Path 1-2

Question 1

Most common cystic disease in children

Answer 1

Renal dysplasia

Question 2

Increased incidence with Turner syndrome

Majority fused at lower pole

Answer 2

Horseshoe kidney

Question 3

Autosomal recessive
PKHD1 gene (fibrocystin)
More than 50% develop end stage renal disease within the first decade

Answer 3

Juvenile polycystic kidney disease

Question 4

Juvenile polycystic kidney disease is associated with ___

Answer 4

Hepatic cysts
Congenital hepatic fibrosis
Maternal oligohydramnios
-Potter facies in newborns (low set ears, parrot beak nose, lung hypoplasia)

Question 5

Autosomal dominant 
Chromosome 16
PKD1 gene (polycystin)
Associated with stroke due to rupture of intracranial berry aneurysms 
Tx: transplant

Answer 5

Adult polycystic kidney disease

Question 6

No inheritance pattern
Commonly seen with IVP
striations in papillary ducts of the medulla (swiss cheese appearance)
Recurrent UTIs, hematuria, renal stones

Answer 6

Medullary sponge kidney

Question 7

Most common cause is renal dialysis
(~50% of pts on long term dialysis)
Tubules obstructed by interstitial fibrosis or oxalate crystals
Small risk for renal cell carcinoma

Answer 7

Acquired polycystic kidney disease

Question 8

Most common adult renal cyst
Derived from tubular obstruction
May produce hematuria
Requires needle aspiration to distinguish from renal cell carcinoma

Answer 8

Simple retention cysts

Question 9

Only a few glomeruli are abnormal

Answer 9

Focal glomerulonephritis

Question 10

All glomeruli are abnormal

Answer 10

Diffuse glomerulonephritis

Question 11

More than 100 nuclei in the affected glomeruli

Answer 11

Proliferative glomerulonephritis

Question 12

Thick glomerular basement membrane

Answer 12

Membranous glomerulonephritis

Question 13

Thick glomerular basement membrane and hypercellular glomeruli

Answer 13

Membranoproliferative glomerulonephritis

Question 14

Involves only a segment of involved glomeruli

Answer 14

Focal segmental glomerulonephritis

Question 15

Proliferation of parietal epithelium

Answer 15

Crescentic glomerulonephritis

Question 16

HTN
Hematuria
Proteinuria >150mg/day but <3.5g/day

Answer 16

Nephritic syndrome

Question 17

Most common type of glomerulonephritis worldwide 
IgA deposits in mesangial region 
Recurrent hematuria
Mild proteinuria 
Crescentic RPGN, rarely
Abnormally glycosylated IgA1 
Antiglycan antibodies

Answer 17

IgA glomerulopathy aka Berger disease

Question 18

Pathogenesis of Berger disease

Answer 18

Defect in galactose-containing sugar chains called O-linked flu and to hinge region of IgA prior to their secretion by B cells

Question 19

Clinical presentation of Berger disease

Answer 19

Common in older children and young adults

-reoccurring gross hematuria after infection of respiratory, GI, or urinary tract

Question 20

Commonly in children 6-10 y/o after group A beta hemolytic strep infection of skin or respiratory tract
Caused by immune complexes formed in situ
IgG deposits

Answer 20

Acute proliferative glomerulonephritis

Question 21

What types of strep are nephritogenic

Answer 21

1
4
12

Question 22

Young child abruptly develops fever, malaise, nausea, oliguria, and hematuria (smoked or Coca Cola colored urine) 1-2 weeks after recovery from sore throat

Dysmorphic red cells or red cell casts in urine
Mild proteinuria
Periorbital edema 
HTN
Decline in C3

Answer 22

Acute proliferative glomerulonephritis (Poststrep or postinfectious)

Question 23

How does post staph glomerulonephritis differ from post strep?

Answer 23

IgA vs IgG

Question 24

Primarily nephritic type glomerular diseases:

Answer 24

1. IgA glomerulopathy (Berger disease)
2. Poststrep glomerulonephritis
3. Diffuse proliferative glomerulonephritis (SLE)
4. Rapidly progressive crescentic glomerulonephritis

Question 25

Diagnosis of post strep glomerulonephritis

Answer 25

Increased antistreptolysin O Anti-DNase B tigers Positive streptozyme test

Question 26

1. Subepithelial IC deposits with granular IF | 2. Subendothelial IC deposits ("wire looping") with granular IF

Answer 26

1. Post strep glomerulonephritis | 2. Diffuse proliferative glomerulonephritis (SLE)

Question 27

Morphology of diffuse proliferative glomerulonephritis (SLE)

Answer 27

Crescentic GN | Linear IF

Question 28

Generalized pitting edema Hypercoagulable state Hypercholesterolemia Proteinuria >3.5g/day

Answer 28

Nephrotic syndrome

Question 29

``` Nephrotic syndrome: Primary: 1. 2. 3. 4. ```

Answer 29

1. Minimal change disease (lipid nephrosis) 2. Focal segmental glomerulosclerosis 3. Membranous glomerulopathy 4. Membranoproliferative glomerulonephritis (MPGN)

Question 30

What is the most common cause of nephrotic syndrome in children? What is it preceded by?

Answer 30

Minimal change disease (lipoid nephrosis) - URI or routine immunization - secondary cause is Hodgkin lymphoma

Question 31

``` Morphology: Normal glomeruli Fat stains Negative IF EM shows fusion of podocytes ``` *responds well to steroids

Answer 31

Minimal change disease (lipoid nephrosis)