05.05 - Glomerular Structure, Injury (Nichols, Handorf) - Based on word doc Flashcards Preview

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Flashcards in 05.05 - Glomerular Structure, Injury (Nichols, Handorf) - Based on word doc Deck (95):
1

Hyaline Sclerosis in HTN vs DM

In HTN, just afferent arteriole; In DM, both

1

Anti-GBM disease is associated with

Smoking, being male

1

Location of In situ immune complex formation and deposition

Subepithelial

1

What does Trichrome stain highlight

Collagen

2

Treatment of Anti-GBM Ab disease

Removing them with Plasmapharesis

2

IgA Nephropathy is due to

Production of Abnormal IgA that self-aggregates and binds IgG --> immune complex formation --> Mesangial cell activation --> Complement-mediated injurious inflammation

2

What stain highlights the GBM

Jones silver stain

2

What is becoming the most common type of Crescentic Glomerulonephritis

Pauci-immune

3

3 most common types of glomerular disease

(1) HTN, (2) Diabetic, (3) Immune-mediated

3

Most common cause of glomerular disease

Hypertension

3

Platelet mechanisms of glomerular injury in immune-mediated disease

Release AA metabolites

3

In situ immune complex formation with Ab's against podocyte cell membrane antigens causes great majority of

Membranous Glomerulonephritis

4

Calcium binding properties

Features of Entactin

4

What does Global Glomerular Disease refer to

Involves all of a single glomerulus

5

Feedback loop with hypertensive kidney disease

Glomerular Disease causes HTN, and HTN causes glomerular disease

5

What happens to GBM in DM

Glycosylated plasma proteins get trapped --> GBM gets thickened

5

What does Diffuse Mesangial Sclerosis mean

Sclerosis of all the mesangium in one glomerulus

6

What causes Crescentic Glomerulonephritis

Leakage of Plasma Proteins into urinary space (particularly when mixed with Ab's, Immune Complexes, Inflammatory Cytokines, Inflammaotry cells, and ROS)

6

Location of immune complex deposition in Post-Strep Glomerulonephritis

Subepithelial

8

Important Concept: ____ is a major mechanisms of glomerular injury

Antibody deposition

9

Mutations in what result in congenital nephrotic syndromes?

Nephrin and Podocin

10

Urine in Malignant HTN

Marked Proteinuria, Microscopic Hematuria

11

Features of Perlecan

Strong negative charge - prevents Albumin from entering

12

Crescent Glomerulonephritis is associated with

Rapid Progression of Disease

12

What activates the Complement pathway in IgA Nephropathy

Mesangial cells are activated in response to binding and phagocytosing immune complexes --> Proliferate and increase production of ECM proteins and cytokines

13

Features of Laminin

Binds to other components

14

Features of Entactin

Calcium binding properties

14

Form of Glomerulonephritis w/ localization of immune complexes in the mesangium

IgA Nephropathy

14

Subepithelial Humps

Pattern of immune complex deposits in Post-Strep Glomerulonephritis EM

15

Leukocyte mechanisms of glomerular injury and malfunction

Leakage of lysosomal proteases and ROS, Production of AA metabolites that reduce GFR

17

What is the Non-Collagenous Domain

Non-helical globular domain

19

Infectious diseases involve what parts of nephron

Tubules and Interstitium far more often than glomeruli

20

What are Thrombotic Microangiopathies

Loss of normal endothelial function needed to prevent thrombosis

20

Result of Foot Process Effacement

Retraction and loss of SPD; Detachment from GBM and degradation of GBM --> Plasma protein linkage

20

What mediates the accelerated aging in DM

Advanced Glycation end-products (AGE)

21

What is Arterio-Nephro-Sclerosis

(1) Narrowing of lumen from plasma leakage; (2) Gradual ischemic atrophy of glomerulus; (3) End result is global sclerosis of glomeruli

23

In whom is malignant HTN more common

Black males around 40

24

What tends to have a Linear pattern on immunofluorescence and be invisible on EM

Anti-GBM Ab deposition

26

2 diseases associated leading to Arterionephrosclerosis

HTN, DM

27

Wire Loops

Extensive confluent subendothelial deposits thickening the capillary walls

29

Nephrin and its associated protein are crucial to

Maintaining selective permeability of glomerular filtration barrier

31

Gross appearance of kidney in malignant HTN

Flea-Bitten

31

Crescent-shaped area of inflammation w/ proliferation of parietal epithelial cells

Crescentic Glomerulonephritis

32

2 diseases associated with ApoL1 mutation

Arterionephrosclerosis, Focal Segmental Glomerulosclerosis

33

Strong negative charge - prevents Albumin from entering

Features of Perlecan

35

What happens to arterioles in Malignant HTN

Fibrinoid Necrosis of Arterioles

36

What is Crescentic Glomerulonephritis

Crescent-shaped area of inflammation w/ proliferation of parietal epithelial cells

