Renal - Nephritic Syndromes

Question 1

Name four diseases that can cause nephritic syndrome.

(1) __________ __________itis
(2) __________ __________ __________itis
(3) IgA nephropathy (Berger’s disease)
(4) Alport syndrome

Answer 1

Name four diseases that can cause nephritic syndrome.

(1) Poststreptococcal glomerulonephritis
(2) Rapidly progressive glomerulonephritis
(3) IgA nephropathy (Berger’s disease)
(4) Alport syndrome

Question 2

Name four diseases that can cause nephritic syndrome.

(1) Poststreptococcal glomerulonephritis
(2) Rapidly progressive glomerulonephritis
(3) ______ __________ (__________’s disease)
(4) __________ syndrome

Answer 2

Name four diseases that can cause nephritic syndrome.

(1) Poststreptococcal glomerulonephritis
(2) Rapidly progressive glomerulonephritis
(3) IgA nephropathy (Berger‘s disease)
(4) Alport syndrome

Question 3

Nephritic syndromes are a series of glomerular disorders characterized by glomerular _________ and _________.

Answer 3

Nephritic syndromes are a series of glomerular disorders characterized by glomerular inflammation and bleeding.

Question 4

Nephritic syndromes are characterized by a proteinuria of ___ 3.5 g/day.

Answer 4

Nephritic syndromes are characterized by a proteinuria of < 3.5 g/day.

Question 5

Nephritic syndromes are characterized by ____temia and _____uria.

Answer 5

Nephritic syndromes are characterized by azotemia and oligouria.

Question 6

What is found in the urine in patients with nephritic syndromes?

Answer 6

RBC casts and dysmorphic RBCs

Question 7

__________ syndromes are characterized by azotemia, limited proteinuria (< 3.5 g/day), oliguria, and salt retention (resulting in hypertension and periorbital edema).

Answer 7

Nephritic syndromes are characterized by azotemia, limited proteinuria (< 3.5 g/day), oliguria, and salt retention (resulting in hypertension and periorbital edema).

Question 8

What will a biopsy reveal in patients with nephritic syndrome?

Answer 8

Hypercellular, inflammed glomeruli

Question 9

Nephritic syndrome ________-________ deposition activates complement; ____ then attracts neutrophils, which mediate the glomerular damage.

Answer 9

Nephritic syndrome immune-complex deposition activates complement; C5a then attracts neutrophils, which mediate the glomerular damage.

Question 10

_________ syndrome immune-complex deposition activates complement; C5a then attracts _________, which mediate the glomerular damage.

Answer 10

Nephritic syndrome immune-complex deposition activates complement; C5a then attracts neutrophils, which mediate the glomerular damage.

Question 11

What cause of nephritic syndrome can result following S. pyogenes skin or pharyngeal infection?

Answer 11

Poststreptococcal glomerulonephritis

Question 12

Poststreptococcal glomerulonephritis typically only occurs following infections with _______genic strains of S. pyogenes.

Answer 12

Poststreptococcal glomerulonephritis typically only occurs following infections with nephritogenic strains of S. pyogenes​.

Question 13

True/False.

Poststreptococcal glomerulonephritis may arise following infection with some nonstreptococcal organisms as well.

Answer 13

True.

Question 14

How does poststreptococcal glomerulonephritis typically present?

Answer 14

Hematuria;

oliguria;

hypertension;

periorbital edema

Question 15

Poststreptococcal glomerulonephritis is typically seen in ________ (age group) but may present in ________ (age group).

Answer 15

Poststreptococcal glomerulonephritis is typically seen in children but may present in adults.

Question 16

How does poststreptococcal glomerulonephritis typically present on light microscopy with H&E stain?

Answer 16

Hypercellularity;

inflammed glomeruli

Question 17

Poststreptococcal glomerulonephritis is mediated by _________-_________ deposition that presents as __________ ‘humps’ on electron microscopy.

Answer 17

Poststreptococcal glomerulonephritis is mediated by immune-complex deposition that presents as subepithelial ‘humps’ on electron microscopy.

Question 18

Treatment for poststreptococcal glomerulonephritis is ___________.

