Nephritis

Question 1

Glomerulonephritis (GN) presents with more (1) symptoms and findings (nephritic presentation) than the (2) and associated findings in nephrotic syndrome.

Answer 1

1. inflammatory

2. proteinuria

Question 2

The major pathologic finding in glomerulonephritis is the presence of (1) and (2), which leads to (3) within tubules

Answer 2

1. leukocytic infiltration
2. bleeding
3. red blood cell casts

Question 3

The clinical presentation of glomerulonephritis thus includes (1), (2), (3)

Answer 3

1. hematuria
2. proteinuria
3. decreased renal function as determined by glomerular filtration rate (GFR).

Question 4

In decreasing renal function, one sees (1), as well as (2), and (3)

Answer 4

1. elevated serum blood urea nitrogen (BUN) and creatinine
2. oliguria (decreased urine output)
3. salt and water retention which contributes to edema

Question 5

With continuing renal damage progressing from acute to chronic glomerulonephritis, there is also the development of (1)

Answer 5

1. hypertension

Question 6

Hypertension occurs when the kidney is not delivering as much (1) to the (2), so there is (3) being produced in each nephron.

Answer 6

1. sodium
2. distal tubules
3. increased renin, angiotensin, and aldosterone

Question 7

(1) are the essential functional unit of the kidney, and consist of the glomerulus and its associated tubules and blood supply. Each kidney has approximately one million (1)

Answer 7

1. Nephrons

Question 8

A distinct presentation known as rapidly progressive glomerulonephritis (RPGN) is associated with the formation of (1) within (2)

Answer 8

1. crescents

2. Bowman’s space

Question 9

One classic example of acute glomerulonephritis is (1) which occurs in children about 1-2 weeks after recovery from (2)

Answer 9

1. post-streptococcal glomerulo-nephritis

2. a sore throat

Question 10

Post-streptococcal glomerulo-nephritis patients develop (1) (from the mild proteinuria), (2) and (3) urine which contains (4)

Answer 10

1. periorbital edema
2. fever, oliguria
3. cola colored
4. red blood cell casts

Question 11

Post-streptococcal glomerulo-nephritis patients can also develop (1) due to the effects of decreased (2) delivery to (3)

Answer 11

1. hypertension
2. sodium
3. distal tubules

Question 12

Post-streptococcal glomerulo-nephritis causes the nephron to locally produce (1), activating (2), which together increase blood pressure by elevating both total peripheral resistance (3) and stroke volume (4)

Answer 12

1. renin
2. angiotensin and aldosterone
3. vasoconstriction from angiotensin II effects
4. increased blood volume from increased sodium and water reabsorption due to aldosterone effects

Question 13

Adults with post-streptococcal GN have a more atypical presentation and can merely have (1), (2), or (3)

Answer 13

1. sudden hypertension
2. edema
3. increasing BUN (called azotemia).

Question 14

Lab findings in post-streptococcal GN include evidence of the strep exposure such as increased titers of (1); and evidence of immune response (utilization of complement components as evidenced by (2) or other complement factors).

Answer 14

1. antistreptococcal antibodies

2. decreased serum C3

Question 15

A similar GN to post-streptococcal GN occurs sporadically in association with other infections such as?

Answer 15

bacterial (staph endocarditis, pneumococcal pneumonia, meningococcemia), viral (e.g., hepatitis B, hepatitis C, mumps, HIV infection, varicella, and infectious mononucleosis), and parasitic (malaria, toxoplasmosis).

Question 16

The classic pathophysiologic findings on electron microscopy for post-streptococcal GN is the (1) or large deposits present between the (2)

Answer 16

1. subepithelial “humps”

2. podocytes and the basement membrane.

Question 17

In post-streptococcal GN, there is also localized proliferation of (1) which occlude the lumen resulting in decreased blood flow.

Answer 17

1. capillary endothelial cells

Question 18

Another type of glomerulonephritis is membranoproliferative GN. In type I, there are prominent (1) and (2) visualized by light microscopy due to the increased number of (3) and increased (4)

Answer 18

1. sub endothelial deposits
2. lobulated glomeruli
3. mesangial cells
4. basement membrane and mesangial matrix.

Question 19

The interposition of (1) between (2) creates a double-contour effect which looks like a double basement membrane.

Answer 19

1. mesangial cells and basement membrane
2. endothelial cells and basement membrane

refers to type I membranoproliferative GN

Question 20

The (1) responsible for membranoproliferative GN is not known, but it is known to resolve when patient infections are successfully treated in those rare patients who have an infectious causation.

Answer 20

1. antigen

Question 21

Secondary infectious causes of Type I membranoproliferative GN include ?

Answer 21

subacute bacterial endocarditis (a heart valve infection) or osteomyelitis.

Question 22

Some patients have secondary membranoproliferative GN due to ?