37

Diseases caused by Ab's against epitope in the NC1 domain of alpha3 chain cause

Glomerulonephritis w/ Hematuria

38

Effects if DM on nephron

Mesangial cell hypertrophy and hyperplasia; Diffuse mesangial matrix production; Podocyte injury and apoptosis; Thickening of tubular BM's

39

Most common glomerular disease

Vascular, Hypertensive Nephropathy

40

What causes the damage in IgA Nephropathy

Complement activation generates the injurious inflammation

42

In whom is end-stage renal disease due to HTN more common

8x more common in blacks

43

What can cause Crescentic Glomerulonephritis

Leakage of plasma proteins into urinary space (particularly when mixed with Ab's, Immune Complexes, Inflammatory Cells, and ROS)

44

Renal neoplasms arise from

Tubular Epithelium

46

Result of injury to glomerular capillary endothelium

Loss of normal endothelial function needed to prevent thrombosis

47

Pattern of immune complex deposition in immunofluorescence

Clumps, granular pattern

48

Goodpasture Syndrome

Glomerulonephritis w Hematuria, along w/ Pulmonary Hemorrhage and Hemoptysis

48

What does PAS stain highlight

Cellular cytoplasmic inclusions

50

Effect of supra-normal glomerular capillary pressure on glomerulus

GBM thickening, Mesangial Hypertrophy and Hyperplasia, Mesangial Matrix production

52

When proliferating parietal epithelial cells and infiltrating macrophages and inflammatory exudate and leaked plasma create a visible crescent of inflammation around glomerular tuft of cpaillaries

Crescentic Glomerulonephritis

54

Slit Pore Diaphragm proteins that serve to bind adjacent pediceles

Cadherin, FAT

56

Glomerular diseases that involve the capillary endothelium or subendotheial portion of glomeruli tend to cause

Hematuria

57

Site of Ab deposition in SLE

Subendothelial

58

Compensation for nephron loss requires

Increased filtration per glomerulus and increased glomerular transcapillary pressure

59

In conditions causing severe loss of protein thru slit pore diaphragms, EM frequently shows

Fusion of Foot Processes = Effacement

61

Ab against NC1 domain + Ab's against lung basement membrane

Goodpasture Syndrome

62

Second most common glomerular disease

DM

63

Mesenchymal cells w/ phagocytic and contractile properites

Mesangial Cells

64

With what is C-ANCA associated

Granulomatosis with Polyangiitis

65

Vascular clotting disorders such as HUS, TTP, and DIC involve what part of nephron

Glomerulus

65

Anti-GBM Ab deposition tends to have what pattern

Linear pattern on immunofluorescence and be invisible on EM

67

End result of many glomerular diseases

Fibrous replacement of glomerulus = Glomerulosclerosis

69

What is hypothesized to happen in Post-Strep Glomerulonephritis

Antigens and Ab's arrive in glomerulus separately and form complexes in situ

70

Features of Fibronectin

Connection and adhesion of components

71

Extensive confluent subendothelial deposits thickening the capillary walls

Wire Loops

72

Connection and adhesion of components

Fibronectin

73

Glomerular diseases that involve which side of glomerulus are worse

Capillary side

75

What causes the great majority of Membranous Glomerulonephritis

In situ immune complex formation with Ab's against podocyte cell membrane antigens

76

Glomerular diseases that involve only the podocytes, slit diaphragm, or subepithelial portion of the glomeruli tend to cause

Proteinuria

78

Presentation of Malignant HTN

Symptoms of increased intracranial pressure: Headache, Scotomas, Vomitting

80

What happens to surviving nephrons after nephron loss

Hypertrophy, with hyperplasia of glomerular and tubular cells, longer tubules

81

Hyperplastic Arteriosclerosis + Arterial Fibrinoid Necrosis

Malignant HTN

82

What causes Flea-Bitten Kidney

Small arteries/arterioles damaged by malignant HTN rupture all over kidney

83

Toxic diseases tend to involve what part of nephron

Tubules more than Glomeruli

85

(1) Narrowing of lumen from plasma leakage; (2) Gradual ischemic atrophy of glomerulus; (3) End result is global sclerosis of glomeruli

What is Arterio-Nephro-Sclerosis

86

Glomerulonephritis w Hematuria, along w/ Pulmonary Hemorrhage and Hemoptysis

Goodpasture Syndrome

87

Histopathologic correlate of the clinical syndrome of rapidly progressive glomerulonephritis

Crescentic Glomerulonephritis

88

What does Segmental Glomerular Disease mean

Involves only part of glomerulus

89

Membranous glomerulopathy is characterized by

Increased glomerular basement membrane, w/out an increase in cells

90

What percent of glomerular disease in children is primary? In adults?

95%; 60%

91

With what is P-ANCA associated

Microscopic Polyangiitis, Churg-Strauss

92

Cell binding of basement membrane is mediated mostly by

Integrins

93

Slit Pore Diaphragm proteins that play role in filtration

Nephrin, Podocin

94

Effect of AA metabolites on glomerulus

Reduce GFR

95

AI diseases highly favor what part of nephron

Glomeruli