Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Renal System > Pathology of Glomerular Diseases > Flashcards

Pathology of Glomerular Diseases Flashcards

1
Q

What are the three layers of the glomerulus that fluid filtrates through?

A

Endothelial layer with fenestrated capillaries
Glomerular Basal Membrane composed of type IV collagen
Epithelial layer composed of filtration slits

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

What kind of barrier is the glomerular filtration barrier?

A

Size and charge barrier

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

How is the glomerular filtrate barrier a size barrier?

A

Filtration depends upon the size of the molecular

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

Which molecules are small enough to pass through the glomerular filtration barrier?

A

Glucose and amino acids

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

Which molecules are not small enough to pass through the glomerular filtration barrier?

A

Plasma proteins, red blood cells, white blood cells, and platelets

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

How is the glomerular filtrate barrier also a charge barrier?

A

Positively charged ions are filtered more easily compared to negatively charged ones, which are repelled

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

Why would a kidney biopsy be obtained?

A

To establish a diagnosis, help guide therapy, ascertain the degree of active and chronic changes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What does the routine evaluation of percutaneous kidney biopsy involve?

A

Examination of tissue through:
LM
IF
EM

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

What is the purpose of LM?

A

It uses H&E, which is the standard stain, as well as special stains to define glomerular structures

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

What is the purpose of IF?

A

It localizes and identifies the class of immunoglobulins and complement components

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

How does IF work?

A

It is based on the use of specific antibodies which have been chemically conjugated to fluorescent dyes. They bind directly or indirectly to identify the deposition of abnormal molecules and proteins in the tissue

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is the purpose of EM?

A

To show the presence and precise location of immune complexes

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

What do immune complexes present as under EM?

A

Irregular deposits of electron-dense material and other deposits such as amyloid, diabetic changes, structural changes to GBM and podocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are the classifications of glomerular diseases?

A

Nephrotic Syndrome
Nephritic Syndrome
Asymptomatic hematuria or non-nephrotic proteinuria
Rapidly progressive glomerulonephritis with acute renal injury and rapid loss of renal function
CKD
ESRD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the presentation of CKD?

A

Uremia, increased urea levels in the blood.

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

What is the management of ESRD?

A

Requires renal transplantation or dialysis

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

What are the clinical presentations of nephrotic syndrome? (4)

A

Massive proteinuria
Hypoalbumenia
Hyperlipidemia
Lipiduria

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

What is considered massive proteinuria?

A

> 3.5 g/day

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

What does hypoalbuminemia lead to?

A

Massive edema (anasarca)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What causes the proteinuria seen in nephrotic syndrome?

A

Damage to podocytes, compromised glomerular filtrate barrier –> loss of charge barrier –> proteinuria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Can Nephrotic syndrome be primary or secondary?

A

Both primary and secondary
Primary (FSGN in adults, minimal change disease in children)
Secondary (systemic process; diabetes)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

In which cases can nephritic syndrome present with nephrotic syndrome features?

A

In severe cases of nephritic syndrome where, there is enough damage to GBM to damage the charge barrier.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What is the pathophysiology of edema in the nephrotic syndrome?

A

Hypoalbuminemia –> decraese in oncotic pressure –> edema

Decrease in renal perfusion –> RAAS activation –> vasoconstriction –> increase in hydrostatic pressure –> edema

Increase in aldosterone –> Na and H2O retention –> edema

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What is the effect of hemoconcentration?

