renal: have a problem with impaired perfusion
  2. Renal: intrinsic kidney impairment
  3. Post-renal: distal urinary tract obstruction
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

pre-renal azotemia can be a sign of what?

A

of acute renal failure

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

What are the clinical manifestations of azotemia according to the lecture and what can it manifest as

A

uremia: Not everyone with azotemia exhibits uremia. The kidneys are there to filter the plasma. If the blood is not appropriately filtered by the kidney, you poison yourself which can lead to the manifestations seen in uremia. Uremia can manifest as pericarditis, lethargy, and edema among other things

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What does chronic renal failure look like grossly?

A
  • thinned out cortex
  • cystic spaces
  • lumpy bumpy appearance
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What does chronic renal failure look like histologically?

A
  • sclerosing round body that used to be the glomerula
  • hyaline obliteration
  • thyroidization of the tubules
  • amorphose material in the tubules
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

what diseases can you have intrinsic to the kidney mentioned in this lecture?

A

primary glomerulopathies: Nephrotic and Nephritic
Systemic diseases
Hereditary disorders

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

Dysfunction in the glomerular will show up as?

A

Clinically you will have blood and protein in the urine

  • Hypercellularity (increase in mesangial cells or extracellular matrix)
  • Increase in endothelial cells
  • influx of leukocytes
  • crescents in bowmans space formed (composed of parietal epithelial cells, fibrin, and leukocytes) (also an indication of severe glomerulopathy
  • thickening of basement membrnae
  • hyaline and scleroses of glomeruli
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

what are the different types changes dysfunctional glomerula can sho

A
  • diffuse
  • focal
  • segmental
  • global
  • mesangial
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

what are the two kinds of immune responses leading to glomerulopathy?

A
  1. anti-glomerular basement membrane (anti-GBM) disease

2. immune complexes that form outside the kidney but get stuck and injure the glomeruli

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

what are the two kinds of immunofluorescence leading to glomerulopathy?

A
  • it can be smooth with anti-glomerular basement membrane

- it can be lumpy with immune complexes or anti podocytes

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

what is the antigen that binds to the anti-GBM

A

the type IV collagen of the basement membrane

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

what is the immunofluorescence pattern you see with the podocytes

A

heymann: The Heymann pattern is granular because of the presence of skip areas

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

the immune complexes can deposit where?

A

subepithelia, subendothelial, and mesangial giving you granular immunofluorescence

20
Q

what are the two types of nephritic syndromes

A
  • acute proliferative glomerulonephritis

- crescentic glomerulonephritis

21
Q

what are the characteristics of acute proliferative glomerulonephritis

A
  • main feature is hematuria
  • azotemia
  • red cell cast
  • oliguria
  • high blood pressure
  • some proteinuria and edema, however not as much as nephrotic syndrome
22
Q

what can lead to acute proliferative glomerulonephritis?

A

post-streptococcal caused by group A b-hemolytic streptococcal due to patients not taking the full 10 day load of antibiotics

23
Q

how does post-streptococcal cause acute proliferative glomerulonephritis?

A

immune complexes are formed that deposit in the kidneys

24
Q

what does acute proliferative glomerulonephritis look like?

A
  • swollen kidneys; petechial
  • hypercellularity glomeruli; inflammatory agents neutrophils, monocytes, mesangial cells, endothelial cells
  • diffuse process becuase it affects multiple/all glomeruli
  • granular, IgG, IgM, C3+ (immune complex mediated
  • EM you will see subepithelial humps
25
Q

what are the characteristics of rapidly progressive (crescentic) glomerulonephritis?

A

VERY VERY VERY HORRIBLE Prognosis

  • no known etiology
  • all the symptoms associated with acute nephritis syndrome
  • 50% of cases are idiopathic and presents in end stage renal disease
  • Patients are usually older >40
26
Q

what are the three types of rapidly progressive crescentic glomerulonephritis?

A

Type 1- Linear (anti-basement membrane antibody), Anti-GBM
Type 2- Granular (immune complex deposition)
Type 3- Negative IF (pauci-immune)

27
Q

What are the associations and characterisitics of type 1- linear (anti-basement membrane antibody), Anti-GBM

A
  • anti IV Collagen of the basement membrane
  • associated with good pastures disease (with pulmonary hemorrhage)
  • shows smooth linear deposits
28
Q

What are the associations and characterisitics of type 2 granular (immune complex deposition)

A
  • it is immune complex mediated
  • associated with SLE(systematic lupus), Henoch Scholoen IgA, postinfectious
  • presents with granular deposits
29
Q

What are the associations and characteristics of type 3 negative IF (pauci immune)

A
  • anti-neutrophil cytoplasmic antibody
  • associated with Wegners, and microscopic polyangitis
  • The immunofluorescence pattern for type III RPGN is neither granular nor smooth
30
Q

What immunofluorescence denotation can be given to type 3?