Answer 22

malignancy.

Question 23

Type II membranoprolferative GN is also known as (1) because of the electronmicroscopic findings of (2) in the (3)

Answer 23

1. dense deposit disease
2. very electron dense immune complex deposition
3. basement membrane.

Question 24

Most patients with Type II membranoproliferative GN have a serum (1) autoantibody called (2) which stabilizes the (3) of the alternative complement pathway to produce prolonged cleaving activity

Answer 24

1. IgG
2. C3 nephritic factor
3. C3 convertase enzyme

Question 25

Stabilization of the C3 nephritic factor results in activation of the alternative complement pathway with (1) deposition in the glomerular (2) and a corresponding (3)

Answer 25

1. C3 and properdin 2. basement membrane 3. decreased serum C3 and factor B. refers to Type II membranoproliferative GN

Question 26

However, patients with Type II membranoproliferative GN have normal serum levels of (1) (because (2) are not activated in this alternative pathway).

Answer 26

1. C1 and C4 | 2. immune complexes

Question 27

Type II MPGN often recurs in (1), suggesting that glomerular injury is mediated through some unknown humoral factor.

Answer 27

1. renal transplants

Question 28

Another renal disease which can produce either a nephritic or a nephrotic presentation is (1). This disease preferentially affects the (2) which have the most concentrating ability

Answer 28

1. focal segmental glomerulosclerosis (FSGS) | 2. juxtamedullary glomeruli

Question 29

FSGS produces increased (1) that affects some of the (2) (focal) and initially only a part of an affected (3) (segmental).

Answer 29

1. collagenous matrix 2. glomeruli 3. glomerular tuft

Question 30

FSGS is seen in conditions where the renal mass can’t meet the renal demands such as decreased renal mass due to congenital (1) or acquired (2) or conditions which overwork the kidney such as (3)

Answer 30

1. unilateral agenesis 2. reflux nephropathy 3. obesity or reduced oxygen conditions (sickle cell disease or congenital heart disease).

Question 31

During the late 20th century, FSGS was commonly seen in (1) patients although the incidence in both of these populations has decreased and idiopathic FSGS is becoming increasingly common.

Answer 31

1. heroin addicts and in black HIV

Question 32

The lesions observed in FSGS can be either (1)

Answer 32

1. sclerosis or hyalinosis

Question 33

Sclerosis consists of (1) deposition in the (2)

Answer 33

1. extracellular collagen | 2. mesangium and capillary loops.

Question 34

Hyalinosis (homogeneous and eosinophilic) is thought to be due to (1) which enter (2) structures as a consequence of (3)

Answer 34

1. plasma proteins 2. glomerular 3. endothelial or capillary injury.

Question 35

There are a number of different variant types of FSGS including ones in which the sclerosis either affects just the (1) region or collapses due to continuing injury

Answer 35

1. perihilar

Question 36

HIV FSGS can be associated with the (1) variant.

Answer 36

1. collapsing

Question 37

Rapidly progressive GN (RPGN) represents a serious syndrome with multiple causes. The patients present with a sudden onset of ?

Answer 37

oliguria and nephritis.

Question 38

Renal biopsy in cases of Rapidly progressive GN (RPGN) shows the formation of (1) which are formed by proliferation of the (2) followed by infiltration of (3)

Answer 38

1. crescents 2. parietal epithelial cells lining Bowman’s capsule 3. monocytes and macrophages.

Question 39

The presence of (1) in (2) is thought to be the initiating factor which leads to crescent formation in RPGN

Answer 39

1. fibrin | 2. Bowman’s space

Question 40

RPGN can be due to (1) or the so-called pauci-immune form, which involves (2)

Answer 40

1. antibodies or immune complexes | 2. antineutrophil cytoplasmic antibodies (ANCA).

Question 41

Goodpasture Diseases is a type of RPGN caused by (1) antibodies directed against the Goodpasture antigen (a peptide within (2) which cross-reacts with (3) to cause (4)

Answer 41

1. anti-glomerular basement membrane 2. alpha3 chain of type IV collagen 3. pulmonary alveolar basement membrane 4. crescentic glomerulonephritis and pulmonary hemorrhage

Question 42

The anti-glomerular basement membrane antibodies in Goodpasture Disease attract complement producing linear deposits of (1) within the (2)

Answer 42

1. IgG and C3 | 2. glomeular basement membrane.

Question 43

Other RPGN patients have a granular deposition of (1) within the (2) and associated (3) and have the disorder known as Henoch-Schonlein purpura

Answer 43

1. IgA 2. glomeruli 3. cutaneous bruising

Question 44

The most common type of RPGN is the pauci-immune type, which is due to circulating (1)

Answer 44

1. anti-neutrophil cytoplasmic antibodies (ANCAs).