A

It will trigger osmoreceptors in the brain to secrete ADH –> more water retention –> more edema

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
What does the decrease in cardiac secretion of natriuretic factors cause?
Exacerbation of edema
26
How are natriuretic factors secreted?
Through the stretching receptors of the atrium
27
What are the complications of nephrotic syndrome?
Infections Arterial and Venous thrombosis Iron defecinecy anemia
28
What causes infections to be a complication of NS?
Loss of complement proteins and immunoglobulins in the urine
29
What is a more common complication, arterial or venous thrombosis?
Renal vein thrombosis
30
Why is thrombosis a complication of NS? (6)
1. Increased urinary loss of endogenous anticogulant antithrombin - III 2. Hyperfibrinogenemia from increased synthesis in liver 3. Decreased fibrinolysis 4. Increased platelet aggregation 5. Endothelial changes from hyperlipidemia 6. Altered levels of complement proteins
31
What causes the increased platelet aggregates?
Hyperlipidemia
32
What causes IDA to be a complication of NS?
Transferrin also might leak in chronic cases
33
What is nephritic syndrome?
Inflammatory process
34
What are the clinical presentation of nephritic syndrome?
Hematuria, and RBC casts in urine Oliguria Azotemia Hypertension Proteinuria with/without edema
35
Why is there oliguria in nephritic syndrome?
The glomerulus is busy with the inflammation and is not able to the urine
36
What causes the RBC casts?
RBCs leak and accumulate in the tubule and take the shape of the tubule
37
What is the difference between proteinuria in NS and nephritic syndrome?
In nephritic syndrome, the protein found in the urine is within the subnephrotic range
38
What is seen in biopsy of nephritic syndrome?
Hypercellular/inflamed glomeruli
39
Can the nephritic syndrome be primary or secondary?
Primary and secondary Primary: post-infectious glomerular nephritis, crescentic glomerulonephritis Secondary: systemic process; SLE
40
What can cause abnormalities of the glomerular function?
Damage to major components of the glomerulus, epithelium (podocytes), basement membrane, capillary endothelium or mesangium
41
What are the mesangial cells?
Extracellular matrix, support cells
42
What is the main mechanism under most of the primary causes of glomerular disease?
Immune mechanisms
43
What are some non-immune mechanisms that can also cause glomerular diseases?
Podocyte injury & nephron loss
44
What is nephron loss?
Nephron will die, the adjacent will proliferate, the fibrosis will spread to the adjacent, and so on
45
What is the immune mechanism of glomerular injury?
Formation of antibody-antigen immune complexes --> complement activation & leukocyte recruitment
46
How does neutrophil activation cause glomerular injury?
Release of cytokines, immune activation --> glomerular damage
47
What is the most common nephrotic syndrome in children?
Minimal change disease (lipoid nephrosis)
48
What causes minimal change disease?
Hyperlipidemia
49
What is the pathogenesis of minimal change disease?
Injury to podocytes --> T cell dysfunction --> glomerular permeability factor is affected --> foot process fusion --> decrease in anion property of GBM & loss of negative charge --> selective proteinuria
50
What is the primary cause of minimal change disease? What is the prognosis?
Idiopathic --> excellent response to corticosteroids, but some recur and become steroid dependent May follow recent infection or immunization
51
What is selective proteinuria?
Only albumin passes through, if non-selective, then albumin and other proteins would pass through
52
What is secondary causes of minimal change disease (lipoid nephrosis)?
Second to Hodgkin lymphoma, thus pathogenesis is unclear
53
What are the histopathological views of minimal change disease?
LM: normal glomeruli IF: negative EM: effacement of podocyte foot processes
54
What is focal segmental glomerulosclerosis (FSGS)?
Increasingly common cause of NS in adults & frequent cause in children
55
In which population is FSGS common?
Hispanic and African American adults
56
What is sclerosis?
Accumulation of extracellular collagenous matrix to mesangial areas
57
What is considered focal?
Less than 50% of glomerulus ivolved