Answer 18

Treatment for poststreptococcal glomerulonephritis is supportive.

Question 19

How frequently does poststreptococcal glomerulonephritis progress to renal failure in afflicted children?

Answer 19

Rarely

(1% of cases)

Question 20

Some adults with poststreptococcal glomerulonephritis develop a ________ ________ glomerulonephritis.

Answer 20

Some adults with poststreptococcal glomerulonephritis develop a rapidly progressive glomerulonephritis (RPGN).

Question 21

What is rapidly progressive glomerulonephritis?

Answer 21

Nephritic syndrome that progresses to renal failure in weeks to months

Question 22

In addition to the clinical picture, how does rapidly progressive glomerulonephritis appear on imaging?

Answer 22

Light microscopy on H&E: crescents in Bowman’s space (comprised of fibrin and macrophages)

Question 23

In rapidly progressive glomerulonephritis, the crescents seen on light microscopy are comprised of what?

Answer 23

Fibrin and macrophages

Question 24

What is the most common cause of nephropathy worldwide?

Answer 24

IgA nephropathy (Berger’s disease)

Question 25

IgA nephropathy (Berger's disease) is characterized by IgA \_\_\_\_\_\_\_\_-\_\_\_\_\_\_\_\_ depositions in the glomerular \_\_\_\_\_\_\_\_\_\_\_.

Answer 25

IgA nephropathy (Berger's disease) is characterized by IgA **_immune_**-**_complex_** depositions in the glomerular **_mesangium_**.

Question 26

IgA nephropathy (Berger's disease) typically presents in __________ (age group) as gross or microscopic hematuria with ______ \_\_\_\_\_\_.

Answer 26

IgA nephropathy (Berger's disease) typically presents in **_children_** as gross or microscopic hematuria with **_RBC_** **_casts_**.

Question 27

\_\_\_\_\_ nephropathy (Berger's disease) typically presents in children as gross or microscopic _________ with RBC casts.

Answer 27

**_IgA_** nephropathy (Berger's disease) typically presents in children as gross or microscopic **_hematuria_** with RBC casts.

Question 28

Via what imaging modality can the mesangial immune deposits associated with IgA nephropathy (Berger's disease) be visualized?

Answer 28

Immunofluorescence

Question 29

**True/False**. IgA nephropathy (Berger's disease) may slowly progress to renal failure.

Answer 29

True.

Question 30

IgA nephropathy (Berger's disease) presents with immune-complex depositis in what location?

Answer 30

The glomerular mesangium

Question 31

IgA nephropathy (Berger's disease) usually follows what?

Answer 31

**Mucosal infection** | (e.g. gastroenteritis)

Question 32

Which cause of nephritic syndrome is characterized by the presence of episodic hematuria and RBC casts present in the urine?

Answer 32

**IgA nephropathy** | (Berger's disease)

Question 33

Alport syndrome is an inherited defect of what?

Answer 33

Type IV collagen

Question 34

Alport's syndrome most commonly follows what inheritance pattern?

Answer 34

**X-linked** (typically affects the males in a family)

Question 35

Alport's syndrome results in thinning and splitting of the glomerular _________ \_\_\_\_\_\_\_\_\_.

Answer 35

Alport's syndrome results in thinning and splitting of the glomerular **_basement_** **_membrane_**.

Question 36

What are the three major S/Sy of Alport's syndrome?

Answer 36

(1) Isolated **hematuria** (2) Sensory **deafness** (3) **Ocular** disturbances

Question 37

Linear immunofluorescence indicates which rapidly progressive glomerulonephritis?

Answer 37

Goodpasture syndrome

Question 38

Granular immunofluorescence indicates which two etiologies of rapidly progressive glomerulonephritis?

Answer 38

(1) Poststreptococcal glomerulonephritis (MC) (2) Diffuse proliferative glomerulonephritis

Question 39

A lack of immunofluorescence could indicate which three etiologies of rapidly progressive glomerulonephritis?

Answer 39

(1) **Granulomatosis with polyangiitis** (Wegener's granulomatosis) (2) **Microscopic polyangiitis** (3) **Eosinophilic granulomatosis with polyangiitis** (Churg-Strauss disease)