Firecracker IV

Question 1

First presenting symptom of Goodpasture syndrome

Answer 1

hemoptysis

Question 2

Second presenting symptom of Goodpasture syndrome

Answer 2

Hematuria

Question 3

Why do you get hemoptysis (and cough and SOB) in Goodpasture syndrome?

Answer 3

Pulmonary hemorrhage

Question 4

Why do you get hematuria in Goodpasture syndrome?

Answer 4

Rapidly progressive renal failure with S/Sx of nephritic syndrome

Question 5

Three presenting signs of pulmonary hemorrhage

Answer 5

hemoptysis, cough, dyspnea

Question 6

Presenting signs of RPGN

Answer 6

hematuria (2nd presenting symptom), RBC casts, azotemia, oliguria, mild/moderate hypertension, mild/moderate proteinuria and edema

Question 7

Timeline of Goodpasure

Answer 7

Hemoptysis –> couple weeks –> hematuria

Question 8

What are the deposits in IgA nephropathy

Answer 8

Mesangial deposition of IgA1, often with C3 and properdin

Question 9

Which Ig is produced by mucosal tissue & follows infections of mucosal tissue

Answer 9

IgA

Question 10

Adolescents/young adults with nephrotic syndrome and a nephritic component (eg, hematuria) → slow progression to renal failure

Answer 10

MPGN (membranoproliferative glomerulonephritis):

Question 11

MPGN type I and type II glomerular appearance

Answer 11

Glomeruli are large and hypercellular due to WBC infiltrate, proliferation of mesangial cells and endothelial cells, and ↑ mesangial matrix

Question 12

Mesangial ingrowth and new GBM (glomerular basement membrane) synthesis in response to deposition of immune complexes → duplication/splitting of GBM → thick GBM w/ “tram-track” or “double contour” appearance (esp. evident w/ silver or PAS stains)

Answer 12

MPGN types I and Ii

Question 13

location of IC in MPGN type 1

Answer 13

Subendothelial

Question 14

location of IC in MPGN type 2

Answer 14

membranoproliferative

Question 15

complement in MPGN type 1

Answer 15

Activates both classical and alternative complement → ↓ serum C1, C4, C3

Question 16

complement in MPGN type 2

Answer 16

• Activates alternative complement only → ↓ serum C3; normal C1 and C4
• Associated with C3 nephritic factor, an IgG autoantibody that stabilizes C3bBb (alternative complement C3 convertase) → persistent C3 activation → ↓ serum C3

Question 17

composition of ICs in MPGN type 1

Answer 17

Subendothelial immune complex deposits of C3, IgG, and early complement components C1, C4

Question 18

composition of ICs in MPGN type 2

Answer 18

Intramembranous immune complex deposits of C3 and IgG only

Question 19

AI causes of secondary MPGN (type 1)

Answer 19

• SLE (systemic lupus erythematosus)

- SS (sjögren’s syndrome)

Question 20

Infection causes of secondary MPGN type 1

Answer 20

• Hepatitis C, usually with cryoglobulinemia
• Hepatitis B
• HIV
• Schistosomiasis

Question 21

malignancy causes of secondary MPGN type 1

Answer 21

• CLL (chronic lymphocytic leukemia)

- Lymphoma

Question 22

besides panacinar emphysema, alpha1-antitrypsin deficiency causes

Answer 22

secondary MPGN type 1

Question 23

benign congenital disorder characterized by cystic dilations of medullary collecting ducts (“swiss cheese” appearance of medulla on intravenous pyelogram); renal cortex is spared

Answer 23

Medullary sponge kidney

Question 24

benign cysts usually located in the renal cortex of normal-sized kidneys.

Answer 24

Localized (simple) renal cysts

Question 25

multiple cysts in cortex and medulla associated with end-stage renal disease and chronic dialysis therapy

Answer 25

Acquired renal cystic disease

Question 26

Most patients are asymptomatic in medullary sponge kidney, but the most common symptoms/complications include:

Answer 26

1) Recurrent kidney stones (60% of cases) 2) Hematuria 3) UTI

Question 27

Most common type of asymptomatic renal mass

Answer 27

simple renal cysts

Question 28

most patients are asymptomatic with simple renal cysts, but what might still be found on urinanalysis

Answer 28

Microscopic hematuria is occasionally a benign, incidental finding on urinalysis.

Question 29

Patients on dialysis with acquired renal cystic disease have an increased risk of

Answer 29

renal cell carcinoma

Question 30

1. Hereditary nephritis → hematuria, RBC casts 2. Sensorineural hearing loss 3. Ocular abnormalities

Answer 30

Alport syndrome

Question 31

types of ocular abnormalities seen in alport syndrome

Answer 31

(e.g., lens dislocation, posterior cataracts, corneal dystrophy)

Question 32

85% of cases of Alport syndrome are due to an X-linked recessive defect in the

Answer 32

α5 chain of type IV collagen

Question 33

Regardless of inheritance pattern or the specific defect involved, all cases of Alport syndrome are caused by

Answer 33

defective type IV collagen assembly

Question 34

defective type IV collagen assembly → structural-functional defects in the:

Answer 34

- GBM - Cochlear hair cells of the inner ear - Lens and cornea of the eye

Question 35

Alport disease on EM

Answer 35

Irregular thickening/thinning of the GBM with splitting/lamination of the lamina densa → this often gives the GBM a unique “basketweave” appearance

Question 36

Kimmelstiel-Wilson

Answer 36

diabetes nephropathy

Question 37

nodular formation in diabetic nephropathy due to deposition of

Answer 37

type IV collagen (aka microangiopathy)

Question 38

Common causes of pre-renal azotemia that can progress to ischemic ATN include

Answer 38

``` hypovolemia, impaired cardiac function, hypoalbuminemia, renal artery stenosis, NSAID use ```

Question 39

ATN initiation phase is during the first 36 hours after the initial insult.

Answer 39

There is a slight decrease in urine output and an increase in BUN.

Question 40

The maintenance phase starts 36 hours after the initial insult in ATN

Answer 40

marked oliguria and increasing BUN.

Question 41

The recovery phase of ATN occurs when the tubule epithelium regenerates.

Answer 41

Diuresis is drastically increased, and BUN returns to baseline.