58
What is considered segmental?
Not all of the glomerulus
59
What are the primary causes of FSGS?
Idiopathic, injury to podocytes
60
What are the secondary causes of FSGS?
Secondary to other conditions: HIV, heroin abuse, SCA, associated with other GNs AD (can be genetic, podocyte proteins, podocin) Familial genetic mutation of NPHS1
61
What are the clinical presentations of FSGS?
Patients may present with nephrotic but hematuria & hypertension may occur
62
Is the primary FSGS responsive to steroids?
Inconsistent response to steroids, 50% progress to CKD
63
What are the histopathological views of FSGS?
LM: segmental sclerosis and hyalinosis, seen as a collection of scarred, obliterated capillaries and accumulation of matrix IF: often negative but may show nonspecific focal trapping of IgM & complement EM: effacement of foot processes similar to minimal change disease
64
What is hyalinosis?
Accumulation of material that is eosinophilic and homogeneous
65
In what population does membranous nephropathy occur?
Adults 30 to 60 years with nephrotic syndrome (caucasian)
66
What is membranous nephropathy characterized by?
Subepithelial immunoglobulin containing deposits along GBM
67
What are the primary causes of membranous nephropathy?
Antibodies agains insitu podocytes antigene PLA2R
68
What are the secondary causes to membranous nphropathy?
Drugs (NSAIDs, penicillamine, captopril, gold) Infections (HBV, malaria, syphilis) SLE, Tumors
69
What is the prognosis of the primary membranous disease?
Poor response to steroids, may progress to CKD
70
What are the histopathological views of membranous disease?
LM: diffuse thickening of the capillary wall IF: granular deposit of immune complex along GBM EM: subepithelial immunoglobulin-containing deposits along GBM, separated from each other by small, spike-like protrusions of GBM matrix
71
Spike and dome appearance is present in which glomerular disease?
Membranous neohropathy
72
What do the "spikes" of the membranous disease represent?
Represent the intervening matrix of the basement membrane between the deposits
73
What are the two types of membranoproliferative glomerulonephritis?
MPGN type 1 Dense-deposit disease (MPGN 2)
74
What are the clinical presentations of membranoproliferative glomerulonephritis?
Present only with hematuria or proteinuria in the nephrotic range; others exhibit a combined nephrotic-nephritic picture
75
What are the secondary causes of type 1 membranoproliferative glomerlonephritis?
SLE, Hep. B and C Chronic liver disease Chronic bacterial infections
76
What are the histopathological views of Type 1 membranoproliferative disease?
Subendothelial immunoglobulin complexes deposits and granular IF
77
What is Type 2 membranoproliferative disease associated with?
C3 nephritic factor --> IgG autoantibody that stabilizes C3 convertase --> persistent complment activation --> decreased C3 levels (dense deposit disease)
78
What is the similarity of histopathological views of both membranoproliferative glomerulonephritis?
Mesangial ingrowth - GBM splitting, "tram-track" appearnce on H&E and PAS stains, viewed on LM
79
What are the histopathological views of membranoproliferative glomerulonephritis?
LM: accentuated lobular appearance and showed proliferation of mesangial and endothelial cells as well as infiltrating leukocytes
80
Which population is acute post-streptococcal glomerulonephritis commonly seen? (APGN)
Children
81
How does APGN develop?
1 to 4 weeks after a group A streptococcal infection of the pharynx or skin (impetigo)
82
Which kind of strep stains cause APGN?
Nephritogenic strains of β-hemolytic streptococci (M-protein virulence factor) evoke glomerular disease
83
What are other infections that can cause APGN?
Pneumococcal and staphylococcal infections & several viral diseases such as mumps, measles, chickenpox, hep B and C
84
What is the effect of glomerular deposition on immune complexes?
Proliferation of and damage to glomerular cells and infiltration of leukocytes, especially neutrophils --> Type III h.r.
85
What are the clinical presentations of APGN?
Acute nephritic syndrome Peripheral and periorbital edema Tea or cola-coloured urine, Hypertension Azotemia
86
What will the blood test results be like in APGN?