A

-“not well studied”, “weakly granular”, and pauci immune

31
Q

a crescentic glomerulonephritis will show what on a PAS stain

A

-collapsed glomerula tufts by the crescent shaped mass of proliferating cells and the leukocytes into the bowmans capsule

32
Q

what are the components of the crescent?

A

fibrin, epithelial cells, inflammatory cells, bowman’s space

33
Q

what are the four types of nephrotic syndromes?

A

presentation: These patients have a lot of proteinuria with low plasma albumin and edema

  • membranous glomerulonephropathy
  • minimal change disease
  • focal/segmental glomerulosclerosis
  • membranoproliferative glomerulonephritis
34
Q

membranous glomerulopathy some characteristics and associations

A

-most common nephrotic syndrome in adults
-immune complexes showing heymann granular presentation
-associated with drugs (NSAIDS and penicillamine), SLE, tumors, infections(hepatitis B or C)
-

35
Q

what does membranous glomerulopathy look like

A
  • on PAS stain thickening of the glomerular capillaries particularly the basement membrane
  • on EM you see immunoglobulin (IgG) subepithelial deposits that may destroy the foot processes
  • On silver stain you see the spikes which are the deposits themselves
36
Q

some minimal change disease characterisitics

A
  • most common in children
  • effacement of the foot processes
  • no change such as hypercellularity
  • related to respiratory infections or immunization
  • great response to steroid, so great prognosis
37
Q

some focal/segmental glomerulosclerosis characteristics

A
  • mostly affects people before the age of 35; mostly african american
  • nephrotic syndrome with heavy proteinuria
  • associated with HIV, heroin, obesity, sickle cell anemia
  • affects podocytes
  • you will see hyaline insiduation and sclerosis
38
Q

membranoproliferative glomerulonephritis

A
  • can present as nephritic syndrome
  • often goes to end stage renal disease
  • idiopathy or secondary to SLE, Heroin, Cancer, Infection (Hep B, C and HIV)
  • affects the mesangial area
  • proliferation of glomerula cells with leukocyte infiltration
  • immune complex deposition with the activation of classical and alternative complement pathway
39
Q

what does membranoproliferative glomerulonephritis look like

A

-thickened capillaries, increased mesangial areas, and doubling of the size to the basement membrane (tram-track on silver stain)

40
Q

what are the two types of membranoproliferative glomerulonephritis

A

Type 1: subendothelial deposition in fenestrated endothelium and the mesangial areas. Also it is more common than the other type

Type 2: Less common and you get dense deposits and thickened basement membrane

41
Q

primary hematuria occurs when?

A

when you have blood in the urine that is not in the range of nephrotic or nephritic syndrome

42
Q

IgA nephropathy and berger’s disease characteristics

A
  • IgA deposits in the mesangium
  • most common worldwide glomerulonephritis and most common in persistent and recurent hematurias
  • seen in young adults and adolescents and is in males more than females
  • usually follows a viral infection
  • usually benign
43
Q

what is the difference between primary and secondary IgA nephropathy?

A

IgA nephropathy can be primary but you can also have IgA deposits secondary to other things such as in Henoch-Shonlein pupura

44
Q

what are the glomerula lesions associated with systemic diseases

A

i. Glomerular lesions can be caused by lupus.
ii. Henoch-Shonlein purpura can also result in glomerular lesions.
iii. Bacterial endocarditis can result in emboli lodging in the kidney causing glomerular lesions.
iv. Diabetic glomerulosclerosis is the most common form of kidney disease

45
Q

what is the most common form of kidney disease

A

diabetic glomulerosclerosis

46
Q

what are the charaterisitics of diabetic glomulerosclerosis

A
  1. 40% of type I and II diabetics end up with severe or endstage renal disease.
  2. These patients might show up with nonnephrotic proteinuria, nephrotic syndrome, or chronic renal failure (azotemia with or without uremia).
  3. What does diabetic glomerulosclerosis look like?
    a. Grossly, the kidney in diabetic glomerulosclerosis presents with a granular surface (lumpy-bumpy).
    b. Diabetic glomerulosclerosis presents histologically with capillary basement membrane thickening, diffuse mesangial sclerosis (scarring) and proliferation, and nodular glomerulosclerosis (Kimmelstiel-Wilson syndrome). Kimmelstiel-Wilson syndrome is characteristic of diabetic glomerulosclerosis and it presents with distinct nodules.
47
Q

what are the two systemic hereditary syndromes

A
  1. Alport syndrome can cause primary hematuria. It is also called hereditary nephritis.
    a. Alport syndrome is associated with nephritis leading to renal failure, nerve deafness, and eye disorders (i.e. cataracts).
    b. Alport syndrome is X-linked.
    c. Alport syndrome presents with thinning and thickening of the basement membrane forming a basketweave pattern.
  2. Thin basement membrane disease can also cause primary hematuria and this is a benign familial hematuria.