Question 45

It is called pauci-immune because few immunoglobulin components are identified in biopsy studies because the disease is mediated more by (1) than by (2)

Answer 45

1. neutrophils | 2. lymphocytes

Question 46

ANCAs can either be to the peripheral antigen myeloperoxidase (1) or to the more central PR3 antigen (2).

Answer 46

1. p-ANCA | 2. c-ANCA

Question 47

Studies in mice indicate that transferring antibodies against (1) can induce RPGN, suggesting that the antibodies cause the disease.

Answer 47

1. myeloperoxidase (target antigen for p-ANCAs)

Question 48

It is believed that the antibodies in RPGN bind to (1) and cause them to adhere to (2), release toxic oxygen metabolites, de-granulate, and kill the (2)

Answer 48

1. neutrophils | 2. endothelial cells

Question 49

Direct injury to capillary walls caused by neutrophil activity on endothelial cells, leads to leakage of (1), and inflammatory mediators into the urinary space including (2). The formation of (2) leads to the proliferation of the (3) and infiltration by (4) which produces the crescent and further damage

Answer 49

1. coagulation factors 2. fibrin. 3. parietal epithelial cells 4. macrophages

Question 50

IgA nephropathy is another type of immune nephritis which can occur and can be either primary or secondary (observed in children with (1) or adults with (2).

Answer 50

1. Henoch-Schonlein | 2. intestinal or liver disease

Question 51

IgA nephropathy presents with (1) and there are prominent (2) deposits in (3) on renal biopsy.

Answer 51

1. recurrent hematuria or nephritis 2. IgA 3. mesangial cells

Question 52

The primary type of IgA nephropathy most commonly occurs in children and younger adults after (1) and can slowly progress to renal failure and even recur in the (2) in some cases.

Answer 52

1. infection | 2. transplanted graft

Question 53

Alport syndrome is an inherited disease which is usually (1) with females having (2) and in males exhibiting the entire syndrome.

Answer 53

1. X-linked | 2. hematuria

Question 54

However, as is often the case with genetic diseases, autosomal dominant and recessive forms also exist in some families.

Answer 54

Alport syndrome

Question 55

Maximally affected patients with Alport syndrome have (1) which progresses to chronic renal failure

Answer 55

1. nerve deafness, eye disorders, and hematuria

Question 56

Alport syndrome is caused by inherited mutations in (1)

Answer 56

1. type IV collagen.

Question 57

In contrast to Alport syndrome, patients with Benign familial hematuria (also called (1) present with (2).

Answer 57

1. thin basement membrane lesion | 2. asymptomatic hematurias and mild proteinuria

Question 58

Electron microscopy of benign familial hematuria shows (1); they have mutations in (2). However, the patients have normal renal function and an excellent prognosis

Answer 58

1. diffuse thinning of the basement membrane | 2. certain chains of type IV collagen.

Question 59

Type I MPGN--> Proliferation of 1) and 2)

Answer 59

Mesangial cells; endothelial cells

Question 60

Capillary walls are thickened,:silver stains show a doubling or replication of GBMs. EM shows this is deposition of new BM between the mesangial and endothelial cell

Answer 60

Type I Membranoproliferative Glomerulonephritis

Question 61

Among these patients, 20% will have crescents, usually only a minority of glomeruli

Answer 61

Type I Membranoproliferative Glomerulonephritis

Question 62

Immunofluorescence microscopy shows granular deposition of immunoglobulins and complement in glomerular capillary loops and mesangium

Answer 62

Type I Membranoproliferative Glomerulonephritis

Question 63

Type I MPGN | IF--> granular staining for C3 in the 1) and 2)

Answer 63

1) capillary walls | 2) mesangium

Question 64

due to Mutations in, alternative pathway complement (factor H)

Answer 64

Type II MPGN

Question 65

Type II MPGN: serum IgG autoantibody, termed 1), which stabilizes the activated C3 convertase enzyme (C3bBb) of the 2). The result is prolongation of C3 cleaving activity

Answer 65

1) C3 nephritic factor 2) alternative complement pathway

Question 66

Type II MPGN often recurs in 1) , suggesting that glomerular injury is mediated through some unknown humoral factor

Answer 66

1) renal transplants

Question 67

EM demonstrates thickening of the basement membrane and intramembranous dense deposits.

Answer 67

Type II MPGN (dense deposit | disease).

Question 68

IF-->bands of capillary wall staining and coarsely granular mesangial staining for C3

Answer 68

Type II MPGN

Question 69

Hereditary forms (mutations in podocyte genes): 1. Podocin 2. Alpha-actin-4 3. TRPC6 (transient receptor potential cation channel 6)

Answer 69

Focal Segmental Glomerulosclerosis

Question 70

ANCA glomerulonephritis is an aggressive,1)-mediated disease that is characterized by 2) and 3)

Answer 70

1) neutrophil 2) glomerular necrosis 3) Crescents