Positive strep titers Decreased complement levels (C3) RBC casts in urine
87
What is the prognosis of APGN like?
Resolves spontaneously in most children, in adults it may progress to renal insufficiency or RPGN
88
What are the histopathological views of APGN?
LM: Glomeruli enlarged and hypercellular, red blood cell casts in the tubules IF: Starry-sky granular appearance (lumpy-bumpy) EM: Subepithelial IC humps, subendothelial, and mesangial deposits
89
What causes the lumpy - bumpy appearance in acute post-streptococcal glomerulonephritis?
IgC, IgM, and C3 depostion along GBM and masangium
90
What is IgA nephropathy known as?
Berger disease
91
What is Berger disease?
Most common cause of recurrent microscopic or gross hematuria, and most common glomerular disease revealed by renal biopsy worldwide
92
How does IgA nephropathy occur?
Concurrently with respiratory or GIT infections
93
What is the hallmark of Berger disease?
Mesangial IgA deposition, abnormal glycosylated IgA1
94
What are the presentations of Berger disease?
Localised variant of Henoch-Schonlein purpura, GI pain (abdominal pain), and joint pain (arthritis)
95
What are the genetic factors of IgA nephropathy?
Abnormal or increased IgA response
96
What are the secondary causes of Berger disease?
Nephropathy seen in Celiac disease and liver disease
97
What are the histopathological views of IgA nephropathy (Berger disease)?
LM: Mesangial proliferation IF: IgA-based IC deposits in the mesangium EM: mesangial IC deposition
98
What is hereditary nephritis?
Group of glomerular diseases caused by mutation in genes encoding GBM proteins (IV collagen)
99
What are examples of glomerular diseases that cause hereditary nephritis?
Alport syndrome Thin basement membrane disease
100
What is Alport syndrome?
Nephritis accompanied by sensorineural deafness and various eye disorders X-linked disease
101
What are the presentations of Alport syndrome?
Hematuria & proteinuria at 5 to 20 years and renal failure at 20 to 50 years
102
What is seen in EM in Hereditary Nephritis?
Thin GBM (early course) GBM develops irregular foci of thickening (late course) --> basketwave
103
What is thin basement membrane disease the most common cause of?
Benign familial hematuria with no systemic infection
104
What is the EM of thin basement membrane like?
Thin GBM
105
What is rapidly progressive (crescentic) glomerulonephritis?
A distinct clinical entity with nephritic syndrome with more pronounced oliguria and azotemia
106
What is the prognosis of rapidly progressive (crescentic) glomerulonephritis?
Poor prognosis, rapidly deteriorating renal function (days to weeks)
107
How do you differentiate between the different diseases that have the same pattern?
IF pattern
108
What are the different types of rapidly progressive (crescentic) glomerulonephritis?
Anti-GBM antibody-mediated crescentic GN Immune complex-mediated crescentic GN Pauci-immune type crescentic GN
109
What is anti-GBM antibody-mediated crescentic GN?
Goodpasture disease: auto-antibodies to GBM and alveolar basement membrane, hematuria/hemoptysis, type 3 HSR Linear IF
110
Wha is the treatment of anti-GBM antibody-mediated crescentic GN?
Plasmapheresis
111
What is immune complex-mediated crescentic GN?
Follow immune complex nephritides, including post-streptococcal GN, SLE, and IgA nephropathy IF is granular IC
112
Where are ANCA found?
In the serum
113
What is Pauci-immune type crescentic GN?
Lack of anti-GBM antibodies or significant IC deposition. Anti-neutrophil cytoplasmic antibodies (ANCA) IF is negative, no linear or granular IC
114
What other systemic diseases is Pauci-immune type crescentic GN associated with?
Systemic vasculitis such as microscopic polyangiitis (p-ANCA) pr Wegners granulomatosis with polyangiitis (c-ANCA)
115
What are the histopathological views of rapidly progressive (crescentic) glomerulonephritis?
LM: crescent moon shape --> Crescents consist of fibrin and plasma protein with glomerular parietal cells, monocytes, lymphocytes and macrophages EM: Ruptured GBM
116
What happens to the crescents eventually?
Obliterate Bowman's space and compress the glomeruli --> scarring and glomerulosclerosis develop
117

Renal System (24 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions